A 40-year-old female, non smoker was referred by her family physician with migraine-type headache complaints and mild nasal congestion. The pain was located over the forehead and behind the left eye. Headaches occurred once every 3 weeks, triggered by weather and barometric changes with mild improvement during the menstruation. The duration of the headache was 3 days approximately and the pain was not associated with nausea, photophobia, vertigo or tinnitus. Its intensity was 9 on a 0–10 visual analogue scale.
The patient reported nephrotic syndrome at the age of 3, for which she had been on prednizolone for 10 years. A history of recurrent episodes of rhinosinusitis was also obtained.
Nasal endoscopy revealed a mild septal deviation, a right concha bullosa of the middle turbinate and a paradoxically curved middle turbinate on the left side. Coronal computed tomography (CT) scan confirmed the above findings and additionally revealed a superior concha bullosa and mild mucosal disease on the left side (Fig. ).
The patient underwent a detailed laboratory examination to exclude any other possible causes of headache. Whole blood cell count, urinalysis, thyroxin level, biochemical tests, tumor markers, as well as Mantoux test and monotest were normal. Chest radiography, electrocardiography, and ultrasonography of the thyroid gland were also normal. Biochemical and serologic tests of hepatic dysfunction were negative. Serologic tests of complement fixation titers of the plasma antibodies against various common viruses were performed and were found normal as well.
The patient underwent nasal rigid endoscopy after application of local anesthetic and decongestant, which revealed the presence of a large superior concha in close contact with the nasal septum (Fig. ). Direct application of local anesthetic (1% tetracaine hydrochloride solution) at the point of contact between the superior concha and the nasal septum improved headache by 4 points (score 5 at the scale of 0–10). Injection of local anesthetic (0.5 ml of 2% xylocaine with epinephrine 1:100.000) into the superior turbinate under endoscopic visualization eliminated the pain completely.
The patient was given endonasal steroids for 20 days without improvement. Then she was advised surgical intervention which she initially denied. The patient was followed for 3 months without any improvement and she, finally, decided to undergo surgery. Septoplasty and bilateral endoscopic sinus surgery was performed, including partial removal of both the pneumatized middle turbinates in conjunction with gentle lateralization and resection of the lower half of the left superior turbinate. The interior of the superior turbinate was found devoid of fluid or any other content. The patient reported prompt relief of her headache and at 13 months of follow-up postoperatively she remains free from headache or any other nasal symptoms. Although the patient did never report anosmia, she was examined by using the "Sniffin' sticks" test 1-year-postoperatively []. The examination showed that odor threshold, odor discrimination and odor identification were within normal limits. It appears, thus, that although part of the olfactory mucosa might had been removed by the partial resection of the superior turbinate, sufficient olfactory epithelium had remained intact, resulting in preservation of normal olfactory function.
Although pneumatization of the middle nasal turbinate has been occasionally reported [], pneumatization of the superior and inferior turbinates are very rare findings [,]. The presence of a superior concha bullosa is not always recognizable with nasal endoscopy alone, due to the minimally accessible area of the upper nasal cavity. For this reason, the superior nasal turbinate has been called the forgotten turbinate []. Coronal CT-scan provides useful information for this inaccessible area.
Association of a massive extensively pneumatized superior turbinate with headache is very rare. In these cases the superior turbinate is forced anteriorly and inferiorly at the area between the middle turbinate and the nasal septum, leading to intranasal mucosal contact []. Although the phenomenon of referred headache owed to intranasal mucosal contact was recognized as early as 1888 by Roe [] and has been since, occasionally, reported, with the advent of functional endoscopic sinus surgery and CT imaging, resurgence of interest in headache of nasal origin has been observed. Stammberger and Wolf [] reviewed the possible mechanisms involved in the genesis of referred headaches in the nasal area. According to them, afferent fibers from pain receptors in the nasal mucosa terminate in the same group of sensory neurons in the sensory nucleus of the trigeminal nerve, as fibers innervating cutaneous receptors, located at several peripheral segmental dermatomes of the ophthalmic and maxillary divisions of the trigeminal nerve. These two common pathways converge along the same final neurons to a common area of the cortex. Accordingly, the cortical center can not distinguish the original peripheral source of the pain impulses and they may be misinterpreted as coming from other skin areas, such as the temple, the zygoma or the forehead. The pain may be perceived also from other end-organs innervated by terminal branches within the trigeminal system, such as dura, intracranial and scalp vessels or the eye [].
An important issue in the generation of headaches of nasal origin is mediation of neuropeptides, such as substance P [,,]. These may be released by mechanical pressure induced in areas of contacting opposing mucosal surfaces, such as the superior turbinate and the nasal septum. Substance P can be liberated at both the central and the peripheral ends of a sensory neuron, mediating not only central pain reflexes, via afferent C fibers, but at the same time local reflexes at the nasal mucosa, resulting from reverse impulses and manifestating as vasodilation, extravasation of plasma, hypersecretion, and smooth muscle contraction. This axon reflex can explain why an initial small localized lesion, such as a limited mucosal lesion or area of nasal mucosal contact, may trigger severe long-standing headaches, frequently projecting to different areas of the head.
Many areas of the nose and the paranasal sinuses have been implicated as causes of referred headaches, including superior concha bullosa as a rare occurrence. Clerico [] was the first who suggested the superior turbinate as a source of referred headache, with features consistent of common migraine. Two more reports on this issue followed [,]. Clerico [] proposed that before surgery, the application of local anesthetic and/or decongestant (diagnostic block) on the area of the superior turbinate, under endoscopic visualization, might confirm this anatomic variation as the source of headache. We performed this test, and although significant reduction of the pain was obtained, the pain was not completely eliminated. This was probably due to the fact that local decongestants and anesthetic sprays are deposited primarily in the anterior half of the nasal cavity and may not adequately reach the superior turbinate. Complete relief of headache was obtained only after injection of local anesthetic into the superior turbinate under endoscopic guidance. The positive finding of this diagnostic test and resolution of the symptoms after surgical intervention prove the association of our patient's headache with the presence of the massive superior concha bullosa.
Finally, the presence of a nasal osteoma as a rare cause of headaches of nasal origin should be mentioned. Paranasal sinus and intranasal osteomas are histologically benign and slow-growing tumors, which are usuallly asymptomatic, but when they enlarge they may produce pressure symptoms such as headaches. They occasionally cause obstruction or infection and may grow into the orbit or intradurally causing neurological manifestations. Paranasal sinus osteomas usually occur in the frontal sinus and less frequently in the other sinuses []. A few cases of intranasal osteomas were also reported, mainly in the middle turbinate and once in the superior turbinate []. All cases of turbinal osteomas were characterized by accompanying headache, probably due to the same pathogenetic mechanisms implicated in headache caused by the presence of a pneumatized superior concha, as previously mentioned. CT scan may assist in differential diagnosis by depicting the lesion and providing detailed information concerning the bony structures of the region.


The second girl (10 years old) complained about back pain after she had fallen from her bike. She had grown normal along the 50th height percentile. X rays of her spine revealed oedematous changes of 3 and compressions of 2 thoracic vertebrae (# 9, 11). At first these were considered trauma related, however, especially posterior oedematous changes of the 8th vertebra were considered not to be typical sequels of a trauma (figure figure ). In addition, a technetium bone scan did show an additional lesion in the right 4th rib (figure ). The rib lesion was also analysed by MRI of the thorax. It did show a strong T2/TIRM/STIR signal. Thus Langerhans' cell histiocytosis was suspected at that time. A biopsy of this rib, however, revealed a sterile lympho- plasmocytoid osteomyelitis suggesting multifocal CNO. There were no elevated inflammatory parameters (ESR, CRP, leukocytes). A differential blood count did not show signs of malignancy. Further workup revealed normal serum levels of beta-HCG (human choriogonadotropin), neuron specific enolase, cortisol and angiotensin converting enzyme. HLA B 27 was present. Total alkaline phosphatase levels (401, 426, 316, 378, 185, 189 U/l) measured over time were in the normal range. Compared to a cohort of genetically diagnosed childhood hypophosphatasia patients [,] these levels were considered unremarkable. However, a nephelometric measurement of the bone alkaline phosphatase revealed a reduced level (8.9 microg/l; normal 36–80 microg/l). Therefore, a more reliable and specific measurement of the tissue non-specific alkaline phosphatase in leukocytes was done. The result was below normal (1.8 nmol/min/mg Protein; normal: 2 – 18) []. In addition, pyridoxal phosphate in the plasma was slightly elevated (31 ng/ml; normal: 5 – 30 ng/ml), phosphoethanolamine excretion in the urine was normal. We suggested that a partial defect in the TNSALP gene was present. She may be a carrier of one severely mutated allele of alkaline phosphatase, because slight elevation of plasma pyridoxal levels has been demonstrated in obligatory heterozygotes (parents of index patients) with severe alleles []. Thus, the patient might be a carrier for the hypophosphatasia trait. The family refused a diagnostic genetic workup.
Based on our experience in treating hypophosphatasia patients with non-steroidal anti-inflammatory agents [,], in addition to the anti-inflammatory treatment of chronic non-bacterial osteomyelitis patients [] we started a therapy using naproxen at a dose of 15 mg/kg/d in both patients. This therapeutic protocol was approved by the ethics committee of the University of Wuerzburg.
The treatment rapidly reduced clinical symptoms including pain and soft tissue swelling (figure ). Parallel to this clinical improvement subsequent MRI analysis after 3, 6 and 12 months revealed subsequent reduction of bone marrow oedema and local gadolinium enhancement. 12 months later MRI showed almost unremarkable lesions (figure ). Mild hyperostosis of the os zygomaticum was still present after 12 months at treatment cessation. 24 months later the patient was still in remission.
The medication did abrogate pain completely after the first 3 months of treatment. Parallel to this clinical improvement subsequent MRI analysis (figure ) after 3, 6 and 12 (figure ) months revealed subsequent reduction of bone marrow oedema and local gadolinium enhancement. 12 months later MRI showed an unremarkable 8th thoracic vertebra, whereas the 9th and 11th still showed compression without sign of acute inflammation (figure ). The rib lesion was unremarkable after 6 months of treatment. No further structural improvement was noted after 24 months. Thus, treatment was terminated.


In January 2002, a 36-year old man presented with diagnosis of 2 mm thick, Clark's level II-III, non ulcerated melanoma of the left heel. He underwent wide local excision and sentinel lymph node biopsy (SLNB). Preoperative lymphoscintigraphy showed uptake of the radiotracer in three nodes of ipsilateral groin that after removal resulted on final pathology negative for metastatic melanoma. In the February 2005 the patient presented at follow-up with clinical nodal disease in the left groin confirmed by fine needle aspiration cytology (FNAC). Preoperative staging did not reveal signs of systemic disease, therefore the patient underwent an inguinal lymph node dissection, that revealed 1 positive lymph node out of 8, and concomitant iliac and obturator lymph node dissection, with 3 positive nodes out of 11. Three months later he developed a mild-grade lymphedema of the inferior left extremity. Ultrasounds study (US) and magnetic resonance imaging (MRI) (Figure , ) revealed evidence of two involved nodes in the popliteal fossa measuring respectively 2.7 × 1.7 and 2.3 × 1.5 cm, positive for metastatic melanoma by FNAC. Therefore, the patient underwent popliteal lymph node dissection according to the technique first described by Karakousis []. In the October 2005 during follow-up control, whole body positron emission tomography (PET) showed uptake of the radiotracer in the superficial tissues of the trunk, left thigh, left leg and in the abdomino-pelvic area (Figure ). Computerised tomography (CT) was negative for metastatic disease. The patient so underwent explorative laparotomy that revealed peritoneal carcinosis confirmed by pathology. After surgery he received chemotherapy (fotemustine 100 mg/m2 plus dacarbazine 900 mg/m2 intravenously, every three weeks, for a total of nine cycles) and in September 2006 the patient is alive with no evidence of disease (Figure , ).


A 23-year old male patient who had severe mandibular hypoplasia referred to the Department of Oral and Maxillofacial Surgery for treatment. His main complaints were unaesthetic appearance, snoring, wheezing, and difficulties during respiration, speech, and chewing.
In the extraoral examination, severely convex profile with a receding chin and a prominent nose was observed. Intraoral examination revealed a Class II Division I malocclusion with an excessive overjet. Maxillary arch had a triangular form. Right lateral incisor was missing, mild rotation of the left central incisor, palatoversion of the left lateral incisor and buccoversion of the left canine were observed due to arch length deficiency. Lower second premolars, first and second molars were missing at both sides and first premolar was also absent at left side. Mesial tipping of right and left third molars was observed due to missing of the adjacent teeth (Figure , , and ).
To determine the skeletal deformity, cephalometric analysis and measurements on stereolithographic three dimensional skull prototype model obtained through three dimensional computerized tomographic images of the patient were used. The stereolithographic model was built by Spectrum Z510 Color 3D Printers (Z Corporation-Burlington, USA).
Cephalometric analysis indicated severe skeletal Class II discrepancy confirmed by an ANB of 13°. Point A and point B were 6 mm and 42 mm behind the nasion vertical (NV), respectively. These norms supported each other and revealed that the patient had a severely retruded mandible. Ramus length (Ar-Go) was 41 mm and corpus length (Go-Gn) was 49 mm revealing deficiency of both ramus and corpus. Increases in the mandibular plane, gonial angle and Y-axis emphasized the posterior rotation of the mandible. The anterior facial height was 129 mm, posterior facial height was 64 mm and the ratio of these values was 49.61 % revealing that the patient had a long face. Overjet and overbite were measured as 15 mm and 4 mm, respectively. The cephalometric landmarks used in our report are demonstrated in Figures , and and the pre and post treatment measurements of the cephalograms are presented in Table .
Ramus and corpus lengths were also measured on the stereolithographic models. The preoperative distance between condyle and gonion was 50 mm and the distance between gonion and menton was 69 mm for the right side. They were 44 mm and 68 mm, respectively for the left side. The patient had severe mandibular hypoplasia due to inadequate ramus and corpus length, therefore using an extraoral multiplanar distractor was inevitable. Tritrac® External Distractor (Ucmed Medical Ltd. Co.-Ankara, Turkey) was used for the treatment of the presented case.
Preadjusted appliances (0.018 × 0.022 inch) were placed in the maxillary and mandibular arch and open coil spring was used to open space for the left lateral incisor. Following the leveling phase, 0.016 × 0.022 inch arch wires were placed on the upper and lower teeth.
Because of the limitation of the mouth opening, nasotracheal intubation was performed with fiberoptic bronchoscope. Under general anaesthesia, after completing the intraoral incisions and exposing the cortex of mandibular corpus and ramus on both sides, the device was secured to the mandible via 6 percutaneous pins. This system had 3 pairs of pins to be secured; one pair to the ramus, the other to the angulus and the last one to the corpus region inferior to the mandibular canal. During this procedure extraoral incisions and trochar were used. The mandibular osteotomy was then performed with round and fissure burs and osteotomes. Two osteotomies on each ipsilateral side were planned. One of the osteotomies was on the ramus and the other one was anterior to the second molar. After the completion of the osteotomies, the distractors were tested and were placed paralell to each other. Then, the incisions were primarily closed.
A 7 days of latency phase was waited for callus formation. Afterwards, distraction was performed at a rate of 0.5 mm, twice a day. Ramus was distracted for 7 days and corpus was distracted for 45 days. While the corpus was being distracted, the angles of distractors were reduced 5° on days 10, 20, 30, 40 so as to decrease the gonial angle. Paralellization of the distractors were protected during this procedure. Once the desired bone length had been acquired, 3 months of consolidation was allowed. Following the consolidation phase the distractor was removed under local anaesthesia and the extraoral wounds were debrided and primarily closed and left for recovery.
The patient used class II intraoral elastics at night time in order to prevent relapse and elastic traction was completed after 3 months. The patient was followed-up for one more month in order to determine whether relapse would occur. Brackets were debonded 4 months after consolidation period. The total treatment time was 14 months.
Finally the prosthetic rehabilitations of the missing teeth in the mandible were solved with stationary restorations.


A 67 year-old male with a past history of angina and arthritis presented with a one week history of melaena and lethargy. He was found to be anaemic (Hb 6.7 g/dl, MCV 92.2 fl). Endoscopy revealed moderate haemorrhagic gastritis and a Campylobacter-like organism (CLO) -positive duodenal ulcer, but no signs of recent bleeding. His non-steroidal anti-inflammatory medication was stopped and eradication therapy prescribed. He remained an in-patient for one week, during which time seven units of blood were transfused to correct his Hb to 11.6 g/dl.
Six weeks later he presented with abdominal pain and further melaena requiring a 2-unit blood transfusion. Endoscopy revealed mild antral gastritis and a normal duodenum but no source of bleeding. Colonoscopy was normal. Two weeks later endoscopy was repeated which showed a small amount of fresh blood in the duodenal bulb, but no obvious lesion was seen to account for the bleeding.
Three weeks later, he remained symptomatically anaemic, requiring further blood transfusion. Because of the history of melaena he underwent a fourth endoscopy which revealed a small, highly vascular polyp in the duodenum just beyond the angulus which bled easily on contact. Multiple biopsies were taken. The endoscopist was concerned about the possibility of a pancreatic neoplasm eroding into the duodenum and therefore an abdominal CT was arranged. Surprisingly, this demonstrated a large left renal mass with evidence of left adrenal and lung metastases, and a polypoidal mass in the medial wall of the second part of the duodenum arising from the pancreas (Figure ). Histology from the duodenal polyp showed small, vacuolated, clear cells highlighted by immunostaining with CAM 5.2 and showing strong reactivity with Vimentin. These findings confirmed the diagnosis of metastatic RCC.
Over the three month period from initial presentation to diagnosis he required transfusion of a total of twenty units of blood for recurrent symptomatic anaemia. Given the history of recurrent melaena, coeliac angiography was undertaken with a view to trans-catheter embolisation. This was performed successfully via occlusion of the anterior and posterior pancreaticoduodenal vessels using coils (Figure and ).
He subsequently underwent palliative left nephrectomy and histology from the resected specimen confirmed a grade 2 clear cell variant of RCC. He completed a course of interferon therapy and was able to lead a normal life for approximately 18 months, with no recurrence of anaemic symptoms or melaena. He subsequently developed painful maxillary metastases for which he received a course of radiotherapy, but died at home nearly two years after his initial presentation.


A 75 year old male with a past history of left nephrectomy 9 years previously for RCC was referred for out-patient investigation of iron-deficiency anaemia (Hb 10.3, MCV 78.9). Gastroscopy revealed a CLO-negative duodenal ulcer. Duodenal biopsies were within normal histological limits. Proton-pump inhibitor and iron therapy was prescribed and the patient was scheduled for review in the out-patient department.
He remained anaemic three months later and repeat gastroscopy and a colonoscopy were arranged. Colonoscopy was normal, but gastroscopy revealed a fleshy vascular polyp in the duodenal bulb which was biopsied. A CT scan of the chest and abdomen, and a bone scan revealed a pancreatic mass invading the duodenum (Figure ) but no metastatic disease elsewhere. Histology from the polyp showed multiple large clear cells which stained positively for MNF116 and Vimentin, confirming the diagnosis of metastatic RCC.
The patient was unable to tolerate interferon therapy. He became jaundiced due to tumour invasion of the distal common bile duct which was managed successfully by endoscopic biliary stenting. He received a total of 13-units of blood for symptomatic anaemia around the time metastatic disease was diagnosed, but since being commenced on a proton pump inhibitor his haemoglobin has remained stable.


The patient was a 41-year-old male who presented with pain in the nape of the neck of one week duration in December 2005. Also there was a history of headache on and off for the past one year and vertigo for two and a half years. Magnetic resonance imaging showed a predominantly solid tumor with ill defined margins. The mass showed a heterogeneous hyper-intense signal on T2 weighted images with no obvious cystic degeneration. It was occupying the cerebral parenchyma of the left temporal and parieto-occipital regions. There was involvement of posterior part of body and splenium of the corpus callosum with extension of the lesion across midline to the right parietal region. The left side of midbrain, thalamus and basal ganglia appeared distorted along with tentorial herniation. The posterior part of the body of the left lateral ventricle was compressed and the third ventricle was displaced to the right side. The tumor was close to but did not reach the cortical surface. Calcification and perilesional edema were absent (Figure ). Craniotomy was done with near-total excision of the tumor. There were no post operative complications and the patient was discharged after a few days. No radiotherapy was offered to patient and there were no signs of recurrence till last follow up.


A 57-year-old Caucasian male was first seen by a rheumatologist in 1998 for arthralgias and myalgias of his proximal upper extremities. His medical history included obesity and hypertension. There was no family history of rheumatologic disease. He worked as a glazier, and had no substance abuse history. Physical examination showed synovitis of his metacarpophalangeal joints and wrists. Erythrocyte sedimentation rate was >120 mm/hr, and rheumatoid factor was negative. He was diagnosed with seronegative rheumatoid arthritis, and prednisone was initiated at 10 mg daily in 1999. His arthralgias and myalgias responded clinically to the prednisone.
In late 1999, because of new symptoms of fatigue, weight loss, and fevers, 10 mg of weekly methotrexate was added to his regimen. An infectious disease consultation was obtained to rule out infection as a cause of his symptoms, and an extensive workup was performed. A chest radiograph was normal, and computed tomography (CT) scans of the abdomen and pelvis were unremarkable. A bone marrow biopsy was normal and cultures for bacteria, mycobacteria, and fungi were negative. Multiple blood cultures for bacteria were negative. With the exception of a persistently elevated sedimentation rate of 133 mm/hr, laboratory results were normal, and no infectious etiology for his symptoms could be found. Because of a lack of response, his methotrexate was discontinued after 3 months of therapy. His weight loss and fevers stopped, and only his fatigue continued.
In January 2002, still on oral prednisone, he was diagnosed with degenerative arthritis of both shoulders. His prednisone was increased to 15 mg daily, and azathioprine 100 mg daily was added. In April 2002, his left acromioclavicular joint was injected with a corticosteroid for symptomatic relief. In June 2002, both acromioclavicular joints were injected with corticosteroids. In December 2002, he first noted an erythematous, warm rash that began simultaneously on both shoulders at the sites of his shoulder injections.
In April 2003, he transferred his rheumatological care to our institution. His immunosuppressant regimen consisted of prednisone 10 mg daily, azathioprine 100 mg daily, and hydroxychloroquine 200 mg twice daily. He complained of fevers, fatigue, worsening bilateral shoulder pain, and progressive bilateral shoulder rash. In addition, he noted new dyspnea without cough. His shoulder pain now prevented him from carrying objects and performing his job. His left shoulder rash, shown in Figure , was macular, warm, erythematous, non-tender, and blanched with pressure. At its center was a 5-centimeter crusted ulcer with modest serosanguinous drainage, overlying the acromioclavicular joint. The surrounding erythema had progressed to include his entire left breast to the level of the nipple. It extended slightly onto the left arm and upper back, and did not cross the midline. On the right shoulder, there was a 5-centimeter circular patch of mottled erythema and warmth without ulceration or drainage, overlying the right acromioclavicular joint and symmetric with the central focus of the left-sided rash. There was no appreciable lymphadenopathy. Physical examination of the shoulders was limited by pain with both active and passive range of motion in all directions. There was a palpable effusion of the left shoulder, but not the right. Physical examination of his fingers, wrists, elbows, knees, ankles, and feet was otherwise normal with no evidence of synovitis.
The consulting rheumatologist concurred with the original diagnosis of seronegative rheumatoid arthritis. However, due to the presence of the unexplained rash, alternative and additional diagnoses were considered, including dermatomyositis, sarcoidosis, cutaneous leukemia or lymphoma, and infection. Erythrocyte sedimentation rate was >140 mm/hr, and C-reactive protein titer was 1:16. Anti-nuclear antibodies, uric acid, rheumatoid factor, C3, and C4 were normal. White blood cell count was 13,400 cells/mm3 with a neutrophilic predominance, hemoglobin was 10.6 g/dL and platelet count was 400,000 cells/mm3. Serum albumin was 2.8 gm/dL. Serum electrolytes, renal function, liver tests, and creatine kinase were normal. A serum electrophoresis showed polyclonal hypergammaglobulinemia, and ferritin was elevated at 356 ng/ml, suggesting chronic inflammation. A chest radiograph was normal. A skin biopsy of the left shoulder cellulitis in July 2003 revealed non-caseating granulomatous inflammation consistent with sarcoidosis. Stains for fungus and acid-fast bacilli (AFB) were negative, but cultures were not performed.
He was diagnosed with cutaneous sarcoidosis and treated with azathioprine 100 mg daily, prednisone 40 mg daily, and rofecoxib 25 mg daily. Chest CT revealed subtle reticulonodular parenchymal changes with lower lung zone predominance. Though the CT findings were compatible with the diagnosis of sarcoidosis, the lower lobe involvement and absence of lymphadenopathy were atypical.
Because the clinical syndrome was not entirely consistent with sarcoidosis, repeat skin biopsy was performed in September 2003, this time with fungal and AFB cultures in addition to the routine pathology stains. Once again, pathology revealed granulomatous inflammation consistent with sarcoidosis with negative fungal and AFB stains. However, AFB cultures grew mycobacteria after only eight days. The diagnosis of MAI was made by DNA probe, and confirmed by culture. Bacterial and fungal cultures were negative. Serology for HIV was negative.
Antimicrobial therapy consisting of azithromycin, ciprofloxacin and ethambutol was initiated in December of 2003 by an infectious diseases consultant. Azathioprine was discontinued and corticosteroids were tapered to 7.5 mg po qd, which was the lowest tolerable dose for the patient because of a return of his myalgias and arthralgias. Shoulder plain films and magnetic resonance imaging (MRI) revealed large joint effusions and evidence for avascular necrosis, greater on the left side (Figure ). Joint aspiration of the left shoulder revealed MAI by culture, and surgical drainage and debridement was performed in January 2004. Intraoperative findings included multiple rice bodies within the joint and a large cloudy effusion. Post-operatively, he was prescribed intravenous amikacin, which was discontinued after 2 weeks due to the development of renal insufficiency. In March 2004, because the cutaneous lesions over his shoulders continued to progress despite initiation of antimicrobial therapy and decrease in immunosuppression, ciprofloxacin was changed to moxifloxacin, and rifabutin was added to his regimen.
The patient completed 21 months of multi-drug therapy with relief but not complete resolution of his rash and shoulder pain. In September 2005, his azithromycin, moxifloxacin, ethambutol, and rifabutin were discontinued on a trial basis because of cumulative drug toxicities. Unfortunately, fevers returned and the rash over the left anterior shoulder worsened. A repeat aspiration of his left shoulder confirmed persistence of MAI septic arthritis with a sensitive isolate. A follow-up CT scan of the chest showed no progression of his previously seen nodules. Antimycobacterial therapy has been re-initiated, and he has undergone a second debridement of his left shoulder. He has since responded to continued antimicrobial therapy documented by a negative joint aspiration, although he continues to have severe arthritic pain from permanent joint damage.
One of two possible mechanisms probably explains the occurrence of MAI in the skin and shoulder. The first possibility is that MAI was introduced directly into the tissue at the time of injection. MAI is ubiquitous in the environment, including water sources, and can contaminate injectable solutions []. Because MAI grows slowly, a six-month lag between injection and clinical presentation, as seen in our patient, is biologically plausible. Hoffman et al estimated that 40% of the 19 patients with septic arthritis due to atypical mycobacteria in his series had had previous joint injection; however, only 1 of the injected patients had MAI []. It is not reported whether any of the 7 MAI septic arthritis patients in Kozin et al had been previously injected or aspirated []. Czachor et al reported a kidney transplant patient who developed MAI septic arthritis months after a diagnostic arthrocentesis for the diagnosis of gout []. In our patient, the temporal relationship between the shoulder injections, the subsequent appearance of simultaneous bilateral MAI skin infections at the site of the injections, and the eventual occurrence of left shoulder MAI septic arthritis, provides strong circumstantial evidence for the causative role of joint injection in this patient. Cellulitis of the overlying skin has not been described in previous reports of MAI septic arthritis [-,].
The second possibility is that the patient had undiagnosed disseminated MAI at the time of joint injection, and the trauma of injection caused concentration of the organisms at the injection sites. There is some evidence that this phenomenon occurs in patients with disseminated MAC. Freed et al reported a case of MAI soft tissue infection documented post-mortem at an IV injection site in an HIV- and MAI-infected patient []. Meadows et al reported an abscess due to MAI at the site of an intramuscular injection in an HIV-infected patient []. Prior to intra-articular injection, our patient had been treated with corticosteroids for approximately 3 years as well as short-term methotrexate and azathioprine. While this degree of immunosuppression makes disseminated MAI possible, there is little evidence to support the theory of pre-existing undiagnosed disseminated MAI infection in our patient, though it remains a possibility. Another possibility may be increased susceptibility to mycobacterial disease due to an underlying genetic defect in the IFN-γ response pathway [].


An eight year old Malay boy, with a history of Henoch-Schonlein Purpura and G-6-PD deficiency, presented with left hand swelling and punctate rashes on the dorsum of his left hand, four hours following his transfer out from intensive care unit (ICU).
The history dated back to two weeks prior to admission when he noted rashes on the dorsum of his feet and had intermittent, diffuse abdominal pain. He then developed migratory pain in his ankle joints, knee, elbow and small finger joints in that order. He refused treatment.
He had no drug allergies. Developmental milestones were normal and immunization was complete.
A week later, he was admitted to the general pediatrics ward for intermittent vomiting, severe generalized abdominal pain and passing red-current stools. The pain was constant, unrelenting and aggravated by solid food intake despite intravenous omeprazole (16 mg bd), metoclopromide (3 mg tds) and tramadol (30 mg 4 hourly). Rashes now developed on his left flank, buttock and medial aspects of both feet. He was febrile and micturation was normal.
His vital parameters were as follows: BP 127/96, PR 99/min, RR 24/min and SpO2 97% on room air. Clinical examination revealed non-blanching purpuric rashes on the dorsum of his feet, left flank and buttock [Fig. ]. There was also localized tenderness on deep palpation of his left iliac fossa. Hematological, coagulation and renal profiles were within normal limits.
Urgent transabdominal ultrasound did not show any "pseudokidney" or "doughnut sign" to suggest intussusception." Peristalsis was normal. All intraabdominal organs were normal and there was no free fluid. There was, in the left iliac fossa, a bowel loop filled with echogenic material likely to be a stool."
Thirty-six hours later, he was admitted to the pediatric ICU owing to poor intake, severe per rectal bleeding and deteriorating general conditions. Fluid challenge with 300 mls of Ringer's Lactate solution and 2 units of packed red cell transfusion were given. Intra-arterial cannulation of the left radial artery was performed for continuous blood-pressure monitoring. Intravenous fluids, methylprednisolone (16 mg od), PCA morphine (bolus 0.5 mg, lockout at 15 mg), intravenous ceftriaxone (800 mg od) and metronidazole (120 mg tds) were administered.
Following two days of stabilization, he was transferred out from ICU after the removal of his arterial line and urine catheter. Clinical examination in the morning showed diffuse, oedematous swelling on the dorsum of the left hand and all fingers with non-blanching purpuric rash restricted to an area 4 cm in diameter. There were no signs of fluid extravasations, inflammation or infection at the site of cannulation.
An urgent Doppler ultrasound of the left forearm was performed to exclude a thrombo-embolic event. Both radial and ulnar arteries and corresponding veins were patent. Normal Doppler flow pattern was obtained within these vessels up to the level of cubital fossa.
In the late afternoon, the whole left hand was swollen intensely, with extension of the purpura from the hand, up the wrist into the forearm. The metacarpophalangeal joints of all the left fingers were extended with flexion of all the distal interphalangeal joints. Capillary refill was normal. Emergency fasciotomy decompression of the dorsal [Fig ], thenar and hypothenar compartments [Fig ] of the left hand were performed. Serous fluid accumulation was noted in all compartments with marked tissue oedema. Additional hematoma was noted in the hypothenar compartment. The rashes and swelling subsided quite quickly and he was discharged uneventfully on the fifth post-operative day.


A 11-year-old, 50-kg female with a diagnosis of CD10+ ALL was admitted to the hospital with fever and intermittent chest pain. The patient was in complete remission (CR) following SHOP-99 chemotherapy. At the time of hospital admission, she was receiving G-CSF for severe granulocytopenia post-intensification with cytarabine, and had also received broad-spectrum antibiotics and fluconazole prophylaxis during previous episodes of febrile neutropenia.
The physical examination was normal and the patient's general condition good. The blood examination showed 12,500 leukocytes/mm3 (82% neutrophils). An urine examination, a chest X-ray, and an abdominal ultrasonography did not reveal abnormal findings. Blood, urine, and stool cultures were also negative. She was initially treated with cefepime and teicoplanin. G-CSF was discontinued, after which the neutrophil count stabilized at around 1,700/mm3. CRP increased steadily up to 5.6 mg/dL despite broad-spectrum antibiotic therapy. One week later, while continuously febrile, the patient experienced worsening chest pain. A chest CT scan revealed several nodules 1 cm or smaller in diameter at the apex of both lungs (Figure ). The Aspergillus galactomannan antigen was negative. Cultures of separate blood samples obtained percutaneously and from the central venous catheter, yielded Fusarium spp. The species of the infecting Fusarium could not be identified and in vitro susceptibility could not be tested. Liposomal amphotericin B (3 mg/kg/day) was then started. Although the dose of liposomal amphotericin B was increased to 5 mg/kg daily and the central venous catheter was removed (cultures of the catheter tip were sterile), the patient remained febrile and chest pain continued to worsen over the following 7 days. Blood cultures remained positive for Fusarium spp. and CRP levels increased up to 10.6 mg/dL (Figure ). Caspofungin was then initiated (70-mg load followed by 50 mg daily). Fever disappeared within 48 hours of caspofungin onset (Figure ). Chest pain improved significantly, blood cultures became negative, and CRP levels went down to the 1–2 mg/dL range over the following days. The patient presented two isolated fever spikes 8 and 12 days after the onset of caspofungin, which corresponded to a phlebitis episode and a limited reaction to L-asparaginase, respectively. Two weeks later, a new chest CT scan demonstrated progressive resolution of the lung nodules (Figure ). Intravenous antifungals were discontinued and chemotherapy and suppressive therapy with oral voriconazole 200 mg twice daily was started. Three months later, a chest CT scan showed complete resolution of the pulmonary lesions, while the patient was asymptomatic (Figure ). Thus, voriconazole was discontinued. Twenty-five months after discontinuation of all antifungal therapy, the patient remains healthy in the absence of any symptoms of fungal infection and in CR of her neoplastic disease.
Fusarium resistance to most antifungals and the severe immunosuppression-notably long-lasting, severe neutropenia – in oncohematological patients make Fusarium infections commonly fatal []. Despite its low activity against Fusarium [], amphotericin B remains the drug of choice. Voriconazole is licensed for the treatment of fusariosis based on in vitro data and a series of case reports with a reported response rate of ~40% []. Posaconazole also has potential for therapy of systemic fusariosis []. In any case, responses obtained with monotherapies remain too low and unsatisfactory.
We decided to treat our patient with amphotericin B and caspofungin for two reasons: (i) In our view amphotericin B remains the mainstay of therapy for fusariosis in pediatric patients; experience with voriconazole in this patient segment is limited [-]; (ii) Concurrent antifungal therapy is now generally considered an alternative way of improving outcome in difficult-to-treat invasive mycoses. In a recent in vitro study amphotericin B and voriconazole rendered mainly additive or subadditive interactions against Fusarium spp. []. Yet, the concern remains that combining amphotericin B and azoles may lead to antagonism. In addition to the azole inhibition of the synthesis of amphotericin B's pharmacological target ergosterol, amphotericin B-related damage to the fungal cell membrane may interfere with the influx of azoles []. In vitro caspofungin-inactive against Fusarium-showed synergistic or synergistic-to-additive interactions with amphotericin B for at least half of the Fusarium isolates []. By inhibiting cell wall synthesis, caspofungin may presumably enhance the penetration of amphotericin B [].
We hypothesize that combination antifungal therapy likely contributed to the sucessful treatment of our patient's severe invasive fusariosis, since clinical improvement only became evident after initiating caspofungin. Yet, the effect of caspofungin in the successful outcome of our case is not completely clear. The clinical response observed could also be the result of prolonged administration of liposomal amphotericin B and the lack of severe neutropenia. Finally, the administration of suppressive voriconazole and the early re-initiation of chemotherapy allowed by rapid infection control probably also contributed to the good long-term outcome. Of note, there have been a few case reports of response to caspofungin in combination with amphotericin B [,] and as monotherapy [], although in some of these reports there was a clear association of response to recovery from neutropenia.


A 21-year-old man went to the emergency department of São Francisco Xavier Hospital (Lisbon) saying that he had appendicitis and needed an operation. He also said that his appendix and his liver were interfering with his voice. According to his mother, for the last three years the patient had displayed periods of behavioural changes, with aggressive behaviour and unwarranted laughter. Recently, he had been fired from several jobs for being late. The patient justified his behaviour by saying that he couldn't sleep at night, and described what seemed to be complex auditory hallucinations in the second and third persons with a depreciatory content.
In the previous two months the clinical picture had deteriorated, with disorganised thoughts and "periods in which he wasn't there", during which he did not answer any questions or initiate any conversation. According to the patient himself, at such times, he was perplexed because the words people said appeared to make no sense.
During the mental state examination, the patient was alert and oriented in space and time. He displayed delusions with a hypochondriac theme that focused on concerns about the state of his liver and his appendix, and auditory/verbal hallucinations with a depreciatory content. The patient was euthymic, and his feelings were appropriate, with no blunting or flattening. He did not display any insight into his condition. The neurological exam did not reveal any changes and the Mini-Mental State Examination [] was normal (29/30).
His prior medical history included a head trauma at the age of 16 that had been caused by a motorcycle accident and had apparently not been serious. No cranial computer tomography (CT) had been done at that time. The patient admitted to a regular consumption of cannabis since the age of 13, together with alcohol abuse that had recently worsened. He also had a sporadic consumption of cocaine and methylenedioxymethamphetamine (MDMA).
His family history included a suicide attempt by his half-brother a few months before, which had not been associated with any psychotic condition.
The blood tests were normal except for the toxicological traces, which revealed the presence of cannabinoids in the urine sample (52 ng/ml).
The patient was compulsorily admitted to the hospital under the terms of the Portuguese Mental Health Law.
Despite the fact that the cannabinoid levels became normal during the first few days of his stay in the hospital, the patient's psychotic symptoms persisted.
A cranial CT revealed the presence of an arachnoid cyst at the level of the left Sylvian fissure, with a marked mass effect on the left temporal and frontal lobes and the left lateral ventricle. There was also an extensive pneumatisation of the left frontal sinus. A cranial nuclear magnetic resonance (NMR) was performed in order to get a more detailed image. It confirmed the nature of the lesion and revealed the existence of a left temporal lobe hypoplasia that was associated with the arachnoid cyst (Figures , , ).
It also showed that the left frontal sinus, which was more developed, was in contact with the arachnoid cyst. An EEG revealed unspecific changes in the median temporo-parietal zones, which were more widespread on the left side. A neuropsychological examination showed various alterations, with impairment of verbal memory, attention, ability to plan and increased impulsiveness with a tendency towards anti-social behaviour.
The patient started antipsychotic therapy with risperidone 2 mg tid. Due to the fact that no links between arachnoid cysts and psychotic symptoms have been clearly established and no focal or intracranial hypertension signs were observed, the neurosurgical department concluded that even tough there was a mass effect, the risk of operating was higher than the potential benefits.
The psychotic symptoms improved progressively during the stay, with amelioration of the psychomotor agitation and remission of the auditory/verbal hallucinations. Although it was clear that there was a significant improvement in relation to the delusional hypochondriac ideas, a complete remission of these symptoms was not achieved.
The patient was discharged after a 4-week inpatient stay and received follow-up outpatient care with psychiatric and neurosurgical appointments. Three months after discharge, the patient was working part-time and attending a technical course on computer hardware. He showed the same psychotic symptoms and remained without any insight into his condition.


21 years old female was referred as complete vaginal agenesis. She was examined and Rokitansky Kustner was diagnosed. She was operated under general anesthesia in october 2004. Full-thickness skin graft taken from inguinal region was used. The prosthesis was secured in place for 5 days after the operation and then it was gently removed for first look to the grafts. For the first postoperative three weeks, wound care was done with serum physiologic, furacine (nitrofurantione) cream and rifocine (rifampisine). Then the prosthesis kept in place for 7 months with cleansing it for one time in every week. The only problem encountered during postoperative course, a granulation tissue formation along with the graft suture lines and electrocautery was applicated for 2 times. Additionaly aloe-vera cream was used for wound care during the period of 2nd–7th postoperative months in which a complete epithelization was achieved. She has been sexually active without any problem (Figure and ). Hair growth problem in the newly created vaginal space was solved with an epilatory cream application.


30 year-old female was also referred for amenore. Gynecologic examination showed that vaginal agenesis was present and a rudimentary uterus was palpated with rectal examination. Ovaries were seen normal dimentions by ultrasounographic evaluation. A further Caryotype analysis showed 46XX and Mayer-Rokitansy-Küster-Hauser syndrome (mullerian agenesis) was diagnosed for the patient. She was operated at january of 2006 with the same technique. A split-thickness skin graft was used and a monoblock skin-graft was wrapped around the prosthesis and placed into the pouche which was dissected according to Mc Indoe thechnique. 4/0 chrome catgut (Dogsan, Turkey)was used for suturing the edges of grafts. The same wound care protocol was applied to the patient and no skin graft loss was seen. She was followed up for 5 months no complication was seen except a minimal granulation tissue formation. This was also managed with electrocautery. An vaginal depth of 11 × 3 cm was obtained and maintained in the patient. The patient is still under follow-up at early postoperative period and an ideal wound healing course was also noted (Figure ).


A 55 year old gentleman underwent emergency ascending aortic replacement and aortic valve repair (valve resuspension) for a type A aortic dissection. He made an excellent recovery following this procedure and postoperative transthoracic echocardiogram (TTE) showed mild aortic valve regurgitation. A TTE repeated 4 weeks later showed presence of moderate aortic valve regurgitation. Follow-up investigations at six months showed that the ascending aorta repair was intact and the dissection flap extending to the left common iliac artery with minimal flow in the false lumen. At this stage there was no change in the degree of aortic regurgitation. However, cardiac catheterisation was performed at 8 months which revealed severe aortic regurgitation, systolic pulmonary artery pressure of 51 mmHg and impaired left ventricular function. Gradual failure of the aortic valve repair to resuspend the valve was speculated to be the likely cause of significant aortic regurgitation. Aortic valve replacement was contemplated.
Various routes for cannulation for cardiopulmonary bypass (CPB) were discussed due to previous sternotomy and the X-ray finding of close proximity of the repaired ascending aorta to the sternum. During the first operation, an attempt was made to expose the right axillary artery but the artery was not accessible. CPB was established via the right common femoral artery (CFA). The left CFA was not a viable alternative in view of the dissection flap extending down to the left common iliac artery. It was therefore elected to institute CPB through the right common carotid artery (CCA). The right CCA and the left sapheno-femoral junction were exposed. A longitudinal incision was made along the medial border of the sternocleidomastoid muscle at the level of the thyroid cartilage. The internal jugular vein (IJV) and common carotid artery (CCA) were exposed. Two slings were passed behind the CCA to aid the control of the artery for the subsequent steps of the procedure. Skin incision and preparations for the sternotomy were made. Heparin was administered to maintain an activated clotting time (ACT) greater than 400 seconds. Once this was achieved a curved clamp was applied to the exposed carotid artery and a longitudinal 2 cm incision was made. A dacron graft was then sewn onto this incision in such a fashion so as to direct the inflow of the blood towards the aortic arch (figure ). The arterial cannula for CPB was connected to the other end of the graft. The venous cannula was then introduced into the left femoral vein (figure ) via the left sapheno-femoral junction using the Seldinger technique. CPB was instituted with careful monitoring of the head for any evidence of oedema or petechial haemorrhage. Patient was gradually cooled to a core temperature of 32°C initially and then to 28°C. Sternotomy was then performed followed by exposure of the heart and the ascending aorta. The native aortic valve was exposed by making an incision in the previous interposition graft to the ascending aorta. The aortic valve was excised and replaced by a size 25 mm mechanical valve. The aortotomy was then closed. Haemostasis was achieved and the patient was weaned successfully off the CPB machine. The CCA incision was closed with 4/0 prolene followed by skin closure. Once the venous cannula was taken out the great saphenous vein was tied off. Postoperatively, no haemodynamic support was required and the patient was discharged after 6 days with no evidence of neurologic, cardiac or renal impairment.


A 55-year-old woman was admitted under the general surgeons with a three-day history of colicky right hypochondrial and epigastric pain together with three-weeks of reduced appetite and weight loss of 5 kilograms. Abdominal examination revealed mild tenderness in the right upper quadrant. Routine investigation revealed haemoglobin of 11.6 gm/dl and marginally elevated alkaline phosphatase of 110 mg/dl. Abdominal ultrasound scans revealed a 7.2 × 6.8 cm thin-walled partially cystic and solid structure adjacent to the inferior margin of the left lobe of liver.
Computerised tomography (CT) of the abdomen confirmed a partly cystic and partly solid mass with some contrast enhancement and low volume ascitis (figure , ). She was discussed in the upper gastrointestinal multidisciplinary team meeting. The mass was thought to be a bleeding liver lesion. Selective mesenteric and hepatic angiography with a view to embolisation was performed.
Angiography showed normal hepatic artery architecture. Distal gastroduodenal and gastro-epiploic catheterisation exhibited tumour neo-vascularisation (figure ), which was not amenable to embolisation. The appearance was thought to be consistent with either a GIST or a liposarcoma due to the hypervascular picture with many early filling abnormal vessels, unusually sustained tumour blush and prominent draining veins. At laparotomy there was an exophytic lesion arising from the antrum of the stomach with signs of bleeding into the tumour. The tumour was locally excised with clear margins.
The histology confirmed a 4.5 × 3.5 cm GIST with evidence of recent haemorrhage, strongly positive for CD117 with negative resection margins and a favourable mitotic number of <5/50 HPF. The patient made an uneventful recovery and has remained recurrence free for 22 months.


A 39-year-old man underwent augmentation ileocystoplasty 34 years previously to enlarge his shrunken bladder following chemotherapy and radiation therapy to his bladder and prostate for embryonal rhabdomyosarcoma at the age of 5. He was completely continent and was voiding normally after the augmentation ileocystoplasty. He had no family history of gastrointestinal malignancy. He presented with gross hematuria. A voiding cystogram showed a polypoid mass at the level of the ileovesical anastomosis. An intravenous pyelogram showed no evidence of a mass or obstruction in the upper urinary tract. Computerized tomography scans showed normal kidneys without obstruction. Flexible cystoscopy revealed a solitary 5 cm papillary tumor of the ileal segment in close proximity of the site of ileovesical anastomosis. Transurethral loop resection was used to completely excise the tumor to the underlying muscle. Histopathologic examination revealed a tubular adenoma with high-grade dysplasia (Figures &), and the adjacent ileal mucosa showed features of colonic metaplasia (Figure ). Based on the patients's age of 39 (less than 50 years old), immunohistochemical staining for the major DNA mismatch-repair (MMR) proteins MLH1 and MSH2 was performed to exclude the possiblity ofan inherited form of intestinal neoplasia, hereditary non-polyposis colorectal cancer (HNPCC). Immunohistochemical staining for MSH2 and MLH1 revealed that the high-grade dysplastic ileal epithelial cells retained both MSH2 (Figure ) and MLH1 (not shown) expression, supporting anabsence of microsatellite instability (MSI) in this neoplasm. Clinically, the complete transurethral resection of the tumor was considered adequate conservative management, and he has undergone follow-up cystoscopy at 3-month intervals for the past year with no evidence of recurrence.


A 56-year-old male patient visited the outpatient clinic of the Otolaryngology Department due to otorrhea of the right ear. It had started 3 days prior during diving in Gapyeong Valley, a mountainous area of South Korea. He experienced mild pain as his right ear touched the water surface while diving, but did not complain of subjective hearing loss, dizziness, or fever. However, he felt water in the ear canal. Clinical examination revealed swelling and debris accumulation in the right external auditory canal (EAC) with an intact, erythematous tympanic membrane (TM) and clear, odorless otorrhea (Figure ). In addition to the pre-existing hearing loss, a pure-tone audiogram showed 10 and 5 dB air-bone gaps at 250 and 500 Hz, respectively (Figure ). He was taking aspirin and clopidogrel for hypertension and previous myocardial infarction. Microbiological culture was performed on swab specimens for otorrhea. Specimens were transferred to a clinical microbiology laboratory and inoculated on chocolate agar and 5% sheep blood agar. Gram staining showed frequent white blood cells and Gram-negative rods. Small grayish colonies had grown on MAC and BAP agar with grade of ‘many’ after 1 day (Figure ). Identification by matrix-assisted laser desorption/ionization time-of-flight (MALDI-ToF) mass spectrometry using a Vitek MS (bioMérieux, Marcy-L'Etoile, France) revealed Yokenella regensburgei. PCR-based 16S rRNA gene sequencing identified the isolate as Y. regensburgei, revealing 99.72% identity (1403/1407 bp) with Y. regensburgei ATCC 49455(T) (GenBank accession number JMPS01000045). Susceptibility testing was performed using the Vitek 2 Automated Antimicrobial Susceptibility System (bioMérieux). The isolate was susceptible to amikacin, cefotaxime, ceftazidime, cefepime, ciprofloxacin, gentamicin, ertapenem, imipenem, tigecycline, piperacillin/tazobactam, trimethoprim/sulfamethoxazole, and aztreonam, and resistant to ampicillin, cefazolin, and amoxicillin/clavulanic acid. Before pathogen identification, debridement and 50% diluted acetic acid irrigation were applied. During week 2, EAC swelling and otalgia had improved but otorrhea persisted, and a pinpoint perforation occurred in the inferior anterior portion of the TM (Figure ). Because of middle-ear infection due to diving-related tympanic injury, 500 mg of oral ciprofloxacin was prescribed, twice daily for 2 weeks. During week 3, otorrhea was recultured and Y. regensburgei was no longer isolated. The TM perforation was completely healed by week 4, and the EAC had dried up (Figure ). The patient's ear infection and hearing loss were then completely treated.


A 42-year-old male patient visited our center complaining of progressive involuntary movement for 2 months. He had neither a history of a perinatal problem nor a family history of epilepsy or other neurological disorders. He had experienced normal motor and social development. Although he had graduated high school, his academic performance was at the bottom level. At 14 years of age, he presented with episodic aphasia. He was diagnosed with epilepsy and prescribed an antiepileptic drug (carbamazepine). Since then, the symptom was controlled. Approximately 3 months prior to the visit, there was one event of behavior and speech arrest with awareness. Two months ago, involuntary movement of the entire body, including eyelid myoclonus and tremulous head movements, has emerged. The movements were provoked by action and gradually deteriorated (Figure ).
On neurological examination, generalized myoclonus was provoked by action but persisted at resting status. He showed an ataxic gait characterized by a wide base, short step length, and irregular stride. Until then, he could walk without assistance. He demonstrated dysarthria but no dysphagia. The deep tendon reflex was increased, and the Hoffman sign was present on both sides. The Bedside MMSE score was 21 (time -1, calculation -5, comprehension -1, pentagon -1).
On 24 hours of video scalp EEG monitoring, bilateral occipital lobe spikes (Figure ) and generalized intermittent rhythmic delta slow activities were detected. His brain and whole-spine MRI were unremarkable. In the skin biopsy, there was no Lafora body. Targeted sequencing for MERFF and DRPLA detected no mutations.
Seven months after the visit, he presented with aggravation of gait disturbance. He could not walk without assistance. After 1 year, he had difficulty in swallowing. At that time, however, follow-up brain MRI revealed no significant interval change compared with the initial image (interval of 11 months).


History and initial presentation
A 50-year-old tubectomised lady, who was a known case of diabetes mellitus for four years (hemoglobin A1C [HbA1c]: 7.3), came with complaints of abdominal pain, distension, vomiting, and fever of three days duration, along with decreased urine output for the past one day. The pain was dull aching and more on the left side. The patient also complained of two to three episodes of vomiting per day, which was non-bilious and not bloodstained but did not report obstipation or constipation. She consulted a local hospital where an ultrasonography (USG) was done, which showed a focal peritoneal collection in the suprapubic region. She was therefore referred to a tertiary center and presented at our emergency room (ER).
On arrival at our ER, the patient was noted to be stable but appeared unwell. Her heart rate was 115 beats per minute (bpm), and her blood pressure recorded was 110/70 mm Hg. On per abdominal examination, there was distension noted with guarding and tenderness in the left iliac and hypochondriac areas. There were no excoriations, or open wounds suggestive of an entry point of infection, and no scar other than her tubectomy scar. Per speculum examination findings were unremarkable. A per vaginal examination revealed a vague mass, minimally tender corresponding to 14 weeks. Cervical motion tenderness or forniceal tenderness was not elicited.
Investigations
At presentation, blood gas analysis was generally normal except for lactate of 4.6 mmol/L. Renal function and complete blood counts were within the normal range. A contrast-enhanced computed tomography (CECT) of the abdomen was done to localize and quantify the collection and rule out serious causes like perforated viscous, ischemic bowel, and volvulus. CECT abdomen revealed a large collection in the pelvis (Figure ) with enhancing areas extending into the right adnexa and anterior abdominal wall with pneumoperitoneum, with no obvious communication with bowel loop. Clinically, the initial differentials considered were pyosalpinx, adnexal cyst, ovarian malignancy, and ectopic pregnancy. Radiologically, adnexal, omental, or neoplastic causes were suggested. Pregnancy was ruled out with a urine pregnancy test, and tumor markers sent for malignancy were negative.
Treatment
A preliminary diagnosis of pelvic abscess was made, and she was started empirically on metronidazole and piperacillin-tazobactam. She was taken up for an emergency laparotomy by the obstetrics and gynecology team after 10 hours of initial presentation. A midline vertical abdominal incision was made. Dissection revealed blackish discoloration in the umbilical area, and the general surgery team was called to debride the same. The incision was deepened to the rectus sheath, and a necrotic rectus sheath was noted (Figure ). The uterus, bilateral ovaries, and tubes appeared to be normal. A purulent collection of approximately 1 L, along with sloughed out rectus sheath, rectus abdominis muscle, and transverse abdominis muscle, was noted, while bowel appeared normal. The collection was drained, and temporary closure was done with an intraperitoneal and subcutaneous drain in situ. Necrotic tissue that was excised was immediately sent for histopathological evaluation as well as culture and sensitivity. Intraoperative blood gas analysis was suggestive of severe metabolic acidosis with lactates of 4.5 mmol/L; hence, elective extubation was deferred. The patient was shifted to the intensive care unit (ICU) for mechanical ventilation, a possible requirement of hemodialysis, and further management.
Postoperative care
The patient was continued on empirical antibiotics piperacillin-tazobactam and metronidazole. Meanwhile, the anaerobic culture sent of the tissue sample collected intraoperatively grew a single organism, Finegoldia magna. All other cultures of bronchoalveolar lavage (BAL), blood, urine, and tissue, including mycobacterial and fungal cultures, were negative. Histopathological examination of the tissue sent intraoperatively from the anterior abdominal wall showed fibromuscular and fibroadipose tissue chiefly showing necrosis.
Despite initial therapy, the patient had persistently high lactates because of which clindamycin was added on the third postoperative day. The patient was taken up for a relook laparotomy 60 hours after the first surgery. Intraoperatively, 350 ml purulent collection was drained, slough in transverse abdominis muscle was debrided, and slough extending up to the fourth intercostal space was noted. Pelvic and left intercostal drains were placed, and the patient was shifted back to the ICU. Despite extensive source control and antibiotic therapy, the patient continued to deteriorate and developed septic shock. She succumbed to her illness on the seventh day of ICU admission.


A 14-year-old boy with a diagnosis of ulcerative colitis based on colonic histology, serology and a normal barium study of his small bowels was admitted with a five-day history of nonbilious vomiting and bloody diarrhea. Additional symptoms included recent onset hematuria, and low-grade fevers to 100.4 C over the prior four days. He had also sustained a 25 lb weight loss in the last six months, indicating a lack of disease control.
As an outpatient, his maintenance therapy included mesalamine (1 gram three times a day), and mercaptopurine (75 mg once per day). In addition, he had been started on prednisone approximately 7 weeks prior for treatment of an ulcerative colitis flare. His current dose of prednisone was 10 mg once a day. Soon after symptoms begun, he had been placed on ciprofloxacin as treatment for a presumptive flare.
Physical exam showed he was afebrile, with a heart rate of 130 beats per minute, respiratory 16 breaths per minute and blood pressure 115/67 mmHg. He was alert although with a sallow appearance. Abdominal exam revealed a soft nontender nondistended abdomen. Rectal showed normal external exam with grossly bloody stool. Initial blood work showed hemoglobin of 12.3, a normal white blood cell count, normal differential and normal platelet count with a mildly elevated prothrombin time of 16.2 with an international normalized ratio (INR) of 1.2. Urine analysis showed a specific gravity of 1.035, 3+blood, +ketones and > 100 RBC per high powered field and 0–5 WBC per high power field. Abdominal ultrasound revealed irregular shaped bladder wall.
Patient was placed on intravenous fluids (IV) as well as metronidazole (IV). Blood and urine cultures were sent for analysis. Stool was sent for culture and for Clostridium difficile toxin analysis.
Serial repeat lab works the following day revealed a dropping hemoglobin (7.4 g/dL) and platelet count (64 K/mm3) increasing PT/PTT (21.3/47 seconds) with an INR of 1.8. Blood smear showed moderate amount of elliptocytes, schistocytes, microcytes and fragmented red blood cells. Initial DIC panel revealed an elevated D-dimer of 4.9 mcg/mL with a normal thrombin time and fibrinogen. Thrombin time subsequently increased to > 120 seconds. D-dimers increased to 10.3 mcg/mL. A computerized tomography (CT) scan of the abdomen revealed a marked thickening and irregularity of the bladder wall as well as wall thickening of the rectosigmoid, ascending, transverse, and descending colon (Figure ). Urology was consulted and felt that this represented a submucosal hematoma.
Patient was begun on broad-spectrum antibiotics because of concerns regarding possible bacteremia and a worsening DIC laboratory picture. Blood, stool and urine cultures returned negative. Viral cultures and monoclonal antibody staining for adenovirus detection in the urine was negative. Despite a worsening in the DIC panel, the patient remained clinically unchanged. IV steroids were begun approximately 36 hours into patient's hospital stay. Patient had a stabilization of PT/PTT/INR/thrombin time and D-dimer, and a subsequent normalization of labs over the following 4-day period ( Figure , , , , , , ). Patient's diarrhea and hematuria resolved as well. Colonscopy revealed chronic colitis consistent with ulcerative colitis. Cystoscopy revealed a fibrin clot consistent with submucosal hematoma. Patient was discharged from the hospital on a steroid taper, and remains in remission to date.


A 71-year-old Japanese woman was referred to our hospital for evaluation of her elevation of serum gamma glutamyl transferase levels to 135 IU/L (reference value 16–73 IU/L), which were incidentally found in a biochemical test in medical health check. She had no symptoms of epigastralgia or jaundice. Her physical examination and past medical history was unremarkable. She did not smoke or drink. The remainder of her serum chemistries, complete blood count, and coagulation profile were all normal. Serum levels of tumor marker such as carcinoembryonic antigen (CEA) and carbohydrate antigen 19-9 (CA19-9) were within normal limits.
Abdominal ultrasonography (US) and computed tomography (CT) demonstrated a cystic lesion measuring 2.0 cm in maximal diameter at the left lateral segment of the liver with peripheral left lateral anterior subsegmental bile duct (B3) dilation (Figure , and ). Magnetic resonance (MR) imaging also confirmed a cystic lesion at the left lateral segment of the liver, but the presence of a mural nodule in the cystic lesion or mucin was not confirmed (Figure ). MR cholangiography showed a cystic lesion at the left lobe of the liver, but a filling defect in the bile duct and a communication between the cystic lesion and bile duct could not be defined (Figure ). Duodenoscopy showed a widely patent papillary orifice with extruded mucoid material, and endoscopic ultrasonography (EUS) could not detect a tumor component in the bile duct or cystic lesion (Figure ). Endoscopic retrograde cholangiogram (ERC) demonstrated amorphous filling defects in the common bile duct corresponding to mucin, and percutaneous transhepatic cholangiograms (PTC) revealed communication between the hepatic cyst and the major bile duct (Figure ). The cytological examinations through both ERC and PTC routes were negative for malignancy.
Left and caudate lobectomy of the liver with extrahepatic bile duct resection and reconstruction was performed because the lesion seemed to be a potential malignancy. Gross examination of the resected specimens revealed no obvious mass protruding into the lumen, and only a markedly dilated bile duct was observed at a glance (Figure ). Microscopically, the cystically dilated bile duct was lined by tall columnar epithelium with micropapillary features and mucin hypersecretion, but the tall papillary growth with fibrovascular cores or villous structures showing mass component was not present (Figure ). These neoplastic cells with hyperchromatic nuclei and loss of cell polarity, lacking stromal invasion was observed (Figure ). Ovarian-like stroma typically seen in hepatobiliary cystadenomas was not observed in the wall. On immunohistochemistry using standard streptavidin-biotin-peroxidase method, meoplastic cells were positive for MUC2 and MUC5AC, but negative for MUC1, CK20. The patient has been well without any evidence of recurrence for 38 months since her operation.


A 54 year old man suffering from congestive heart failure (NYHA class II) and dilated cardiomyopathy was admitted because of syncope. Baseline ECG revealed sinus rhythm, QRS complex duration 190 ms and LBBB. Echocardiography showed an enlarged left ventricle and ejection fraction of 25%. Coronary angiography documented normal coronary arteries and a vascular structure, suspected to be PLSVC, during the venous phase. Implantation of a (CRT-D) was planned.
Cannulation of the left subclavian vein was performed. Venography confirmed a PLSVC, draining into the coronary sinus next to the origin of the postero lateral vein, and the existence of a small innominate vein, draining into the right superior vena cava. A screw-in dual coil lead (Endotak Reliance™, model 0165, Guidant Corp., St. Paul, MN, USA) was introduced in the apex of the right ventricle (RV) via the innominate vein. Stimulation threshold measured 0.5 V at 0.5ms with an impedance of 1185 Ω, R wave amplitude was 15.3mV with slew rate of > 4 V/s, high-voltage shock impedance was 47 Ω. The left ventricular (LV) lead (Easytrak® 3, model 4525, Guidant Corp.) was easily advanced through the PLSVC into the postero lateral vein (). Its stimulation threshold was 0.7V at 0.5 ms, R wave amplitude 13.6 mV, slew rate > 4V/s and impedance 1059 Ω.
An attempt to introduce a second lead (atrial lead) through the innominate vein failed because of the small vein diameter. Subsequently a screw-in, 59 cm length lead (Flextend®, model 4088, Guidant Corp.) was successfully advanced into the right atrium via PLSVC-coronary sinus and was screwed in its anterior wall. Stimulation threshold measured 1.5 V at 0.5 ms, P wave amplitude 2.5 mV, slew rate 0.5 V/s and impedance 832 Ω. The leads were connected to a CRT-D device (Contak® Renewal™ 4HE, model H199, Guidant Corp.), implanted in the left pectoral area. The defibrillation threshold test was not performed during the implant session. The following day, ventricular fibrillation induced by a T wave shock was successfully defibrillated with 21J. During 18 months follow-up atrial, RV and LV sensing and stimulation thresholds were excellent.


The patient is a 64 year old female who had a whole body FDG-PET scan in June 2004 as part of an employee wellness program. The scan was negative except for a focal abnormality in the cecal area which had an SUV of 8.9 (Figure ). The patient was completely asymptomatic. A colonoscopy performed two years earlier was normal.
Two months after the FDG-PET scan, a colonoscopy was performed. There was no visible abnormality in the cecum (Figure ) but prompted by the positive PET scan, a biopsy was obtained. During the biopsy, the gastroenterologist noted abnormal tissue consistency and suspected a blanket of polyps. Multiple biopsies were obtained although no gross abnormality was visible. The pathology results showed a villotubular adenoma without high grade dysplasia. An exploratory laparatomy was performed one month after the colonoscopy to remove the tissue with abnormal histology. Gross pathology showed an exophytic polypoid lesion in the cecal pouch, adjacent to the ileocecal valve. The lesion measured 4.5 × 3 × 0.5 cm. Microscopic examination revealed villous adenoma with focal high grade dysplasia, negative for invasive carcinoma (Figure ). Surgical margins and lymph nodes were free from dysplasia. A subsequent whole-body FDG PET/CT scan performed 10 months postoperatively was found to be normal.


A 24 year old gravida 5 woman with no history of alcohol or drug abuse, and no previous history of cardiovascular disease, presented to the emergency department (ED) 5 months after an uneventful full term spontaneous vaginal delivery followed by tubal ligation. She complained of a severe epigastric and right upper quadrant abdominal pain, nausea and vomiting. Physical examination revealed normal vital signs, epigastric and right upper quadrant tenderness without peritoneal signs. Abdominal ultrasound showed thickened anterior gall bladder wall without stones or sludge and no pericholecystic fluid. Liver enzymes and amylase were within normal limits. She was given analgesics and subsequently discharged from the ED following complete resolution of her symptoms. Three days later she returned with a severe, worsening right upper quadrant and epigatric pain, nausea and shortness of breath. She also complained of left lower extremity and back pains. At this time, her blood pressure was 130/100 mmHg with a heart rate of 100. She had bilaterally decreased air entry on lung examination, intermittent apical third heart sound with a regular rate and rhythm, right upper quadrant and epigastric tenderness, bilateral costovertebral angle tenderness and a negative Murphy's sign. An acute abdominal x-ray series was normal. Chest x-ray (Fig. ) showed moderate cardiomegaly and prominent bibasilar interstitial markings. Electrocardiography (Fig. ) revealed a normal axis with diffuse T-wave inversion. A transthoracic echocardiography (TTE) (Fig. ) revealed biventricular dilatation with a left ventricular end-diastolic dimension of 65 mm, a depressed fractional shortening of 9%, an ejection fraction of <15% and a left ventricular anterior wall thrombus. The patient was diagnosed with peripartum cardiomyopathy with congestive heart failure, liver congestion and a left ventricular mural thrombus. She was placed on bed rest with telemetry monitoring and started on therapeutic dose of low molecular weight heparin, nitrates, diuretics and an ACE inhibitor. Although, her congestive heart failure symptoms improved, her abdominal pain worsened. She had elevated lactic acid level (3.3 mmol/L) and white cell count (12,200/mm3), with no evidence of liver congestion on imaging. Right heart catheterization revealed normal cardiac output and right heart pressures with no evidence of intracardiac shunt, and a cardiac index of 2.6 L/min/m2. Computed tomography (CT) scan revealed findings suggestive of a biventricular thrombi and an 8 mm low attenuation lesion at the dome of the right lobe of the liver (Fig. ), multiple wedge defects in both kidneys (Fig. ), and a partially occlusive thrombus within the common iliac and right external iliac arteries. An arteriogram subsequently confirmed bilateral iliac and superficial femoral artery thrombi. Assays for IgG and IgM anticardiolipin antibodies were negative (6.8 GPL and <4.0 MPL respectively), and rheumatoid factor was less than 20 IU/mL. HIV I and II, hepatitis B and C, and RPR, were all negative and her ferritin level was within normal limits. She underwent successful bilateral lower extremity thrombectomies. Her renal function remained stable throughout her admission. She improved clinically on anticoagulation and heart failure therapy and repeat TTE 5 days after she presented showed no evidence of thrombi in either ventricle. Her lactic acid levels normalized on day 6 of hospitalization and she was discharged 2 weeks after she presented with follow up in the outpatient cardiology clinic.


A 58-year-old African American male was seen in hepatology clinic for a surveillance liver ultrasound following a diagnosis of hepatitis C. The patient had experienced mild intermittent upper abdominal pain consistent with a history of gastroesophageal reflux disease. He had no weight loss, change in appetite or bowel habits, jaundice, or general malaise. Past medical history included hyperlipidemia, peptic ulcer disease, and hepatitis C (antibody positive, PCR negative). The patient's physical examination and laboratory studies were otherwise normal. A surveillance abdominal ultrasound demonstrated a well circumscribed mass measuring 3.4 × 2.9 cm within the head of the pancreas. A computed tomography (CT scan) further confirmed an enhancing mass involving the head of the pancreas (Figure ). No evidence of extra-pancreatic disease was found on further evaluation. Although tumor-associated antigen levels (CEA, CA 19-9, and CA-125) were normal our differential diagnosis still included adenocarcinoma of the pancreas. Endoscopic ultrasound (EUS) guided needle biopsy was then performed but the pathological findings were non-diagnostic. Our concern for possible undetected malignancy remained and a diagnostic laparoscopy was performed followed by PD. The patient recovered well following the procedure and was discharged home on the 15th postoperative day. He continues to receive close outpatient surveillance without additional treatment.
Within the PD specimen, a protuberant 4 × 3 × 3 cm circumferential mass at the superior border of the pancreatic head was appreciated (Figure ). The external surface of the mass was tan/gray, firm, and smooth. Cut sections of the mass also revealed a partially encapsulated large nodule with tan/gray fleshy to firm parenchyma infiltrating the pancreas at its distal portion. Surgical margins were clear (Figure ). Regional lymph nodes were identified near the mass; microscopic examination revealed that all were hyperplastic lymph nodes. The remaining surgical margins were negative.
This lymphoid lesion within the pancreatic head showed marked vascular proliferation and hyalinization of germinal centers. Involuted germinal centers were surrounded by concentric rings of small lymphocytes. i.e. "onion-skinning" penetrated by hyalinized vessels (Figure ). Moreover, the interfollicular zones were rich in plasma cells and hyalinized vasculature. These features are consistent with localized hyaline-vascular type CD. Distribution of T and B cell markers (CD20, CD3, CD45) was normal and markers indicative of lymphoid malignancies (i.e. CD30, CD15, CD5, CD10, BCL-2) did not show evidence of such entities. Furthermore, PCR analysis of paraffin-embedded tissue revealed no clonal rearrangement of the immunoglobulin heavy chain or T-cell receptor gamma and beta chain genes. MALT1 (18q21) clonal rearrangement by FISH was negative. Together, these morphologic and immunohistochemical findings are most consistent with a diagnosis of CD.


We report the case of a 51-year old previously healthy immunocompetent Caucasian male who developed acute illness with fever, dyspnoea and expectoration of bloody sputum during a diving holiday in Croatia. The patient was admitted to a local hospital where empiric antibiotic therapy with azithromycin and ciprofloxacin for radiologically confirmed pneumonia was initiated. Before onset of the acute illness, symptoms suggesting previous (e.g. viral) respiratory tract infection were not observed. After 3 days, the patient was referred to our clinic via an ambulance air service.
Upon initial examination, the patient presented with severe sepsis including renal failure. Staphylococcal necrotizing pneumonia was diagnosed based on culture results (blood and broncho-alveolar lavage specimens), clinical criteria, and a high-resolution CT-scan demonstrating signs of severe pulmonary infiltration (Fig. , and Chest-X-ray: Fig. ). Extensive lung tissue necrosis was observed. The antibiotic regimen was changed to a combination of linezolid, imipenem and clindamycin. In correlation to the clinical presentation including high-grade fever [39.2 degrees celsius], laboratory assessment showed signs of severe inflammatory reaction (C-reactive protein 221 [mg/L], procalcitonin 12.4 [μg/L], white blood cell count 15.3 [×109/L], platelet count 182 [×109/L]. Over the ensuing days of treatment, physical examination and radiological imaging revealed abscess formation surrounding the right sternoclavicular joint. Later, progressive diffuse furunculosis next to the left clavicle (Fig. ) occurred.
Blood cultures and bronchoalveolar samples obtained under triple antibiotic therapy were repeatedly tested positive for methicillin-sensitive Staphylococcus aureus (MSSA). Because of the unusual severity of the staphylococcal infection, community-acquired Panton Valentine Leukocidin (PVL)-positive MSSA septic shock was suspected []. This was confirmed by PCR demonstration of the respective PVL-genes, as previously reported by us []. Furthermore, the isolates were subjected to surface protein A (spa) sequence typing []. All isolates investigated exhibited spa-type 284 which corresponds to multilocus sequence type ST121. This clonal lineage has been associated with furunculosis in the past decade in Europe and is associated to recent outbreaks [].
Within 6 days of triple antibiotic therapy with linezolid, imipenem and clindamycin, the patient developed progressive respiratory and haemodynamic failure including development of the Acute Respiratory Distress Syndrome (ARDS) requiring mechanical ventilation (FiO2 0.8, paO2 79 mmHg, PEEP 15 cmH2O, peak inspiratory pressure 33 cmH2O). Furthermore, vasopressor support was necessary for a subsequent period of more than two weeks. Ongoing abscess formation in multiple skeletal muscles (Mm. supraspinatus, triceps, quadriceps, gluteus maximus) required repeated surgical interventions and drainage despite organism-sensitive antibiotic therapy from the first treatment day (minimal inhibitory concentrations (mg/L): oxacillin and clindamycin both ≤ 0.25, linezolid ≤ 0.5). Several transesophageal echocardiograms never showed signs of infectious endocarditis. Assessment of an underlying immune disorder showed normal fasting serum blood glucose levels (before onset of severe sepsis), normal quantitative complement levels (C3, C4), and normal immunoglobulin (Ig) levels, including Ig-subclasses. Furthermore, flow assorted cell sorting (FACS) analyses were performed. These analyses revealed the presence of panlymphopenia during sepsis with a normal CD4/CD8 ratio. B- and T-cell numbers were found to be within the limits of normal after reconvalescence.
After two weeks of ongoing metastatic soft tissue infiltration under triple antibiotic therapy (linezolid, imipenem, clindamycin), the anti-microbial therapy was changed to a combination of daptomycin and clindamycin. Continued antimicrobial and surgical therapy finally led to a stabilisation of the patients' condition. The patient was discharged from hospital on day 40. Oral antibiotic treatment with moxifloxacin was prescribed for an additional 45 days. Twelve months after discharge, the patient was found to be symptom-free and to be able to perform routine activities of daily life. PVL testing of the patient's household members revealed no PVL colonization of the patient's family.


A 57-year-old woman (180 cm, 60 kg) was presented to the emergency department with upper abdominal pain and left-sided chest discomfort for a couple of days. In addition, she reported of nausea. Upon physical examination, percussion sounds over the left lower thorax were a bit dull and cervical lymph nodes were palpable with a size of up to 1 cm. The abdomen was generally tender without signs of peritonitis; bowel sounds were normal. The ECG showed no abnormalities. Routine laboratory tests revealed mild leucocytosis and a slightly elevated C-reactive protein level. Troponin, myoglobine, and creatine kinase levels were within normal limits. An upper gastrointestinal endoscopy was performed. Whereas the oesophagus, the lower stomach, and the duodenum were
without abnormalities, a small hole in the fundus was visible after inversion of the scope ().
The “hole in the stomach” was intubated with a catheter and contrast dye was administered. Fluoroscopy revealed a diaphragmatic hernia with a large herniation of the gastric fundus (). Immediate laparotomy showed a 3 cm orifice of the
diaphragm (). The orifice was widened and a partial necrosis of the incarcerated fundus was resected (). The diaphragmatic orifice was covered with polydioxanone. The patient recovered fully and
was discharged 12 days after laparotomy.


A 32-year-old Caucasian woman with BMI 46 presented to “A&E” with
dysuria, urinary frequency, and incontinence, along with bilateral loin
tenderness and vomiting. Her initial observations were as follows: pulse 135,
BP 151/114, and temp. 36.6. Urine dipstick revealed 3+ protein, blood,
leucocytes, and ketones. Liver and renal function tests, uric acid, amylase,
random blood sugar, and clotting screen were within normal range; Hb 10.0,
white cell count of 26.2 with Neutrophils at 23.32, platelets 454, and
Cßreactive protein >180 mg/L. Haematology report also showed microcytic
hypochromic blood picture. Midstream urine was sent for culture and sensitivity.
The immediate impression was that of acute pyelonephritis and treatment
commenced with IV Cefuroxime and surgical review requested. On abdominal
examination, a large, mobile, nontender mass was palpated with positive
urine pregnancy test.
Subsequently, transabdominal scan revealed a single live term fetus with
cephalic presentation, reduced liquor (amniotic fluid index of 5), effective foetal
weight of 3.9 kg,
and with right lateral placenta. She was then transferred to delivery suite.
This was her first pregnancy and both partners were unaware of the ongoing
pregnancy until diagnosed. She gave vague history of constant vaginal
discharge. Until then, she was on continuous contraceptive pills. Past medical
and surgical history suggested nothing significant, and there was no ongoing
psychiatric illness. Interestingly, she presented to general practitioner (GP)
at least twice with complaints of backache and urinary symptoms prior to
delivery. Although GP recommended
treatment for UTI with trimethoprim, she did not take them due to those
medications causing sickness.
Upon admission to delivery suite, her observations were pulse 127, BP
160/102, and temp. 37.6 with 3 in 10 uterine contractions. Also noted were 2+
generalised oedema, 3+ proteinuria, and normal reflexes. Cardiotocograph
reading showed baseline tachycardia at 170, variability <5, no accelerations,
and variable decelerations. Vaginal examination revealed cervix 5 cm dilated,
1+ caput, vertex −2 station, and malodorous vaginal discharge on rupture of
membranes. She underwent emergency caesarean section under GA for fetal
distress, severe chorioamnionitis, and moderate preecclampsia. There was delay
in starting the caesarean due to difficulty in securing the IV access, three
failed attempts at spinal, failed catheterisation as pus pouring from the
vagina, and due to body habitus.
A live male baby weighing 4400 grams was delivered and handed over to
paediatrician. Intraoperative findings revealed offensive liquor in the form of
frank pus draining from the uterus, J-shaped uterine incision due to difficulty
in delivering the shoulders, pus draining from foetal nose and mouth, normal
looking bilateral tubes and ovaries, and a corrugated catheter drain left in
the peritoneal cavity due to peritoneal contamination of uterine contents.
Vagina was swabbed and Foleys catheter was inserted into bladder. Estimated blood loss
was at 700 mL.
Placental and cervical swabs were taken for culture and sensitivity, and
placenta was sent for histology. Postoperatively IV Augmentin continued for 48 hours
and then oral for 21 days, Labetalol 200 mg orally twice daily for 2 weeks,
iron supplements for next 3 months, subcutaneous Tinzaparin 4500 IU for 5 days,
and leg stockings until she mobilised. She also received 3 units of blood transfusion
in view of her Hb 6.1 gm/dL. All her observations including haematological and
biochemical parameters improved over 72 hours. Drain removed on Day six. She
was rubella immune and negative for Hepatitis B, Syphilis, and HIV 1 and 2. No
pathogenic organisms isolated from urine specimen, placental, and cervical
swabs. Placental tissue on culture exhibited scanty growth of Pseudomonas aeruginosa sensitive to Ceftazidime, Ciprofloxacin, Gentamicin, Imipenem, and Tazocin. She
recuperated uneventfully and discharged home on Day 16.
The APGAR scores for the baby at 1, 5, 10, and 20 minutes were 2, 3, 5,
and 7, respectively. Baby was in poor condition at birth and had to be
resuscitated and admitted to Special Care Baby Unit. Arterial and venous blood
gas pH values were 7.217 and 7.130, respectively. Similarly arterial and venous
base deficits were −8.9 and −7.9 mmol/L. Baby developed septicaemia due to Staphylococcus
epidermis and were treated with antibiotics for seven days. There were
developmental delays and a diagnosis of Grade 3 hypoxic ischaemic encephalopathy
was made. Baby was discharged home on Day 22 after establishing bottle feeds.


A 64 year old woman presented to the Emergency Department with complaints of abdominal pain, nausea and an abdominal mass in the right upper quadrant. She had a history of excessive coughing due to acute bronchitis. Additionally she had diabetes mellitus and hypertension under medical therapy. She had a diagnosis of Polycythemia Vera for 10 years and had been taking an antineoplastic agent containing hydroxyurea 500 mg per day since then. Physical examination revealed a large mass in the right upper quadrant of the abdomen extending to the lower abdomen. The mass was tender on palpation, but there was no rebound tenderness and muscular rigidity. Carnett and Fothergill tests were positive. Bowel auscultation revealed no pathologic sound. Mean arterial pressure was 140/80 mmHg, pulse rate was 86/min, hemoglobin level 10.56 g/dL, hematocrit 37.3%, platelet 528000/UL, activated partial tromboplastin time (aPTT) 21.8 sec, prothrombin time (PT) 17.7 sec and international normalized ratio (INR) 1.45. The other biochemical tests were normal. Ultrasound (US) examination of the abdomen confirmed a nonmobile heterogeneous mass on the right upper quadrant extending below the liver with a suspect of intestinal loop within the mass. Further imaging study of computerized tomography (CT) revealed a hematoma on the abdominal wall (Figure ). Complete blood cell count examination on the 12th hour of admission revealed a decrease in the hemoglobin and hematocrit levels (7.63 g/dl and 22.6% respectively). Ecchymosis occurred on the third day on the abdominal wall. After intravenous fluid replacement, 3 units of erythrocyte transfusion, 2 units of fresh frozen plasma and analgesic treatment with strict bed rest, patient was discharged from the hospital on the 8th day of admission.


A 60 year old woman presented to the Emergency Department with complaint of acute abdominal pain. She had history of chronic constipation. Physical examination revealed a painful palpable mass within the left lower quadrant of the abdomen. There was no muscular rigidity or rebound tenderness. Mean arterial pressure was 110/80 mmHg, pulse rate was 76/min, hemoglobin level 10.46 g/dL, hematocrit 32.3%, platelet 199000/UL. The other biochemical tests were normal. US revealed suspect of invaginated intestinal loop with a sign of "Target sign" (Figure ), but the clinical examination did not confirm the prediagnosis of intestinal obstruction. CT revealed a rectus sheath hematoma. During the 3 day follow up, there was no change in the hemodynamic conditions, so no transfusion was required. Ecchymosis occurred on the third day on the abdominal wall. Partial resorption of the hematoma was observed in the follow up CT 45 days after discharge.


A 76 years old woman presented to the Emergency Department with complaints of abdominal pain, and an abdominal mass in the right upper quadrant. She had a history of congestive heart failure, chronic atrial fibrillation, chronic obstructive lung disease, ischemic cerebrovascular disease and myocardial infarction. The patient had been taking warfarin sodium 5 mg/day for atrial fibrillation for 2 years without medical supervision. On physical examination painful mass in the left lower quadrant and suprapubic region of the abdomen was observed. Ausculatation of the abdomen revealed hypoactive bowel sound. Mean arterial pressure was 140/80 mmHg, pulse rate 118/min, hemoglobin level 13.32 g/dL, hematocrit 30.26%, platelet 199000/UL, PT: 79.2 sec, aPTT: 56 sec, INR: 6.68. The other biochemical tests were normal. Abdominal CT revealed rectus sheath hematoma (Figure ). During the follow up period 6 units of fresh frozen plasma was administered. Due to the hemodynamic stability patient was referred to the Internal Medicine Department.