A 29-year-old female married since 1 year, primigravida with 3 months amenorrhea and ultrasonographic gestational age of 8 weeks and 5 days, presented with acute onset of abdominal distension, vulval edema and breathlessness. There was no vomiting, trauma, fever, bleeding, or spotting per vaginum. She had regular past menstrual cycles and no symptoms suggestive of renal, cardiac, or liver disorders. Ultrasonography done a few weeks prior to presentation revealed a normal intrauterine pregnancy and adnexae [].
The patient was conscious, cooperative, afebrile, with no tachycardia and normotensive. Cardiovascular system examination was normal. Breath sounds were reduced on the right side. Abdominal examination revealed shiny skin, tense ascites with no guarding, rigidity, or tenderness. Vaginal examination revealed an anteverted uterus with bilateral nontender forniceal fullness. History was reconfirmed; patient had not sought any medical advice or treatment for fertility enhancement.
Blood investigations revealed hyponatremia (125 meq/l) and hypoalbuminemia (3.4 gm/dl). Hemoglobin, white blood cell count, platelet count, coagulation profile, liver enzymes, blood urea nitrogen, and serum creatinine were normal. Estradiol was elevated at 12927 pg/dl (normal range 188-2497 pg/dl). Thyroid profile and β-hCG were normal. Ultrasonography revealed a multiloculated cystic lesion of size 17 cm × 11 cm with vascularity within the wall of cystic lesion. Ascites and bilateral pleural effusion were present []. Features of OHSS were confirmed on magnetic resonance imaging (MRI) [].
Vital parameters, intake-output, and abdominal girth were monitored. Additional intravenous fluids were omitted, oral fluids were encouraged, and diuretics were initially required. Hyponatremia gradually improved. In view of severe hypoalbuminemia, intravenous albumin infusion was instituted (25 g in 0.1 L solution per day for 7 days). No interventional procedure was done for ascites and pleural effusion, both of which reduced, along with the adnexal mass. Unfractionated heparin 5000 U subcutaneous injection twice daily was instituted and continued for 9 days. She was discharged after 2 weeks of inpatient stay.
The remainder of the antenatal period was uneventful. Regular ultrasonographic monitoring showed further reduction in size of adnexal mass. At 40 weeks’ gestation, patient underwent cesarean section in view of nonprogress of labor and delivered a healthy male of 2.85 kg. Intraoperatively, an left ovarian simple cyst of 5 cm × 5 cm was observed, for which no intervention was done. Postoperative MRI revealed simple left ovarian cyst of 2 cm × 2 cm, right side normal and involuting uterus [].
To investigate further, if ovarian hyperstimulation observed in this patient was due to any mutation in FSHR gene, the patient was called back after delivery. The entire exonic region of FSHR gene was screened for known/novel mutations as described earlier.[] To our interest, we identified a novel heterozygous mutation in FSHR gene leading to a change at amino acid position 449 []. This was observed to be a transversion at nucleotide position 1346 (AC1346T to AAT) in exon 10 yielding a threonine to asparagine (Thr449Asn) substitution in the transmembrane domain helix 3 of the FSHR. The Genbank accession number for this novel mutation (T449N) is KP340897.


A 46-year-old man was admitted to the internal medicine department for evaluation of an incidental finding of leukocytosis and hypereosinophilia. He complained of a pruritic skin rash on his lower extremities for the past eight months. He denied any fever, weight loss or loss of appetite. No changes in bowel habits, arthralgias/myalgias or any other complaint were present. He denied any travel abroad in the past year or any exposures that may determine etiology of the eosinophilia.
He was not taking any medications and had no medical history of disease. Complete blood count was performed adjacent to his clinical complaints. Leukocytosis of 23,000/μL with eosinophil count of 9430/μL was observed, but platelet and hemoglobin levels were normal. No blastocysts were found in the peripheral blood. Repeated stool samples for parasites and ova were negative. Chest x-rays and electrocardiography were normal. The patient underwent a computed tomography (CT) scan of the chest, abdomen and pelvis, with no pathology found, except for a left inguinal hernia. A skin biopsy from his right leg revealed hyperkeratotic skin with mild angiocentric inflammatory changes, and no signs of vasculitis or malignancy. His bone marrow biopsy revealed normocellular bone marrow with increased number of eosinophils, which may be compatible with reactive changes. BCR-ABL and Janus kinase-2 mutations were negative, as were tests for FIP1L1/PDG-FRA and FIP1L1/PDG-FRB mutations, ruling out myeloproliferative variant HES (M-HES). The patient was offered an empirical trial of albendazole (400 mg twice a day for three days). The patient experienced complete resolution of hypereosinophilia following one week of albendazole treatment. There was no evidence of recurrence at the 10-month follow up visit.


A 32-year-old man, with a history of epilepsy for several years and with no history of travel or known exposure to helminths, was hospitalized in the internal medicine department after a short episode of unconsciousness without convulsions. Two days before his admission, he experienced right abdominal pain, without any fever or vomiting. His blood count revealed marked leukocytosis of 40,000/μL with eosinophil count of 26,000/μL. Hemoglobin and platelet counts were normal. He had normal blood chemistry and no immature cells on blood smears. Flow cytometry and bone marrow biopsy revealed no sign of malignancy. BCR-ABL and Janus kinase-2 mutations were negative, as well as FIP1L1/PDG-FRA and FIP1L1/PDG-FRB mutations for detection of M-HES. CT scans of his chest and abdomen were normal. Serology for amoeba and strongyloides was negative. Gastroscopy with mucosal biopsy displayed signs of gastritis and Helicobacter pylori infection, but no signs of eosinophilic infiltration. Empirical therapy with albendazole (400 mg twice per day for three days) was initiated, followed by a rapid decline in eosinophil count.


Our patient was a 42-year-old man and nonsmoker who presented with an 18-year history of nonprogressive dysphonia characterized by hoarse voice. He did not describe any dyspnea or cough. He was previously well and exercised frequently without symptoms. Initial bronchoscopic assessment in 2001 confirmed multiple large nodular lesions along the laryngeal and upper tracheal wall without major airway narrowing. Biopsy of the laryngeal nodules showed subepithelial deposits of amorphous eosinophilic materials consistent with amyloid. These materials revealed apple-green birefringence on Congo red stain that confirmed the diagnosis.
Repeat bronchoscopic evaluation in 2013 demonstrated progressive infiltration of amyloid to the lower trachea (Fig. ). The laryngeal features remained unchanged. However, there had been no symptom progression. The spirometry was within normal limits. Evaluation of heart, liver, and kidneys did not detect systemic involvement.


A 60-year-old female presented to the gynecologist, complaining of gradual enlargement of a right-sided tender vulvar mass. The mass was of 15 cm × 10 cm size, firm-to-hard, and fixed to the underlying soft tissue. Considering the age of the patient, the presence of a hard, fixed mass raised the suspicion of a malignancy. She had an unremarkable gynecologic history, including normal Pap smear; no intrauterine contraceptive device (IUCD) was used. On local examination, the skin was found to be smooth and showed as though healed and few active discharging sinus with yellowish granules. The whole right leg was tense and edematous. FNA yielded a purulent aspirate. The slides were stained with hematoxylin and eosin (H and E), Papanicolaou stain, May-Grünwald-Giemsa (MGG) stain, and Ziehl-Neelsen stain was done to rule out tuberculosis. The smears showed colonies of Actinomyces with radiating filaments showing an intense basophilic staining. The colonies showed branching, and were surrounded by radiating, hyaline, eosinophilic material representing the Splendore-Hoeppli phenomenon and exudates of neutrophils []. The Ziehl-Neelsen stain was negative for acid-fast bacilli. Gram staining revealed gram-positive branching filaments. A diagnosis of actinomycosis was made and excisional biopsy was advised. The excisional biopsy also revealed abscesses with actinomycotic colonies surrounded by neutrophils and chronic granulation tissue. The patient responded very well to a course of penicillin, and edema over the right leg markedly reduced in 15 days.


A 23 year old female was admitted to hospital 3 hours after being hit by motor cycle while she was crossing the road. The patient was lean & thin. The height of the patient was about 152 cm and weight of patient was 45 kg. She sustained closed short oblique fracture of tibia and fibula at middle third junction with no associated injury. Distal pulses were palpable and there was no neurological deficit.
Routine blood investigations and chest radiographs were normal. HIV, HBsAg, HCV was non reactive. Elective surgery was done by unreamed tibial interlocking nail under spinal anaesthesia, 4 hours after admission. Operative procedure took 45 minutes. Her vitals were stable in immediate post operative period. Eight hours after surgery, she was disoriented not responding to verbal command. Body temperature was 39°, and respiratory rate was about 40/min with pulse rate of 136/min. She was investigated and was immediately shifted to I.C.U.
Arterial blood gas analysis was done- pO2 was 49.1 mmHg, pCO2 was 33.8 mmHg, O2 saturation—82.1%, S.Na+-134 mmol/ L, S.K+--4.2 mmol/L. Chest roentgenogram showed opaque shadow / infiltration on left side of chest. Patient was diagnosed as a case of fat embolism and was heparinized in the dose of 5000 IU subcutaneously every 12 hourly. She was intubated, put on ventilator and was oxygenated. At the time of intubation, she had a respiratory rate of 42 / minute, SBP of 95 mm Hg, peripheral pulse of 120 / minute, and O2 saturation of 82.1%. At clinical examination, petechiae in both axillae and on conjunctiva could be seen. Patient was on ventilator for 3 days, was given CPPV breathing for another 2 days and was then extubated without any problems. She was discharged on 6th post operative day in satisfactory condition.


Our patient was 35 year old male who presented to emergency department with history of fall by tripping on a stone (fall on outstretched hand). Post trauma patient had pain and difficulty moving both the shoulders. Patient was a known alcoholic, with previous history of one episode of shoulder dislocation on right side about 2 years prior to the present episode, which was adequately treated with closed reduction and immobilisation for 3 weeks. Patient does not give any history of convulsions (past or present) and no other neuro-muscular problems. On clinical examination, patient's both upper limbs were abducted and externally rotated. Bilaterally shoulder contour was lost with flattening. Other classical signs of shoulder dislocation viz, Bryants test, Callway sign, Hamilton's ruler test were positive. Radiographs of both shoulders were obtained and clinical diagnosis of SBAD was confirmed. Both shoulders were reduced in emergency operation theater under general anaesthesia by Kocher's method and were immobilized in sling. The left was immobilized with 90° elbow flexion and the right at 110° flexion for 3 weeks. Reduction was confirmed post operatively with X-rays. Intermitted assisted excercises where started from second week onwards and at the end of immobilization patient was advised vigorous supervised physiotherapy. By six week post injury patient had full range of adduction, flexion and internal rotation. Abduction up to 110° was possible. Patient was advised to be cautious while doing overhead activities especially which require abduction and external rotation of shoulder.


A 52-year-old female had accidental incised wound over her left wrist which was primarily sutured elsewhere. She was reffered to our centre 6 months after injury with unrelieved wrist pain and progressively growing mass on the wrist. Clinially the mass was subcutaneous, mobile, non pulsatile and appeared solid. USG showed a solid tumor of size 2 x 3 cms and a decision for excision of the mass was taken. Local anaesthesia was given over the area and skin and subcutaneous tissues were dissected to isolate the tumor. It was found to be originating from the median nerve (). The tumor was carefully excised from the surrounding tissue trying to keep the nerve intact. Intraoperative examination was possible as patient was under local anesthesia and minimal motor disability and hypoesthesia were noted (). Tumor was excised and wound sutured in layers. Histopathological examinations revealed wallerian degeneration in evolutionary phase as revealed by disorganized growth in axonal and perineural structures (). The patient had a normal outcome with no pathological findings after one year follow-up


61 years old male presented with decreased range of movement arc of left knee joint and inability to run. He had history of trauma to left knee joint following dog bite 10 months ago. Initially he was treated elsewhere with anti-rabies treatment, antibiotics and analgesics following which wound healed uneventful. Patient also had history of healed leprosy. On clinical examination, a 6cm obliquely placed old healed scar due to bog bite injury, was present at the left infra-patellar area of knee. A palpable gap was present at the infra-patellar area with lack of normal patellar tendon continuity between patella & tibial tuberosity in both extension & flexion. There was no local tenderness, effusion or synovial thickening. The range of movement of knee was 200 to 1350 with extension lag of 200 with patella palpated high up in suprapatellar pouch. Q angle was normal. His hematological investigation including haemogram, ESR, serum calcium, phosphate, alkaline phosphatase, RA factor, uric acid, KFT were within normal limits.
On radiological examination, there was patella alta with loss of soft tissue shadows of continuity of patellar tendon in anterioposterior and lateral films. Insall salvati index was 0.55 confirming diagnosis of patella alta. A diagnosis of 10 month's old neglected rupture of patellar tendon with patella alta was made ().
Patient was treated surgically in supine position and the patella and patellar tendon exposed via middle approach. On exposure patellar tendon was found ruptured in the mid substance only. The ends of the ruptured patellar tendon were freshened and the dense fibrous tissue was released both proximally and distally. The patella could be pulled down. Repair of patellar tendon was done with non-absorbable suture, taking care of the patellar tendon length and augmented with a turn-o-flap of quadriceps tendon of around 8 cm × 3 cm strip over the repaired patellar tendon. This repair was further augmented with an S-S wire passed trans-osseous between patella & a 3.5 mm cortical screw post over tibial tuberosity ( and ).
Post operatively patient was immobilized in cylindrical cast in full extension for 6 weeks. During the immobilization period, weight bearing as tolerated was allowed and quadriceps exercises with straight-leg raises in the cast were performed. After 6 weeks cast was removed and gradual knee flexion & extension exercises started. At 24 months follow up patient has full range of motion of knee with 00 – 1350 with no extension lag with insall salvati index of 0.8 without implant breakage ().


A 42 years old man, who had osteoarthritis of right hip (), had an uncemented total hip replacement done through posterolateral approach. A porus coated, metal backed acetabular component with metal liner and a taper-locked modular femoral head with hydroxyapatite coated femoral stem were used. The femoral head was firmly impacted onto the neck. Stability was confirmed in both flexion and extension and the wound was closed. Initial results was excellent, the patient had no pain and could walk without supportive aids. The patient was regularly followed up to about five years and all the x-rays of those period were normal and had no abnormalities (,).
Five years after postoperatively, while the patient was rising from a chair, the patient experience a sudden click and followed by complete functional disability and impossibility to bear the weight. Standard radiograph showed disassembly of femoral neck and head while the head of prosthesis remained inside the acetabular socket. All the femoral and acetabular components visible in the x-ray are normal in appearance. There are no fracture lines in any components or complete fracture of any components or any other extra, abnormal sized or shaped metal piece visible in the x-ray (). No subsidence could be observed. Open reduction was performed, same head was assembled onto the neck and the head was reduced into the acetabular socket. There was no evidence of visible corrosion of the interface between modular head and neck of femoral components. Intra-operative images are not available as that were not taken during the surgical procedure.
Postoperative period was uneventful and a quick functional recovery was achived.


A 10-year-old boy had sudden onset of left knee pain and swelling 1-day before admission. There was no recent injury reported. He was unable to move the knee or walk due to the pain. There was no other joint pain, fever, or systemic upset. Clinical examination showed an irritable boy with marked left knee effusion. The knee joint was extremely painful with diffuse tenderness and increase in local temperature. The joint was not erythematous. The range of movement of the knee was markedly limited from 30° to 40°. Knee aspiration yielded 40 ml of blood-stained fluid. Radiographs of the knee showed local soft tissue swelling without any bone lesion (). Emergency arthroscopy was performed. Arthroscopic findings showed a 4 cm X 3 cm multi-lobulated vascular mass at the intercondylar notch with active contact bleeding. The mass was so big that it essentially occupied the arthroscopic view of the intercondylar notch (). There was no other similar lesion in any other area of the joint. Complete resection of the mass was performed through open arthrotomy. Histological examination of the resected mass confirmed the diagnosis of PVNS with areas of necrosis (). All specimens had negative bacterial culture. Knee pain and swelling improved gradually after surgery. By 2 months post operation, there was no more knee pain and the patient could run and squat as usual. Magnetic resonance imaging performed at 3 months post operation showed no residual lesion (). The latest follow-up at 1-year post operation was normal with no sign of local recurrence.


In January 2013, a 74-year-old man was admitted to the Department of Neurosurgery at Yonsei University College of Medicine due to gait disturbance and tingling sensation of both legs. The patient was receiving beta-adrenergic antagonist and calcium channel blocker for arterial hypertension. He had lost his vision in the left eye due to cataract and amblyopia in the right eye due to retinitis which had occurred 30 years ago. The patient’s family history included a mother with a brain tumor of unconfirmed pathology, and both brothers with renal cell carcinoma with VHL gene mutation (). Serum carcinoembryonic antigen (CEA) was elevated to 11.78 ng/mL (normal value, 0.0 to 5.0 ng/mL). Although he had no history of diabetes mellitus, serum glucose level was 462 mg/dL and HbA1c was 11.7%, which led to a new diagnosis of diabetes mellitus, and initiation of insulin therapy. Whole spine magnetic resonance imaging (MRI) showed multiple enhancing nodular lesions of the spinal canal suspected leptomeningeal metastasis of unknown primary malignancy (). Positron emission tomography whole body scan showed intense fludeoxyglucose uptake in a 3-cm-sized heterogeneous mass in the right kidney and focal mural thickening in the sigmoid colon ( and ). Colonoscopy showed a 5-cm-sized ulceroinfiltrative lesion in the sigmoid colon and a polypoid lesion in the rectum. Subsequent histological examination confirmed the diagnosis of adenocarcinoma and high grade tubular adenoma with focal carcinoma in situ transformation, respectively. Incisional biopsy of the spinal mass was performed and the histological examination revealed hemangioblastoma.
The patient underwent laparoscopic right partial nephrectomy, laparoscopic anterior resection of the sigmoid colon, and transanal endoscopic operation of the rectum. Histologic examination of the kidney revealed renal clear cell carcinoma (size, 1.9×1.7 cm), Fuhrman nuclear grade 3 (pT1aN0M0; stage I). In the sigmoid colon, gross pathology showed an ulcerofungating tumor (size, 4.2×3.6 cm) and 3 of 23 examined regional lymph nodes were metastasis. Subsequent histological examination confirmed typical features of colon adenocarcinoma (pT3N1bM0; stage IIIB). The rectum specimen was confirmed to be a 0.5-cm-sized well differentiated adenocarcinoma (pT1N0M0; stage I). The postoperative CEA level was 4.24 ng/mL.
Regarding clinical decision processes, we initially suspected VHL disease. Subsequently, polymerase chain reaction (PCR) sequencing analysis of VHL gene showed p.Glu70Lys (c.208G > A) mutation in exon 1, confirming the diagnosis of VHL disease (). Result of endocrine hormonal evaluation for pheochromocytoma was within normal range.
His two sons were screened for VHL disease using PCR sequencing analysis, and one progenity showed p.Glu70Lys (c.208G > A) mutation in exon 1, identical to his father. He underwent ophthalmic examination, abdomen-pelvic computed tomography scan and blood test for catecholamine metabolites, but there were no abnormal findings.
After 3 weeks from discharge, he was admitted to hospital complaining of worsened gait disturbance and numbness of both soles. Brain MRI showed three enhancing lesions in bilateral cerebellar hemispheres and left cerebellar tonsil, suggesting hemangioblastomas (). The patient underwent gamma knife surgery in cerebellar lesions and palliative radiotherapy to T9-S1 spine with a total of 45 Gy in 25 daily fractions.
After one month, follow-up imaging studies showed stable lesions in the brain and spine, but there was a newly developed hepatic metastasis in S6 and an enlarged aortocaval lymph node (). Concurrent serum CEA level increased to 19.54 ng/mL. The multidisciplinary team decided to perform surgery, laparoscopic wedge resection of liver and excision of aortocaval lymph node. Histologic examination of liver revealed a 1.6×1.2-cm-sized metastatic adenocarcinoma, moderately differentiated, clinically from colon. Pathologic finding indicated that the aortocaval lymph node was metastatic carcinoma, favoring renal clear cell carcinoma. He is on regular follow-up in the outpatient clinic and imaging studies show no evidence of recurrence and CEA level is stabilized within normal range.


When she was 47 years old, renal biopsy was performed for renal insufficiency without any abnormalities on urinalysis. She had no family history of kidney disease. Subsequent clinical information is not available.


A 25-year-old male was brought to the emergency department with an alleged history of fipronil compound consumption following which he developed three episodes of generalized tonic-clonic seizures and altered sensorium. There was no other significant history and no past history of head trauma, stroke or seizure disorder. Patient on examination had a normal pulse, blood pressure, and O2 saturation. Pupils were bilaterally pinpoint. Central nervous examination revealed that apart from altered sensorium patient had coarse tremors, incoordination. Per-abdomen examination revealed the retention of urine. Computer tomography of the brain was normal [], routine blood investigations such as hemogram, renal function tests, liver function tests, and electrolytes were also normal. The patient was given with 4 mg of lorazepam on arrival to the hospital. The patient had two more episodes of seizures even after two repeated doses of lorazepam. Injection midazolam 1 cc given as stat dose and repeated every 4 h following which seizures did not occur. The patient regained consciousness after 10 h and was responding to oral commands. However, the patient remained delirious for 3 successive days. On additional antipyschotics along with benzodiazepines patient gradually improved within a week and got discharged on 8th day.


A 9-year-old girl who was previously diagnosed with TS was referred to our department due to weakness of both lower legs for the past 3 days. She had lymphedema at birth and was diagnosed with 45X/47XXX mosaic TS in the neonatal period. At the time of diagnosis, she received echocardiography and abdominal ultrasonography for evaluation of common complications in TS, and the results were all normal. She demonstrated several characteristics associated with TS, including high-arched palate, low hairline, low-set ears and shield chest, and she had been treated with GH replacement therapy for the past 3 years for management of her short stature. She did not have any family history of cardiovascular disease. Her father's height was 176 cm, her mother's height was 164 cm, and the midparental height was 163.5 cm. Her height was 133 cm (between the 10th and 25th percentile) and body weight was 31.7 kg (between the 25th and 50th percentile). During the 3 years of GH replacement therapy, her height increased by as much as 16.5 cm, with a growth rate of 5.5 cm/yr.
On presentation, her blood pressure was 97/49 mmHg and heart rate was 100 bpm with a regular heartbeat. Cardiac and chest auscultation were normal, and peripheral pulses were symmetric and strong. She did not have a fever or any other signs of infection or inflammation.
Her mental status was alert and there was no evidence of increased intracranial pressure. Motor function decreased to grade 3 in the left lower leg, and to grades 4-5 in right lower leg. She showed decreased dorsiflexion of the left great toe, but plantar flexion was intact. Lower leg sensation and deep tendon reflexes of both knees also were intact.
Results of routine blood examination and coagulation tests were all within normal range. To rule out infection or inflammation, several viral studies of blood and cerebrospinal fluid were performed, and the results were all negative. Serum levels of insulin-like growth factor-1 and insulin-like growth factor binding protein-3 were 487.04 ng/dL and 72,000 ng/dL respectively, which were both within normal range according to her age.
Echocardiography showed no abnormalities. Brain computed tomography performed in the emergency department also showed no abnormalities. Therefore, we performed whole-spine magnetic resonance imaging (MRI) and spinal angiography (, ). On the whole-spine MRI, T2-weighted images showed acute intradural extramedullary spinal hemorrhage and suspicious vascular malformation at T 11-12. On the spinal angiography, there was no remarkable evidence of spinal artery aneurysm or tumor staining.
She was treated with intravenous high dose dexamethasone (0.6 mg/kg) for 6 days, and gradually recovered motor function of both lower legs. On hospital day 6, she underwent rehabilitation for standing and walking. On hospital day 9, she could walk without assistance. We tapered the dexamethasone dose over 3 days, and then we changed to oral prednisolone (0.3 mg/kg). On hospital day 13, her motor function was grade 5 in the right lower leg and grades 4-5 in left lower leg. At that point, she was discharged. Follow-up spine MRI performed 7 days later, showed resolving process of the subacute intradural extramedullary hemorrhage and the still existing cavernous malformation at T 11-12 (). After the discharge, she had no problems in walking or standing, and she has been regularly followed up in our outpatient clinic every 6 months. There have been no more emerging symptoms up until her last visit.


An 18-year-old boy was referred to the endocrine centre at the Sher-i-Kashmir Institute of Medical Sciences, Kashmir for the evaluation of suspected adrenal insufficiency. He was born full term, from a consanguineous marriage should be omitted to a native Kashmiri couple. His younger brother had died at the age of 1 year with possible adrenal insufficiency and his two other younger siblings were healthy ().
The patient reported generalised weakness, asthenia, fatigue, anorexia and progressive hyperpigmentation of skin for 6 months. Examination revealed a lean build patient (body mass index, 12.64 kg/m2), with hyperpigmentation of tongue, buccal mucosa and skin and a nasal twang to voice. His blood pressure was 100/60 mm Hg with postural drop. Neurological examination revealed wasting of thenar and hypothenar muscles (). The deep tendon reflexes were hyperactive and the sensory examination was unremarkable.
Baseline investigations revealed normal complete blood counts, serum creatinine and normal electrolytes (serum Na, 142 mmol/L; serum K, 4.1 mmol/L). Basal serum cortisol (8:00 AM) was very low (0.41 µg/dL) and failed to increase after intravenous injection of 250 µg of tetracosactin (30 minutes and 60 values of 0.47 and 0.45 µg/dL, respectively). The plasma adrenocorticotropic hormone (ACTH) levels were markedly elevated (1,262 pg/mL; normal range, 9 to 52 pg/mL). A diagnosis of primary adrenal insufficiency was established. A contrast enhanced computed tomography (CT) of abdomen failed to visualize adrenals indicating atrophic adrenals. The tuberculin test was negative.
The patient had significant past history. He initially presented to ophthalmologist at the age of 8 years with absence of tears. Schirmer test was suggestive of alacrimia. At that time he had no other complaints and was prescribed artificial tears. Four years later he presented with dysphagia especially to liquids. Barium meal examination revealed persistent narrowing in the distal region of esophagus near gastroesophageal junction. A diagnosis of achalasia cardia was made which was managed with esophagomyotomy. He had again presented at the age of 15 years with severe muscle wasting of thenar, hypothenar muscles and small muscles of feet. Nerve conduction tests revealed axonal peripheral neuropathy.
On the basis of primary adrenal insufficiency (with normal mineralocorticoid balance), alacrimia, achalasia cardia, and peripheral neuropathy a diagnosis of Allgrove syndrome was established. We have not performed the genetic study about the mutation of AAAS gene. A negative tuberculin test together with absence of adrenal calcification on abdominal CT made tuberculosis unlikely as the basis for adrenal insufficiency in our patient. He was started on oral hydrocortisone tablets 15 mg after breakfast and 10 mg at 6:00 PM. Patient reported a striking improvement on follow up at 4 weeks.


An 88 year old 63 kg woman, presented to the University of Arizona Medical Center after she tripped and fell at her home. She complained of left hip and wrist pain. Patient denied any past medical or surgical history. She was not on any medications and had no known drug allergies. Physical examination revealed tenderness on palpation of the left hip with deformity and tenderness over the ipsilateral wrist. Radiographs confirmed a nondisplaced left intertrochanteric femur fracture and an ipsilateral dorsal bending distal radius fracture. She was neurovascularly intact in bilateral upper and lower extremities.
Informed consent regarding closed reduction and splinting of the distal radius fracture were discussed with patient and family, all consenting. The 22 gauge needle was inserted dorsally 3 cm proximal to fracture site, at a 30 degree angle. The needle was advanced to the fracture site once the skin was penetrated. A flash of blood was present. Over a thirty second interval, a 20 mL mixture of lidocaine 1% and bupivacaine 0.25% was infiltrated into the fracture hematoma. Neither anesthetic contained epinephrine. Within 15 seconds following the completion of injection, the patient became difficult to arouse, responding only to sternal rub. She developed mild tonic clonic movements of her upper and lower extremities lasting 15 seconds. Vital signs remained stable. Electrocardiogram showed no abnormalities. Approximately one minute after completion of injection, patient began responding verbally. At approximately 5 minutes post injection, patient was awake, alert, and oriented. She did not recall the events surrounding the injection. She underwent closed reduction of her distal radius fracture and a sugar tong splint was applied. She was admitted to the medicine service, underwent cephalomedullary nail fixation of her femur the following day, and was discharged from the hospital to inpatient rehabilitation on hospital day 4. Upon 2 and 6 week follow up, she had no neurologic sequelae.


Thirty six year old unemployed male patient presented with swelling of right leg that was gradullay increasiung since last two years. there was no history of significant trauma and the general status was good. Clinical examination revealed a firm steady swelling, tender on palpation, sitting in the antero lateral face of the leg. It measured 15 cm in its long axis. There was no collateral venous circulation and skin appeared normal. The x-ray of the leg showed a diaphyseal cortical erosion image associated with an aspect of soft parts densification (). Computer tomography examination revealed a tumor process with involvement of the medullary cavity and extending to the anterior muscle (). Furthermore, abdominal ultrasound and chest radiography revealed no abnormal findings. The histological examination after biopsy revealed a bone adamantinoma. The patient was reviewed two months later and had an overall swelling distorting the whole leg associated with severe pain.
Significant lysis and a pathological fracture of the tibia were discovered on x-ray (). Radical treatment was indicated and amputation in the upper third of the leg was done. Histological analysis of the lesion confirmed the biopsy result by showing a tumor proliferation made of lobules with palisade edges and large fibrous’s focus rich in vessels. The immediate postoperative course was uneventful and the patient was regularly followed. The evolution was marked by the installation of a massive pleurisy and worsening of the general status leading to death in 17 months.


A 42 years old female presented with bilateral elbow swelling since 3 yrs. She suddenly noticed small pea sized swelling with pain on right elbow 3yrs back and after 2 months started on left side. Both swellings gradually increased to present size of a golf ball ( & ). Swellings were painless but associated with minimal restriction of elbow flexion movement. She had no history of trauma, micro trauma, fever, discharging sinus, other joints involvement and renal stone. On clinical examination a solitary mobile globular 5cm x 5cm in size non tender soft to firm in consistency without any signs of inflammation present over the posterior aspect of both elbows. Elbow joints showed no evidence of effusion or instability. Elbow movements were painless with terminal 15* restriction of flexion. All other joints were clinically normal. Details of investigations as per following : She underwent open bursectomy. The bursa was excised in toto;() a careful examination during surgery did not show any pedicle or communication with the elbow joint and specimen sent for histopathological examination ( & ). On histological examination, showed fibrocollagenous tissue with focal collections of chronic foam cells, congested vessels and haemorrhage, multiple irregular deposits of positive birefringent calcium pyrophosphate crystals with surrounding granulomatous lesion (Fig , ). On post operative follow up patient has painless full range of movements and wound healed without complications and patient satisfied ( & ). She was started on cholchicine to prevent another attacks or recurrence. Follow-up after 1 year shows full range of movements and x-rays were done shows no calcification.


An eight years, ten months old girl was referred to the Orthopedic Clinic of the University of Catania, Italy for the diagnostic work up of her scoliosis. She was the third child of healthy, unrelated Italian parents. At the gestation, the mother was aged 31 and the father 33 years. A sister and a brother aged 4 and 2 years, are healthy and do not show hairy abnormalities at their clinical examination. The mother felt normal fetal movements during gestation and the pregnancy was uneventful. The mother denied having had any infections or having used folic acid, any drugs or alcohol during pregnancy and specifically any topical or systemic drugs which can cause hypertrichosis.
The girl was born at 39 weeks gestation by cesarean section with breech presentation. The birth weight was 2700 g, height 49 cm and head circumference 35 cm. The Apgar scores were 8 and 10 at 1 and 5 minutes, respectively. The mother referred to have noticed in the girl a tuft of hair in the thoraco lumbar region a few days after birth and the hairy at the elbow level at the age of 3-4 years. The scoliosis was noticed at the age of 8 years during a scholastic medical checkup.
At the physical examination the girl’s general conditions were good, her weight was 24 kg (25rd centile) and height was 138 cm (50rd centile). During the examination for the scoliosis a tuft of hairs was seen in the thoraco lumbar area (). At the same time abnormal hairiness was noticed at the elbow from mid humerus to mid forearm bilaterally (). The hairs were thin, lanuginose and fair colored and reached the length of 12-20 centimeters. A thoraco lumbar right convex scoliosis was present. The big toes were large () with recurrent drug resistant infections in the left big toe. Scholastic performance was good. Routine blood and urine analysis and full metabolic and endocrinologic screening yielded normal results. Abdominal ultrasound was also normal. Histological studies of hair taken from affected areas yielded no anomalies with most of the hair follicles in the anagen phase. No abnormalities were noticed at the brain-MRI. Spine MRI disclosed a wide dilation of the ependymal channel from Th1 to medullar cone with segmentary syringomyelic cavitation at the level Th1-Th4 and at the Th12-Ll lumbar level with liquoral signal (-). Normal the intersomatic spaces. Feet X-Ray showed large big toes with absence of bone lesions. The karyotype of the patient was normal (46, XX).


In Nonsan-si, Chungcheongnam-do, a gordian worm was passed in the feces of a cat (5-month old, male) which was alive and wiggling vigorously at that time (). The worm was placed in lacto-phenol solution (glycerin 20 ml, lactic acid 10 ml, phenol 10 ml, and distilled water 10 ml) for 24 hr and tentatively identified under a light microscope.
For scanning electron microscopy (SEM), the pieces of parasite was washed 5 times with 0.2 M cacodylate buffer (pH 7.3) and fixed in 2.5% glutaraldehyde, post-fixed in 1% osmium tetroxide at 4˚C. The specimens were then dehydrated in a graded ethyl alcohol series, dried by a CO2 critical point dryer, coated with osmium, and examined by a SEM (S-4800, Hitachi, Ibaraki, Japan) at an accelerating voltage of 15 kV.
For the DNA extraction and amplification, the genomic DNA was extracted from the gordian worm by using a DNeasy® Blood and tissue kit (Qiagen, Alameda, California, USA) according to the manufacturer’s instructions. Several sequences from Gordius albopunctatus, Gordius aquaticus, and Gordius paranensis were aligned using Multiple Sequence Alignment (Clustal Omega, ), and then common Gordius primers were designed using the online tool (Primer3input, ). An 18S rRNA region spanning internal transcribed spacer 2 (ITS2) gene (DNA) was amplified for genomic DNA sequence using PCR. The oligonucleotide sequences of primers employed to detect Gordius 18S rRNA region spanning ITS2 gene (DNA), were 5´-GTCGTAACGGGTAACGGAGA-3´ (forward) and 5´-TTTCGGACCAGGAGAATGAC-3´ (reverse). The primer set were designed around 1,165 bp product, variable for all sequences aligned. Under standard condition such as 95˚C for 30 sec, annealing at 60˚C for 30 sec, and extension at 72˚C for 1 min, PCR reaction was performed in a MyCycler Personal Thermal Cycler (Bio-Rad Laboratories, Hercules, California, USA) using EmeraldAmp GT PCR Master Mix (Takara, Shiga, Japan) with 1 μl DNA aliquot. The PCR products were next visualized via electrophoresis on 1.2% agarose gel, and then purified using QIAquick PCR purification kit (Qiagen).
For the PCR sequence analysis, amplifications were directly sequenced using ABI Prism Big Dye terminator (v. 3.0) ready reaction cycle sequencing kits (Applied Biosystems, Foster City, California, USA) with the same primers as those used in PCR. The sequencing reactions were run on a PE Applied Biosystems 3100 automated sequencer. The sequence data were aligned by Clustal Omega program (clustal O 1.2.1). A phylogenetic tree-based sequence analysis was conducted by neighbor-joining using the blast tree program ().
In gross findings, the body was 19.5 mm in length and 1.0 mm in diameter (), and its color was light brown. In more details, the anterior end (calotte) was rounded with dark color, and the mouth was presented at the center (), but the white tips were not found on the cuticle. The posterior end was bi-lobed with blunt lobes, with each lobe about twice as long as wide (). The cuticle was smooth and slippery.
Using SEM analysis, the cuticle structure was not shown as the elevated (increased or high) cuticular areoles on the surface of the body, but the spines on the cuticles of the anterior portion were sseen (-). While hairs were found on the anterior portion, it was difficult to find spines in the mid-body, as shown in and . The cuticular areoles appeared slightly on the anterior portion. The distinct crescentshaped fold was located behind the cloaca, and the inverted V-shaped ridge containing some bristle was anterior to the cloaca (). Interestingly, male tail lobes were 2 times shorter than their diameter. The post-cloacal crescent was semicircular, with the width of about 200 µm, which was about 50% of the width of the posterior end at the level of the crescent (). During the tail lobes branched directly behind the postcloacal crescent, the bristles on the tail lobe were rare. The arms of the crescent do not extend onto the tail lobes ().
To identify the genotype of the used Gordius specimens, its genomic DNA was analyzed by the sequencing method. The ITS2 region of 18S rRNA in Gordius specimens was identified and compared with that of G. albopunctatus (U99337.1), G. aquaticus (X80233.1), and G. paranensis (AF421766.1), respectively. As a PCR amplicon, partial ITS2 sequences (1,097 bp) of the Gordius specimens contained 38 variable sites of common sequence in which belonged to the same clade as G. albopunctatus, G. aquaticus, and G. paranensis, respectively. The sequence of Gordius specimens was shown with high similarity in G. albopunctatus (97.6%), G. aquaticus (97.6%), and G. paranensis (97.1%), respectively. Furthermore, partial ITS2 sequences of the Gordius specimens reveal the low gap difference of nucleotide in G. albopunctatus (0.7%), G. aquaticus (1.0%), and G. paranensis (0.8%), respectively. Although 5 DNA regions of Gordius specimens was not matched with above sequences of the 3 Gordius sp., the identification was also supported by the fact that they belonged to the same clades as Gordius in the phylogenetic tree based on the used ITS2 rRNA gene. When the sequence of Gordius specimens was compared with genes from several horsehair worms, the sequence data was inferred using the neighbor-joining method. The sequence of Gordius specimens was identified as a species that is genetically close to the Gordius species (U88337.1, X87985.1, X80233.1, AB470227.1, AF421766.1, U51005.1, AY863409.1), Paragordius species (AY428819.1, AF421771.1, AF421770.1, AF421769.1), and Chordoes species (AF421763.1, AF036639.1) ().


A 75-year-old man reported with the blurring of vision in both eyes. He was known to suffer from PXF and had undergone standard uneventful phacoemulsification with in-the-bag implantation of monofocal IOL in both eyes. In the right eye, a single piece poly (methyl methacrylate) (PMMA) IOL (+19 D) and in the left eye, a single piece acrylic foldable IOL (+19 D) were implanted. The surgeries were performed in the right and left eye at 9 and 8½ years, respectively, before to this examination. All the previous dilated annual eye examinations had been normal, with no clinical evidence of IOL subluxation.
On clinical examination, his visual acuity was 20/60 in both the eyes. Slit lamp examination revealed anterior partial in-the-bag IOL dislocation [Figs. and ], capsular contraction and deformation of IOL haptics. Intraocular pressure by Goldmann applanation tonometer (GAT) was 19 mmHg in both eyes. Pupils were dilated with 2.5% phenylephrine eye drops. Indirect ophthalmoscopy revealed normal ocular fundi. Constriction of pupils and IOL repositioning was tried by instillation of 2.0% pilocarpine eye drops in the supine position, but the procedure was unsuccessful. The next day, IOL along with the capsular bag were explanted surgically, [] and secondary scleral suture fixation of single piece PMMA IOL (Auro India) was done in the same sitting for the right eye. The procedure was repeated for the left eye 7 days later. The IOLs were held with micro forceps and explanted through the limbal route. Limited anterior vitrectomy through pars plana route was also performed. The secondary IOL implantation procedures and postoperative period were uneventful. After 12 months of surgery, best corrected visual acuity was 20/30 in both eyes. The manifest refraction was ±1.00 DC × 180° and +1.00 DS/−1.50 DC −180° in the right and left eye, respectively. The eyes were quiet and IOP was 19 mmHg (GAT) in both eyes.


A 68-year-old female, right hand dominant, slipped on ice and fell on her left outstretched hand. There were no open wound, no neurovascular deficit and no other significant injuries. The skin was not at risk. Standard x-rays of the shoulder girdle showed a minimally displaced lateral fracture and a second displaced medial fracture of the left clavicle. A conservative treatment was proposed with regard to the patient’s age and her low functional demand. A week later the assessment was completed by a computed tomography (CT) scan which underlined a displaced medial fracture with shortening of the clavicle bone (). This statement drove us to propose a final fixation of the clavicle by means of two locking plates. The hardware used consisted of two 3.5/2.7 locking compression (LC) anatomical plates with a lateral extension (DePuy Synthes®, United States). A left sided plate was used for the lateral fracture and placed cranially while a right sided plate was used for the medial fracture and placed anteriorly. The patient was operated in beach chair position under general anesthesia. Antibiotic prophylaxis was given at the induction. A longitudinal anterior incision was used. The reduction of the medial side of the intermediate segment of the clavicle was particularly difficult due to the small medial fragment still attached to the sternoclavicular joint, and the rupture of the costoclavicular ligament and the lever arm produced by the coracoclavicular ligaments still attached to the lateral side of the intermediate fragment. Fixation of the lateral fracture facilitated the reduction and fixation of the medial fracture. Three 2.7 mm locking screws were placed in the medial fragment and stabilized the sternoclavicular joint by means of a transosseous non-resorbable suture of #2 FiberWire (Arthrex®, United States) running through two holes of the extension of the right sided plate placed anteriorly and through two tunnels of 2 mm of diameter drilled in the manubrium. Early passive and active mobilization of the left shoulder was begun from the second postoperative day. A sling was used for six weeks as antalgic support and the abduction of the left arm was limited to 90° during this period because of the poor quality of the clavicle bone and the relative stability of the fixation of the medial fracture. After six weeks the patient was practically pain free and presented a limitation of the range of shoulder motion. After three months the patient was able to completely move her left shoulder. After six months bony consolidation of the clavicle was achieved (). After the last control (at 12th months) the Disabilities of the Arm, Shoulder and hand (DASH) score was 5.0 and there was no significant hardware irritation.


A 54-year-old menopausal woman was referred due to long-standing intense itch, anatomically coinciding with hyperpigmentation of the upper right back (). The patient reported constant itch, rated 8~10 on a visual analog scale (visual analogue scale [VAS], 0~10; 0 denoting "no itch" and 10 denoting "worst imaginable itch") and severe, consistent sleep difficulties due to frequent nocturnal bouts of itching.
Previous unsuccessful attempts to treat her NP-induced CP included topical treatment with antifungal, corticosteroid, tacrolimus 0.1%, capsaicin 0.025%, and doxepin 5% creams. Only topical doxepin was shown to provide some relief (2~3 hours duration), but did not effectively relieve nightly bouts of itch. Histological examination of a 3-mm punch biopsy confirmed the presence of postinflammatory hyperpigmentation. The area of alloknesis was identified, which corresponded with the area of hyperpigmentation, except for a 2~4 cm border area with little or no hyperpigmentation.
After informed consent was obtained, the patient was treated with a topical 8% capsaicin patch for 1 hour. The treatment resulted in 7 weeks of complete itch relief, upon which a slow but steady reversal began. During re-examination and retreatment 3 months after the initial treatment, the patient reported that the returned CP was once again highly bothersome and sleep disruptive, but not entirely to the same extent as before the initial treatment. Of note, the intensity but not the area of the hyperpigmentation and the area of alloknesis to the nylon filament stimuli had diminished after the first treatment. Subsequent treatments with capsaicin 8% had comparable efficacy. By the third treatment, the area of alloknesis had retracted by approximately 15%~25% ().


A 49-year-old woman presented with itching and symmetrical bilateral hyperpigmentation below and medially to the scapulae (). She stated that the condition had emerged 5~6 years previously and reported 4~6 daily itch exacerbations, the itch intensity of which she rated at VAS=9. The patient also expressed concerns regarding the implications of the discoloration on her aesthetic appearance. The patient had undergone topical and systemic antifungal therapies in previous unsuccessful attempts to treat her pruritus. A biopsy revealed postinflammatory hyperpigmentation. The area of alloknesis slightly exceeded the area of hyperpigmentation.
After obtaining informed consent, the patient was treated with a topical 8% capsaicin patch for 1 hour. At follow-up, the patient reported that her itch had disappeared completely for 1 week, and thereafter very slowly receded toward baseline levels. Despite this slow reversal, the patient reported that, at the 3-month follow-up/retreatment, her average itch intensity remained lower compared to her pretreatment baseline levels of itch. At retreatment after 3 months, both the hyperpigmentation and the area of alloknesis had decreased considerably.


A 45-year-old woman was referred due to moderate itch of the upper back estimated to have persisted for approximately 20 years. The patient reported a constant itch rated at 3 on a VAS (0~10) and 1~3 daily itch exacerbations, the intensity of which she rated as 6~9. She reported experiencing typically two nightly itch-and-scratch bouts lasting several minutes each. She was taking no concomitant medication.
Inspection revealed no visible skin alterations in the area where the itch was perceived — medially to the left scapula (). The area of alloknesis could be identified using a 10 g nylon filament. After informed consent was obtained, the patient was treated with a topical 8% capsaicin patch for 1 hour. At follow-up, the patient reported that the itch had subsided completely for 2 days but thereafter rapidly returned to baseline levels. She underwent one additional treatment 3 months after with a similarly subpar treatment outcome.


An 81-year-old woman underwent excision of a BCC in the left ala nasi. Following resection of the cancer, a perforator from the LNA was used to reconstruct the defect. A 180° rotation island flap 2 cm in width and 5 cm in length was designed. The aesthetic unit was restored. The location of the perforator corresponded to our cadaveric study. No complications were noted ().


An 83-year-old man underwent excision of a BCC in the left nasal sidewall. The defect was covered with an LNA V-Y perforator flap. Two arteries and two venous perforators were included in this flap. The cranial part of the flap was thinned to the level of the subdermal plexus to improve the aesthetic restoration of the nasal sidewall unit (). The location of the perforators corresponded to that identified in our cadaveric study.


A 70-year-old man underwent excision of a BCC in the right nasal sidewall. The defect was covered with an LNA V-Y perforator flap. Two perforators were included in this flap (). The location of the perforators corresponded to that identified in our cadaveric study.


A 31 year old female with a history of two cesarean sections, the last one of them three
years prior, presented with a painful mass in the left lateral side of the Pfannenstiel
incision which had been steadily growing. The same patient went a month ago to our
institution's urology clinic with pain in the inguinal region. After a detailed history
and physical examination an ultrasonography was performed that revealed a 20X12 mm
hypo-echoic nodular mass, neighboring the rectus abdominis muscle, with minimal
vascularization. The differential diagnosis included endometriosis and a possible
desmoid tumor. The patient was referred to our clinic for further evaluation. The pain
started on the left lower quadrant and radiated towards the inguinal region, and was
associated with menstruation. In physical examination a 2 cm wide mass was palpated in
the previously described localization. Magnetic resonance imaging was performed which
revealed a 20x11 mm mass which is slightly hyper-intense in the T1 sequence, and
contrast enhanced after IV gadolinium injection in the T2 sequence, with increased
signal intensity and nodular appearance in diffusion weighted sequences. These were
found to be consistent with an endometrial implant ( and ). Examination of the
uterine cavity showed effusion which was at most 15 mm in width when measured. A little
free fluid, indicating peritoneal irritation was present in between the intestinal
loops. No pathological lymph nodes were present in the lower abdomen.
The patient was admitted for surgical removal of the mass. A 2 cm wide fibrotic
appearing mass was excised () and was sent
for pathological examination. The patient's complaints resolved after the surgery. She
was discharged with no complications two days after the operation. Four months after the
surgery, the patient came in for a follow-up visit, and had no complaints or
complications. Histopathological examination was consistent with glandular structures of
the endometrium with accompanying endometrial stroma within muscle and connective tissue
().


A 37-year-old female presented with fatigue, fever, weight loss and loose stools for 3 months duration. On examination, few enlarged lymph nodes were found in the left lower cervical and left supraclavicular regions. Biopsy of left cervical lymph node was done, and histopathology by haematoxylin and eosin (H and E) staining revealed Reed-Sternberg (RS) cells with CD30 expression on IHC, suggestive of classical HL. Hence, the patient was referred for 18F-FDG PET/CT for initial staging. PET/CT revealed multiple enlarged hypermetabolic lymph nodes on both sides of the diaphragm involving left lower cervical, left supraclavicular, abdomino pelvic nodes including gastrohepatic, perigastric, periportal, portocaval, peripancreatic, bilateral renal hilar, para aortic, aorto caval, mesenteric, bilateral common iliac and bilateral external iliac regions. There were also extranodal hypermetabolic omental thickening and perihepatic peritoneal deposits along segments IVa, IVb, VIII and porta of liver. In addition, metabolically inactive thin-walled cysts were seen in bilateral adnexae with a metabolically active soft tissue density lesion in the right adnexa. There was free fluid in the pouch of Douglas. Images also revealed diffuse bone marrow (BM) hypermetabolism in the axial and appendicular skeleton with few focal areas of relatively increased FDG uptake in the posterior column of left acetabulum, left ischium as well as metadiaphyseal and intramedullary regions of left femur []. The pattern of metabolically active lesion with peritoneal, mesenteric and omental disease were quite unusual for the given diagnosis of HL, and hence based on the imaging appearances, possibility of NHL was considered. However, in view of the metabolically active soft tissue density lesion in the right adnexa with free fluid in the pouch of Douglas, a second possibility of ovarian malignancy was also considered. Histopathology was reviewed with additional IHC markers that showed negativity for PAX-5 and positivity for CD4, confirming the diagnosis as ALK-1 negative anaplastic large T-cell lymphoma [Figure –]. BM biopsy (by H and E staining) from the left iliac crest did not reveal any evidence of lymphoma infiltration []. The patient was therefore treated with hyper cyclophosphamide, vincristine, adriamycin and dexamethasone (CVAD) regime. Interim PET/CT after two cycles of chemotherapy showed a very good partial response to therapy [].


A 41-year-old HIV-positive man was admitted to our hospital because of fever and cough since 5 days. His medical history revealed intravenous drugs addiction, chronic renal failure and hepatitis C infection. He was started on HAART based on abacavir, lamivudine and efavirenz during the last year. On examination the patient was oriented. His temperature was 37°C, pulse rate of 100 beats per minute and 20 breaths per minute. Chest examination did not reveal rhonchi or crackles. No focal neurological deficit was evident. Relevant laboratory findings showed an hemoglobin level of 7 g/dl, total white cell count of 3,700 cells/mm3 and a normal platelet count. Renal dysfunction was evidenced by serum urea of 47 mg/dl and creatinine of 1.8 mg/dl. Ocular fundus examination was normal. The liver function test and lactate dehydrogenase level (LDH) were normal. A chest radiograph showed bilateral interstitial infiltrates []. Staining and culture of sputum samples were negative for bacteria, mycobacteria and fungi. Blood cultures for the same microorganisms were also negative. The CD4 T-cell count was 35 cells/µl and the plasma viral load was 203,906 copies/ml. Serologic testing revealed the presence of T. gondii IgG antibodies with a positive titer of 1:1024 and IgM negative by indirect immunofluorescence.
Fever and cough continued 7 days after admission to the hospital. On the eighth day a bronchoalveolar lavage (BAL) was performed. Cytological and microbiological examinations of the BAL were negative for bacteria, fungi, Pneumocystis jirovecii and Mycobacterium tuberculosis. Giemsa-stained smears of the BAL were negative for tachyzoite forms of T. gondii. An aliquote of the BAL fluid was assayed for T. gondii using the PCR technique for amplification of a fragment corresponding to the B1 gene. BAL specimens were positive by PCR for T. gondii. Treatment for T. gondii infection was initiated with sulfadiazine, pyrimethamine and folinic acid. Computed tomography (CT) scan of the brain showed marked cerebral atrophy without evidence of focal lesions.
He showed a marked improvement in clinical, radiological, and laboratory findings after the seventh day of therapy for T. gondii. He was discharged from the hospital after 15 days of admission in a good clinical condition.


A 33-year-old male was admitted with fever and cough since 7 days. He was intravenous drug user and had been diagnosed as HIV seropositive 10 years before. He had history of cerebral toxoplasmosis 6 months before. He was discharged from the hospital with improvement in clinical and radiological finding after treatment. The patient was not adhering to treatment with HAART and secondary prophylaxis for T. gondii infection. On admission, he was febrile (38°C), pulse rate of 100 beats per minute and 18 breaths per minute. Chest examination reveals rales in the right lower lobe of the lung. No signs or focal neurological deficit was evident. Laboratory results showed a white blood cell count of 8,600 cell/mm3, an hemoglobin level of 12 g/dl and a normal platelet count. The liver and renal function tests were normal. The LDH level and the ocular fundus examination were also normal. A chest radiograph showed pleural effusion in right pulmonary lobe []. CT scan of the chest showed pleural effusion in the right lung []. Staining and culture of sputum sample were negative for bacteria, mycobacteria and fungi. Blood cultures for the same microorganisms were also negative. The CD4 T cell count was 62 cells/µl. Serologic testing revealed the presence of T. gondii IgG antibodies with a positive titer of 1:1024 and IgM negative by indirect immunofluorescence. Empiric treatment was initiated with Ampicilin plus sulbactam. On the fifth day fever and cough continued. A BAL was performed. Cytological and microbiological examinations were negative for bacteria, fungi, P. jirovecii and M. tuberculosis. Giemsa-stained smears of the BAL were negative for tachyzoite forms of T. gondii. A BAL fluid sample was assayed for T. gondii using the PCR technique for amplification of a fragment corresponding to the B1 gene. BAL specimens were positive for T. gondii. Samples from blood were also positive for T. gondii by the PCR technique. CT studies of the brain showed a left temporoparietal periventricular lesion with mass effect on the middle-line structures [].
Treatment for T. gondii infection was initiated with sulfadiazine plus pyrimethamine and folinic acid. In the following days the clinical condition improved. He was discharged from the hospital after 30 days in a good clinical and neurological condition.


A 24-year-old healthy male presented to King Khalid Eye Specialist Hospital, Riyadh, Saudi Arabia, complaining of blurred vision in his right eye for 1-week. Best corrected visual acuity was 20/70 in the right eye and 20/20 in the left eye. Intraocular pressures were normal bilaterally. In the right eye, there were no keratic precipitates and the anterior chamber was quiet with no cells or flare. Vitreous cavity was quiet and clear. Clinically, evident exudative retinal detachment was visible on dilated fundus exam and documented with optical coherence tomography (OCT). The exudative retinal detachment included the macula with deep creamy yellow choroidal infiltrates adjacent to an old chorioretinal scar along the superior temporal arches. The left eye was unremarkable. Fluorescein and indocyanine green angiography revealed two areas of early hypofluorescence at the choroidal level, which persisted to the final phase with surrounding hyperfluorescence on fluorescein angiography. Late indocyanine green showed defined areas of hypofluorescence corresponding to a scar and recent choroidal ischemia []. IgG and IgM were positive for toxoplasmosis.
Based on the clinical presentation, fluorescein angiography, OCT, and serology, the patient was diagnosed with atypical ocular toxoplasmosis with exudative retinal detachment and choroidal ischemia. The patient was managed with Bactrim and clindamycin for 6 weeks. Oral prednisolone 1 mg/kg was initiated after 48 h of antiparasitic therapy.
Three months after initiating treatment, the clinical presentation improved with complete resolution of subretinal fluid and restoration for vision to 20/20. 6 months from initial therapy, vision remained 20/20 with complete remission of signs and symptoms [].


A 38-year-old male patient, diagnosed with retroviral infection and disseminated tuberculosis presented with weakness and numbness of the right hand of 2 weeks duration. He also noticed an edematous reddish raised lesion on the inner aspect of right hand, which he attributed to the topical agent he used for relieving the numbness. Pain was present on the right elbow and forearm. The patient had been on antitubercular therapy (ATT) followed by antiretroviral therapy (ART), 5 months prior to presentation at our department. The CD4 count was 67 cells/μL during initiation of HAART.
Dermatological examination showed a single well-defined erythematous plaque, 20 × 5 cm with raised borders on the ulnar aspect of right hand and distal forearm, extending to the palm [Figures and ]. A satellite lesion was present on the upper part of forearm, which also showed erythema and edema. All modalities of sensations were lost in these lesions. Both ulnar nerves were thickened with tenderness on the right side. Slit skin smears from the earlobe, lesional, and normal skin were negative. Histopathological examination showed dermal edema, diffuse granulomas composed of Langhans type giant cells, surrounded by epithelioid cells, lymphocytes, multinucleated giant cells, and fibroblasts with perivascular lymphocytic infiltrate, consistent with Hansen's disease borderline tuberculoid type, with features suggesting type 1 lepra reaction [Figures -]. AFB stain was negative. CD4 count after 5 months showed a rise to 160 cells/μL. The viral RNA load was not measured due to lack of facilities.
Thus correlating the clinical, bacteriological, and histopathological features, a diagnosis of Hansen's disease borderline tuberculoid with type 1 lepra reaction presenting as IRIS was made. MB-MDT was started, avoiding rifampicin from the regimen, as patient was also on ATT. HAART was continued and systemic steroids were also given in tapering doses. On follow up there was marked alleviation of his symptoms. However, the patient succumbed within 3 months to a brain tuberculoma.


A 58-year-old woman with 15 years history of insulin-dependent diabetes presented with nonhealing ulcers on her shin since 5 years. Five years ago she noticed redness and thickening of skin over her left shin. It was associated with mild itching. Four months later, she noticed a pinhead sized lesion over the red area, which enlarged and ulcerated. Three similar ulcers [Figure and ] appeared on the shin over the next one month. The ulcers were non healing and extremely painful but not associated with any constitutional symptoms. Local wound care, topical corticosteroids, topical calcineurin inhibitors were tried along with oral antibiotics, dapsone, pentoxyphylline, and analgesics, without much relief. She continued on insulin and oral antidiabetics with good glycemic control. On examination she had multiple discrete tender ulcers over a waxy, erythematous, atrophic plaque on the anterior aspect of her left leg, variying in size from 3 × 2 cm to 1 × 1 cm. The borders of ulcer were undermined, base was indurated, and floor was covered with necrotic debris. Her general and systemic examination results were within normal limits. On investigation her hemogram, renal and liver function tests, and venous Doppler test results were normal. Fasting blood sugar was 120 mg/dL. Pus culture grew Staphylococcus aureus sensitive to linezolid, amoxycillin and clavulanic acid, and tazobactam–piperacillin. Nerve conduction studies did not reveal any abnormality. Skin biopsy from the edge of the ulcer revealed a hyperplastic, sclerotic epidermis and a dense superficial and deep dermal infiltrate of lymphocyte and plasma cells concentrated around the blood vessels and sweat glands [Figure -]. There were several granulomas arranged in a horizontal manner in mid and lower dermis. The upper dermis had numerous eosinophils scattered interstitially. No significant stasis changes were seen. The appearences were consistent with ulcerated NL. We started the patient on oral linezolid 600 mg twice daily for 7 days and chloroquine 250 mg once daily for 4 weeks along with local dressings. There was no change in ulcer size and pain was persistent. Topical human recombinant epidermal growth factor, oral clopidrogel and aspirin were added to the above regime and continued for another 2 months, with no improvement. Oral thalidomide 100 mg was started, with local wound dressings. Pain reduced dramatically within 2 weeks and the ulcers healed completely after 4 weeks [Figures and ]. Thalidomide was reduced to 100 mg once daily and continued for another 6 weeks. There was no relapse over a follow up period of 6 months.


A 45-year-old smoker male presented to surgery outpatient department (OPD) of a Tertiary Care Hospital in Eastern Bihar with a large ulcer on the scalp. According to history, the lesion started as a papule 15 years back which was ulcerated eventually. The ulcer gradually and slowly increased to reach the present size 5 years back. After that it remained stationary. The patient denied any kind of treatment or medical attention prior attending to our OPD. On examination, the ulcer was located on the right frontoparietal region. It had a size of 14 cm × 10 cm. The ulcer was well marginated with focal hyperpigmentation of the border. The ulcer base was covered with nodular growth []. There was no significant regional lymphadenopathy. Considering the long duration and morphology a provisional diagnosis of squamous cell carcinoma was reached. An incisional biopsy was obtained from margin of the lesion under local anesthesia and sent for histopathology.
Epidermis was edematous and necrosed at places with the formation of overlying crust []. The dermis was infiltrated with thin strands of basaloid cells interconnected with each other forming paisley tie appearance []. Peripheral palisading was maintained in most of the basaloid strands []. Tumor stroma was grossly sclerosed. In view of these findings, a diagnosis of an infiltrative variety of BCC with overlying ulceration was made. X-ray of the skull ruled out bony involvement []. Chest X-ray was also normal.
Before we could plan for future treatment modality, the patient was lost to follow-up.


A 40-year-old male patient of American Society of Anesthesiologists grade II with a diagnosis of the pseudopancreatic cyst was scheduled for elective exploratory laparotomy and gastrojejunostomy. After the induction and endotracheal intubation, a lubricated nasogastric tube of size 18 Fr was inserted blindly through the right nostril, but gets coiled in the oral cavity. So a next attempt was made with another 18 Fr nasogastric tube as previous tube get kinked. Resistance was encountered while advancing tube further, so laryngoscope and Magill forceps were used. But after some length it again offered resistance. For confirmation of correct placement of tube aspiration of gastric contents was done but negative and surgeon also couldn't palpate the tube in the stomach. We tried to pull it out, but couldn't as its distal end got stuck in the nasopharynx. We again moved it forward and tried to pull it out gently while rotating but all in vain. Then laryngoscopy was done, and a knot found which was then pulled out with Magill forceps []. It was a true knot at the distal end of the nasogastric tube. Knot was opened up, and the nasogastric tube was withdrawn. There was minimal trauma and bleeding in the nasopharynx. No other complications were seen. Postoperatively, the patient had no complaint and was discharged on the 7th day [].


A 12-year-old male child with a 3-month history of bilateral, constant, radiating lower limb, and back pain was brought to Institute Rotary Cancer Hospital (IRCH) by his father. The patient described the pain as an aching, throbbing, stabbing pain, and rated it a 9/10 on the Verbal Analogue Scale (VAS). He stated that he had not been able to walk or do any of his regular physical activities for a month. He had associated weakness and paresthesias in his legs bilaterally. He was unable to sleep at night for the past 2 weeks, due to back pain that was elicited by lying down. The patient also had associated constipation and urinary retention for which he had to be catheterized.
When the patient came to the pain clinic, he was held in his father's arms, as the patient was paralyzed from the waist down. On physical examination, the patient appeared to be in severe pain. Deep tendon reflexes were not present in bilateral lower limbs. Muscle strength was 2/5 on the Oxford Scale (muscle strength grading scale) in bilateral limbs (hip/knee/ankle). Sensation to pinprick and soft touch were diminished over the gluteal regions and legs. Upon palpation of the patient's legs, he felt electricity running through his legs. The patient denied any other symptoms.
In March 2015, the patient had sudden onset of excruciating left lower quadrant abdominal pain. In April 2015, he underwent an exploratory laparotomy and excision of a growth between the spleen and renal space. Pathology reports suggested the growth was a CD99 and vimentin-positive YST. Further tests were conducted such as an ultrasound to see if there was any involvement of the testes. Results showed no gonadal involvement.
The patient's father stated that the patient was attending school, running, and was pain-free until 15 days postoperatively. The doctors at his local hospital were unable to find the etiology of his postoperative pain. The patient was referred to a different hospital for consultation. A CT scan was done that showed a heterogeneous mass in the left suprarenal region, suggestive of a residual mass that was infiltrating the posterior abdominal wall and muscle. A whole abdomen contrast-enhanced computed tomography (nonionic CECT) revealed a devascularized left kidney possibly due to left renal artery injury. After various inconclusive testing, the patient was referred to IRCH (AIIMS.)
At the time of the patient's presentation at IRCH his condition had progressively worsened. He had developed bilateral paralysis, severe lower lumbar back pain, and bilateral leg pain. Many investigations were initiated which included complete blood count (CBC), liver function tests, complete metabolic panel, blood test checking for thyroid-stimulating hormone (TSH), beta-human chorionic gonadotropin (β-HCG), and alpha-fetoprotein (AFP) levels, comparison CECT scan, ultrasound of neck to check for lymph node involvement.
CBC showed hemoglobin was 10.4 (normal range: 13.5–17.5 g/dl for males), thin layer chromatography was 24.8 (normal range: 4000–11,000). Liver function tests showed that bilirubin levels were below the normal range at 0.2 mg% (normal range: 0.8–1 mg%), TSH and β-HCG were within normal limits, but AFP 3 was 0.52 ng/ml (normal range: 0.89–8.78 ng/ml). Ultrasound of the neck showed no sign of metastasis to lymph nodes or glands. The CECT scan was unchanged compared to the previous scan. The mass seen previously was causing erosion of the left 12th rib with extension into neural foramina with suspicious intraspinal extension. No metastatic lung nodules were visible.
Based on the patient's history, physical exam and investigations, the diagnosis of primary retroperitoneal extragonadal YST extending into the neural foramina of the spinal cord at L1/L2 was made. The metastasis was causing compression of the spinal cord producing his paralysis. For management of this patient the medical oncologist, surgical oncologist, and radiologist were consulted. Due to the extent of cancer, further surgery was no longer an option.[] Although the paralysis was not reversible, the pain, with the help of proper pain management, and palliative care team was treatable.[]
While interacting with the patient and his father, I could see that the patient's father was distraught about his son's condition. Medical treatments for the patient had taken a financial toll on the family. The patient's father had to sell off his land where he was farming in Patna, Bihar in order to pay for proper treatments. He expressed to me that he was willing to sell his house too if it meant we could make his son walk again. The patient's mother became severely depressed and threatened to commit suicide if her son's health did not improve. Despite explaining that at this stage only the pain of the patient could be managed and that the paralysis was permanent, the father was in denial. He kept saying, “Please make my son walk again, that is, all I want.” Family bonds are reasonably vulnerable during the time of distress, and the entire palliative care team provided a positive and supportive environment so that they could communicate their needs most effectively.
The patient was initially started on infusion morphine, which was dose titrated appropriately and later converted to tablet morphine.[] Dexamethasone was prescribed to reduce the swelling caused by the compression of the spinal cord.[] He was given gabapentin for the neuropathic pain, which was converted, to tablet pregabalin. Studies suggest pregabalin provides equivalent efficacy to gabapentin, however, at much lower doses.[] Because lower dosages can be used to treat neuropathic pain, it is likely that pregabalin will be associated with fewer dose-related adverse events such as dizziness, chest pain, difficulty breathing, and constipation.[] Pregabalin requires lower dosages, has a much higher bioavailability (90% vs. 33–66% of gabapentin) and is rapidly absorbed (peak: 1 h).[]
Morphine reduced the pain from 9/10 to 4/10 on the VAS.[] To further relieve the patient's pain, we discussed chemotherapy and attempting a nerve block.[] The father cited apprehensions for both of these treatment options. The patient's father was explained the positive impact the procedure would have on relieving his son's pain, and that his son would be able to sleep peacefully and be pain-free. He was told giving chemotherapy would not have reversed the neurological deficit but would have definitely reduced the pain by treating the cause. The father had seen many unfortunate situations about what can happen after chemotherapy is given and explained to us how he knew some people that had died in his village after chemotherapy was given. He was further explained all the benefits chemotherapy would have on not only the pain the patient was experiencing but also in slowing down the progression of metastasis. All his questions were addressed, and he was told that the situation in which other people might have received chemotherapy might be different than his son's. When explained how attempting a nerve block could be beneficial, the father again refused and said he heard it would permanently damage his son's nerves. The whole palliative care team made many efforts to make the patient's father understand exactly how these treatments would be given as well as how all the side effects would be managed for both the nerve block and chemotherapy, but the father was convinced that these will only cause harm to his son based on what he had seen and what people told him about these medical advances in his village. Even after multiple attempts of ensuring the father that this would be the next best option for his son's pain, consent was not given, so these treatments were not given.[] One of the major components of palliative care is respect for patient's autonomy.[] Due to lack of health literacy there are many proven, working treatments that are simply not done.[]
The patient's pain was managed based on the World health Organizations Cancer pain protocol (WHO ladder).[] This included the analgesics tablet Morphine (P) 10 mg three tablets 4 hourly, tablet paracetamol (acetaminophen) 500 mg one tablet QID and tablet pregabalin 75 mg HS × 6 weeks.[] Anti-emetics were given to avoid nausea and vomiting, the patient was experiencing.[] For GI ulcer prophylaxis tablet pantoprazole 40 mg once daily was prescribed. Tablet dexamethasone 4 mg TDS × 7 days was also given and later changed to 2 mg TDS × 7 days.[] After monitoring the patient's pain and bringing it down to a 3/10 on the VAS, the patient was counseled and sent to a nonprofit organization called Cankids (Cankids is a registered charitable national society working to make a change for childhood cancer in India.) There, he would receive further pain management treatments and psychosocial support through counseling for him and his family.


George, was a 23-year-old single, Caucasian male student in his last academic year and was referred to a University Counseling Centre in Athens. The Centre provides free of charge, treatment sessions to all University students requiring psychological support.
George was diagnosed with Social Anxiety Disorder and with Specific phobia, Situational Type i.e. acrophobia. He was living alone in Athens, as his parents live in a different region of Greece. He was an only child. When asked about his childhood, he said that he had been happy and did not report any traumatic events. He described a close relationship with both his parents and when asked, he did not report any family history of psychiatric or psychological disorders or substance abuse problems.
He complained of severe symptoms of anxiety and phobias during the last six months. He began experiencing severe heart palpitations, flushing, fear of fainting and losing control, when travelling by plane, when crossing tall bridges while driving or when being in tall buildings or high places, however he did not experience symptoms of vertigo. Additionally, he reported significant chest pain and muscle tension in feared situations. His fear of experiencing these symptoms worsened and led him to avoid these situations which made his everyday life difficult. He also experienced similar symptoms when introduced to people or meeting people for the first time. He repeatedly went to see various doctors many times in order to exclude any medical conditions. George stated that he didn’t experience any symptoms of depression, had no prior psychological or psychiatric treatment and/or medication, and had first experienced this problem in the course of the previous year.
At the time of the intake, George was in his final exams which he wanted to finish successfully, and continue his studies abroad. Due to his condition, he decided not to apply for a postgraduate degree in the United Kingdom, which he always wanted, and started looking for alternative postgraduate courses in Greece.
George was referred by a private psychiatrist. The psychiatrist used the Mini International Neuropsychiatric Interview, which is a structured interview based on DSM-IV diagnostic criteria. George met the criteria for a Social anxiety disorder. He also met the criteria for specific phobia limited-symptom, which was secondary to his social phobia. The psychiatrist suggested to George, to better help him with his current symptoms to take selective serotonin reuptake inhibitors (SSRIs). George however refused to take any medication and the psychiatrist referred him to the Counseling Centre. For the specific case we decided to give individual cognitive behavior therapy based on Clark and Wells model for Social Anxiety Disorder, as referred into the NICE guidelines. To better assist conceptualization and treatment and also monitor his progress, two therapists were assigned to George and two assessment measures (STAI and SPAI) were given, prior to the course of treatment, following therapy and at one month follow-up. He also had to complete a self-monitoring scale through-out the 20 weeks of treatment.
The state-trait anxiety inventory (STAI), the appropriate instrument for measuring anxiety in adults, differentiates between state anxiety, which represents the temporary condition and trait anxiety, which is the general condition. The STAI includes forty questions, with a range of four possible responses. In each of the two subscales scores range from 20 to 80, high scores indicating a high anxiety level. Higher scores correspond to greater anxiety.
The Social Phobia and Anxiety Inventory (SPAI) is a 45 item self-report measure that assesses cognition, physical symptoms, and avoidance/escape behavior in various situations. It includes two subscales: Social Phobia and Agoraphobia. A difference score above 60 indicates a potential phobia, and a cut off score of 80 maximizes this identification rate.
George’s pre-treatment scores were, SPAI:126, State Anxiety: 64 and Trait Anxiety: 63. The ultimate goal in each situation was to reduce the client’s level of anxiety.
Cognitive-behavior techniques such as self-monitoring, cognitive restructuring, relaxation, breathing retraining, and assertiveness training were employed to reduce anxiety and fear.
Self-monitoring refers to the systematic observation and recording of one’s own behaviors or experiences on several occasions over a period of time. Self-monitoring can be used as a therapeutic intervention, because it helps the patient to evaluate his/her thoughts, emotions, and behaviors, recognize the feared situations and find appropriate solutions. Kazdin states that self-monitoring can lead to dramatic changes, while Korotitsch and Nelson-Gray add that although the therapeutic effects of self-monitoring may be small, they are rather immediate. George was asked to monitor his thoughts, feelings, and behaviors and record any changes.
George had to complete an Every Day Self-Monitoring Scale for 20 weeks measuring feelings of anxiety (0=no anxiety to 10= most anxiety) and phobia (0=no feelings of phobia to 10=most feelings of phobia), ().
Beck and Emery, have identified three phases in cognitive restructuring: i) identification of dysfunctional thoughts ii) modification of dysfunctional thoughts and iii) assimilation of functional thoughts. During cognitive restructuring, the client starts recognizing his/her automatic or dysfunctional thoughts and emotions that derive from this thoughts. For example, one of George’s thoughts was that: it is dangerous to drive at night, which made him feel very anxious and frightened. However, an adaptive thought could be that: that sometimes is dangerous but also a lot of times are not, due the fact that at night there is less traffic in the streets. Therefore, throughout the sessions he was taught how to substitute several automatic negative thoughts with adaptive ones. He also kept a dysfunctional thought record for 6 sessions, which he discussed with his therapist every week.
Relaxation techniques were used for the treatment of George’s symptoms and more specifically for the physiological manifestations of anxiety and panic.
George was trained in breathing and muscle relaxation exercises, based on Jacobson’s technique and he was given 8 relaxation training sessions, in order to establish a sense of control over his physical symptoms. The client learned to apply brief muscle relaxation exercises in his daily life and especially every time he had to face an uncomfortable situation.
Assertiveness training can be an effective part of treatment for many conditions, such as depression, social anxiety, and problems resulting from unexpressed anger. Assertiveness training can also be useful for those who wish to improve their interpersonal skills and sense of self-respect and it is based on the idea that assertiveness is not inborn, but is a learned behavior. Although some people may seem to be more naturally assertive than others are, anyone can learn to be more assertive. In the specific case the therapists helped George figure out which interpersonal situations are problematic to him and which behaviors need the most attention. In addition, helped to identify beliefs and attitudes the client might had developed, that lead him to become too passive. The therapist used role-playing exercises as part of this assessment.
George completed 20 individual, 50 min therapy sessions that took place within a period of 5 months. During the first session the rationale of the cognitive-behavioral treatment was analyzed and special emphasis was given to educate the patient on Social Anxiety disorder and Specific phobias. An introduction was made to the role that automatic thoughts play in our cognitions and helped him to recognize automatic negative thoughts and feelings. A self-monitoring diary of anxiety was given to him as homework. Emphasis was also given to establishing good rapport and collaboration in the therapeutic relationship. During the second session, George narrated stressful life events and reported specific cases in which the anxiety symptoms increased. He was also taught how to identify the three phases of cognitive restructuring and was given the dysfunctional thought record as homework. The third session was based on teaching him breathing exercises and muscle relaxation. Relaxation techniques were taught by a different therapist, with expertise in stress management and relaxation techniques. George was given 8 such sessions, each lasting 20 minutes while he also practiced the sessions daily at home and completed a Daily-form for progress monitoring.
Sessions 4 to 9 were devoted to ways of challenging dysfunctional thoughts by resorting to adaptive responses. At first we tried to recognize negative automatic thoughts during specific situations and record George’s mood in that situation. After recognizing George’s negative thoughts, emotions and behaviors, we worked on the evidence that supported these thoughts.
The next three sessions (10-12) were devoted to teach him assertiveness skills to learn to socialize with people more effectively. We explored what assertiveness meant for George, what prevented him from being assertive and what were the differences between assertive, submissive and aggressive behavior, which he found really helpful and role-playing exercises were initiated to exercise these skills.
Sessions 13-20 were devoted identifying anxiety provoking situations which were hierarchically classified according to the degree of anxiety they produced. An example is shown in . Exposure to feared situations was performed by facing in vivo each level of the hierarchy and gradually practice each step, until he was confident enough to go on to the next.
Accordingly, situations such as driving, crossing bridges etc were also explored.
During the last session, George referred to overcoming challenging experiences, such as meeting new people, visiting friends living in tall apartment buildings and crossing two high bridges, while driving to visit his parents in a different part of Greece. He effectively challenged his cognitions in all relevant situations and utilized muscle relaxation and breathing exercises to control feelings of anxiety. Last session was also devoted to discuss relapse prevention, ways to avoid it and how to overcome past failures and difficulties. Finally, we discussed how he could modify and apply the skills and techniques that he had learned, in his daily routine.
The post-treatment scores of STAI and SPAI obtained by George at termination indicated an improvement. The Social Phobia score dropped to 100, the Anxiety State score was 41 and the Trait score was 42.
During the follow-up session one month later, George talked about his improvement, he mentioned that his progress continued and that he was not experiencing any of the averse symptoms of the past, while driving, visiting tall buildings/bridges and meeting new people. He continued the relaxation and the cognitive restructuring exercises. The STAI & SPAI scales were administered again. The assessment revealed maintenance of gains in terms of reduced anxiety and fear symptoms with State anxiety score: 38, Trait anxiety score: 39 and SPAI score: 77 ().


A 28-year-old, right-handed, healthy male had been suffering from pain and cramping sensations in both his hands for years since working as a crane driver. His work involved operating a crane with a joystick in each hand. As long as he could remember, he had had big hands compared to those of his peers of the same age. He had no other symptoms. Family history of neuromuscular diseases or congenital abnormalities was negative. Physical examination revealed an increased muscle bulk of both hands and the right forearm, with the right hand being slightly bigger than the left hand. In particular, both abductor pollicis muscles, the dorsal intrinsic hand muscles, and the abductor digiti quinti showed an increased muscle belly volume (fig. ). Motor and sensory functions of the hands were normal, as was the rest of the neurological examination. His feet and jaw were of normal proportions.
Laboratory tests, including sedimentation rate and creatine phosphokinase revealed no abnormalities. T1- and T2-weighted MRI showed diffuse enlargement of the muscle mass, in particular of both abductor pollicis muscles, the intrinsic muscles, the abductor digiti quinti, and the right forearm flexor compartment (fig. , fig. ). The maximal axial diameters of the muscle belly of the first dorsal interosseus muscles were 24 × 16 mm (right) and 25 × 18 mm (left). The only normal values we could find in the literature were from 5 healthy males who had a muscle belly width of 9.6 ± 0.5 mm on ultrasound (mean of radial and ulnar muscle proportion). There are no normal values known for the abductor pollicis muscle [, ]. The right forearm axial diameter measured at maximum (10 cm from the elbow) 54.75 × 53.12 mm, compared to 50.04 × 50.58 mm for the left arm. The muscles had a normal density without signs of architectural change, fatty infiltration, tumors, or fluid accumulation. Visual ultrasound assessment also showed a normal echogenicity (i.e. Heckmatt grade I) of the muscles. X-ray examination revealed no bony abnormalities. Nerve conduction studies were unremarkable for median and ulnar nerve amplitudes and conduction velocities. Needle electromyography examination of the right abductor pollicis brevis, biceps brachii, left extensor digitorum communis, abductor pollicis brevis, and abductor digiti minimi showed sporadic polyphasic, large motor unit potentials with a normal interference pattern. Because of the mild enlargement, its nonprogressive nature, and the absence of other abnormalities, no further workup (such as muscle biopsy or search for a somatic mutation in PIK3CA) was performed.
On the basis of the patients’ history, physical examination, and imaging, a congenital hypertrophy of both hands and the right forearm was diagnosed. Given the work-related nature of the symptoms, he was referred to a rehabilitation physician who successfully instructed him how to avoid cramps and pain.


An 89-year-old female with no prior ophthalmic history presented with cataracts. Preoperatively, a delaminated anterior capsule floating in the anterior chamber was seen on slit-lamp biomicroscopy (fig. ), and the edge of delamination could be seen on retroillumination (fig. ). She underwent phacoemulsification, which required Tryptan blue (Vision Blue, 0.1%; DORC International BV, Zuidland, The Netherlands) staining of the capsule. Histology of the anterior capsule revealed areas of focal splitting (fig. ).


The second patient was also an 89-year-old female. Preoperatively, she had old corneal scars, but the patient denied any history of ocular trauma or inflammation, and no specific cause of the corneal scars could be identified. The delaminated anterior lens capsule was noticed intraoperatively. Phacoemulsification was performed uneventfully without the need for capsular stains. Postoperatively, she was noted to have progressive increase in the cup-disc ratio but a normal intraocular pressure. She was subsequently diagnosed with normal tension glaucoma.


An 85-year-old male presented with good past ophthalmic history. A partially delaminated anterior lens capsule was found intraoperatively. He had uneventful phacoemulsification without the need of capsular staining.


A 72-year-old male had a bi-temporal hemianopia due to a pituitary macroadenoma. His history was otherwise unremarkable. He underwent uneventful phacoemulsification without needing any capsular staining.


An 83-year-old female presented with cataracts. Several years before, she had an acute angle-closure attack and had undergone laser iridotomy. Her intraocular pressure was normal since the angle-closure episode and did not require glaucoma medication. A delaminated anterior lens capsule was diagnosed preoperatively. She underwent uneventful phacoemulsification. Adequate visualization of the lens capsule required capsular stain. Figure shows the double-ring sign, which was noted intraoperatively.


A 92-year-old male with no prior ophthalmic history presented with a dense cataract. Delaminated anterior lens capsules were found in both eyes. Uneventful phacoemulsification was performed. Histology of the anterior capsule confirmed the diagnosis of true exfoliation (fig. ).


A 19-year-old male presented with chief complaints of protrusive lower front teeth and a large lower jaw. Extraoral examination reveals a concave profile, increased lower anterior face height, mild hypoplastic maxilla, positive lip step, hyperdivergent skeletal pattern, and a large mandible [Figure -]. Intraorally, he exhibits a Class III molar and canine relation, mild crowding of upper and lower anterior teeth, dental midlines matching, and reverse overjet of 3 mm [Figure -]. A cone beam computed tomography scan (CBCT) reveals the absence of skeletal asymmetry and no abnormality of the temporomandibular joint [Figure -].


A 21-year-old male reported to the orthodontic clinic with chief complaint of protrusive lower front teeth and large lower jaw. On examination, CBCT scan and extraoral images revealed a severe Class III profile with increased lower anterior face height, shallow mentolabial sulcus, positive lip step, and maxillary hypoplasia with no temporomandibular joint abnormality [Figure -].
Intraoral images revealed a full cusp Class III molar with Class III canine, midline diastema, upper incisor proclination, moderate upper and lower crowding, and reverse overjet of 6 mm [Figure -].