A 25-year-old female, gravida 10, para 0, artificial abortion 10 (G10P0AA10), had a history of dysmenorrhea with irregular duration and intervals of menstrual cycles. She had had no menses for two months before coming to the emergency department with the chief complaint of acute-onset abdominal pain and heavy vaginal bleeding with blood clots. She had neither dizziness nor shortness of breath. Her body temperature (BT) was 37.3°C, heart rate (HR) was 92 times/minute, respiration rate (RR) was 18 times/minute, and blood pressure (BP) was 100/60 mmHg. The pregnancy- enzyme immunoassay test was negative, and beta-HCG was 13.11 mIU/mL, which was in the normal range. In the emergency department, her serum hemoglobin dropped from 13.8 mg/dL to 11.9 mg/dL in 6 hours. The gray-scale ultrasonography revealed multiple varying-sized hypo-echoic lesions (). The color Doppler ultrasonography showed a hypervascular lesion, 4.9 × 3.3 × 2.6 cm with multidirectional turbulent flow (). On admission, the patient was scheduled for angiography and TAE. The angiography showed the feeding artery from the branches of the right internal iliac artery and tortuous veins with relatively early venous return (), which indicated the diagnosis of uterine AVM. Superselective TAE of the right uterine artery using N-butyl cyanoacrylate (NBCA) was performed at first. On checking the left uterine artery, the angiography showed that there were still a few abnormal collateral vessels (). Therefore, bilateral uterine artery embolization was performed (). After the interventional treatment, the patient had mild dull pain in the lower abdomen, which was most likely related to ischemia, and could be relieved by oral non-steroidal anti-inflammatory drugs (NSAIDs). There was no more vaginal bleeding during hospitalization, and she was discharged 2 days after interventional treatment. Forty days after TAE, her menstrual bleeding started, and the follow-up ultrasonography showed no abnormal flow.


A 38-year-old female, G3P0AA3, with a history of regular 30-day menstrual intervals and 4 - 5 days duration of flow visited our outpatient department with the chief complaint of hypermenorrhea and intermittent severe abdominal pain for 12 days. She was in her usual healthy status until 12 days before when she used a massage machine on her belly to relieve constipation. Then she noted intermittent, severe, and dull lower-abdominal pain accompanied by nausea. The next day the vaginal bleeding started, which she thought was normal menses. Over the following 10 days, she found the pain diffused progressively; there was neither relief nor aggravating factor. The vaginal bleeding was persistent with blood clotting. She had no fever, dysuria, urinary frequency, or purulent vaginal discharge. According to the medical record at outpatient department, her temperature was 36.8°C, heart rate was 84 times/minute, respiratory rate was 20 times/minute, and blood pressure was 120/72 mmHg. The abdomen was soft with tenderness, but there was neither rebounding pain nor muscle guarding. The beta-HCG was in the normal range. The gray-scale ultrasonography revealed hypo-echoic cyst-like lesions (). The color and spectral Doppler ultrasonography demonstrated a hypervascular lesion in the body of the uterus, 2.7 × 2.4 × 2.1 cm with multidirectional turbulent flow, and the resistive index (RI) was 0.4 (). The angiography and TAE were arranged afterward. The angiography clearly showed the vascular tangles from the branches of the bilateral internal iliac arteries (). TAE was performed with two microcatheters placed in the bilateral uterine arteries. We injected NBCA slowly till stasis, and the vessels revealed occlusion (). After TAE, the patient had severe abdominal pain and received patient-controlled analgesia for pain control. Because her pain lessened and her condition became stable, the patient was discharged 4 days after treatment. The follow-up color Doppler ultrasonography, which was done 10 days after TAE, confirmed great improvement (). Thirty eight days later, she started normal menstrual periods.


A 22-year-old female, G2P0, spontaneous abortion 1, artificial abortion 1, with regular menstrual cycles in the past had threatened abortion 2 months before she came to outpatient department due to prolonged menstrual flow for 2 weeks with sudden heavy vaginal bleeding. She also noted dizziness and fatigue for days without dyspnea. Her temperature was 36.1°C, heart rate was 76 times/minute, respiratory rate was 18 times/minute, and blood pressure was 116/70 mmHg. The beta-HCG and CA-125 were in the normal range. Gray-scale ultrasonography revealed multiple hypo-echoic myometrial lesions (). Color and spectral Doppler ultrasonography showed a hypervascular lesion, 3.0 × 2.7 × 1.7 cm with multidirectional turbulent flow (), and RI was 0.3. Angiography showed multiple abnormal vessels from the bilateral uterine arteries, particularly from the right side, and rapid venous shunting (). TAE was performed using NBCA to obstruct the bilateral uterine arteries ( and ). After TAE, she complained of severe lower abdominal pain, which was refractory to demerol. Patient controlled analgesia was used and the pain subsided. She was discharged 3 days after treatment. She had menstruation at 65 days after TAE. Three years later, she was admitted to our hospital for normal spontaneous delivery with a gestational age of 39 weeks. The baby girl was healthy, Apgar scores were 9/9, and body weight was 3020 gm. The placenta was delivered by complicated manual removal with both surface (Duncan and Schultze), but there was no gross abnormal finding, including cord abnormalities. Her prenatal examination was uneventful, and there were no complications during delivery or the postpartum period.


A 28-year-old male with PDR, fibrovascular proliferation, and macular edema in both eyes complained of sudden-onset blurred vision in his left eye (OS). The patient had been treated over a 2-year period with focal and scatter laser photocoagulation as well as intravitreal bevacizumab therapy to treat both the proliferative retinopathy and macular edema. Two weeks prior to the onset of his new symptoms, the patient had a best corrected visual acuity (BCVA) of 20/40 OS and a new mild vitreous hemorrhage due to PDR in his left eye which was treated with an intravitreal bevacizumab injection. Examination on the day of the patient's new symptoms revealed a BCVA of 20/50 OS. Spectral domain optical coherence tomography (OCT, Spectralis, Heidelberg Engineering, Heidelberg, Germany) showed a taut anterior-posterior nasal vitreomacular membrane and an adjacent FTMH, measuring 363 µm at its base (fig. ). The patient was started on topical bromfenac 0.9% OS every 12 h (b.i.d.) for adjunctive retinal edema management. Eight weeks later, the patient's BCVA improved to 20/30 OS and OCT examination revealed spontaneous closure of the macular hole and persistent VMT in the nasal macula (fig. ). The macular hole continues to remain closed 9 months following spontaneous closure.


A 30-years old African American male, with no medical comorbidities, presented to the emergency department after being involved in a high-speed motor vehicle collision. Radiographic assessment of the pelvis revealed a left obturator dislocation of the femoral head (). Closed reduction was performed successfully in the emergency department. Radiographs and CT scans identified a 25 x 10-mm osteochondral defect of the posterosuperior quadrant of the femoral head near the head-neck junction with an impaction depth of 5 mm. In addition, a small, comminuted, minimally displaced anterior acetabular wall fracture was also identified but was not amenable to fixation (Figs. - and ).


A 32-years-old male presented with two months history of gait ataxia. On examination right hemiparesis (4/5, MRC grading), left VII nerve paresis (H and B grade III) and right cerebellar signs were present. Rest of the neurological examination was normal.
Head CT revealed a heterogeneous solid cystic mass arising from the brainstem and extending to right cerebello-pontine angle []. MRI revealed a right middle cerebellar peduncle lesion (33 × 30 × 28 mm) extending to right cerebello-pontine angle with necrotic and hemorrhagic areas. The lesion was isointense on T1W, hyper intense on T2W with inhomogeneous postcontrast enhancement [Figure –]. There was mild mass effect over fourth ventricle, but no upstream hydrocephalus.
Right retro-sigmoid suboccipital craniotomy and near total excision of tumor was done. Intraoperatively, tumor was soft, greyish, highly vascular arising from the right middle cerebellar peduncle, infiltrating surrounding neurovascular structures, without any plane of cleavage.
Patient was electively ventilated for 48 hours. Postoperative course was uneventful except for mild impairment of gag reflex, for which nasogastric feeding was started. Patient was discharged on 7th postoperative day.
The tumor tissue was fixed in neutral-buffered formalin and was routinely processed and paraffin embedded. Five-micrometer sections were cut for routine Hematoxylin and eosin staining and for immunohistochemistry.
Microscopic examination showed a malignant tumor arranged in sheets comprising of round to oval to spindle cells with scant amount of cytoplasm and vesicular nuclei with prominent nucleoli. Mitoses were frequent []. Some of the cells had more eosinophilic cytoplasm. The tumor cells showed diffuse immunopositivity for synaptophysin, chromogranin and focal immunopositivity for desmin and myogenin [Figure –]. MIB-1 labeling index was 6%. The tumor cells were immunonegative for p53, GFAP, epithelial membrane antigen (EMA), pancytokeratin (CK) and TTF-1.
Based on above morphological and immunohistochemical features the possibility of medullomyoblastoma was considered.
At last follow-up, his lower cranial nerve function had improved and he was able to take food orally. MRI done 6 weeks after surgery showed no evidence of residual/recurrent tumor []. Patient is now undergoing radiotherapy.


A 22-year-old male presented with a subcutaneous nodule in his right thigh of one and a half years duration which was gradually increasing in size and was noticed after a history of minor trauma. One week back, the nodule had ulcerated with associated pain. There was no other associated significant present or past history. On examination, a subcutaneous nodule was seen over the posterior aspect of mid portion of thigh measuring 2.5 × 2.0 × 1.5 cm. The swelling was firm and freely mobile over the underlying tissue. The overlying skin showed an ulcer measuring 1.0 × 0.8 cm with the rest of the skin showing a bluish hue. Rest of the skin examination showed no evidence of other subcutaneous nodules or any pigmented lesion. Examination of regional lymph nodes was unremarkable. Routine hematological and biochemical investigations done including a peripheral blood smear were within normal limits. With a preoperative diagnosis of hemangioma, the patient was taken up for wide local excision of the lesion. We received a wide local excision specimen measuring 3.0 × 2.5 × 1.5 cm with an overlying skin cover measuring 3.0 × 2.5 cm. The skin showed a bluish hue and an ulcer measuring 1.0 × 0.8 cm []. On cut surface, a jet black unencapsulated tumor was seen with small tongue like projections in the surrounding tissue []. Microscopic examination revealed a tumor, over lined by epidermis, showing focal ulceration along with native adnexal structures []. The deeper dermis showed a tumor arising from the nerve []. The tumor was heavily pigmented and was composed of hypercellular and hypocellular areas []. The hypercellular areas showed spindle shaped cells arranged variably in long and short fascicles and theques with pallisading of nuclei [Figure and ]. These cells had spindle shaped nuclei with evenly dispersed chromatin and indistinct nucleoli along with little amount of fibrillary cytoplasm. Also seen were large ganglion cells with abundant pigment layden cytoplasm with single to multiple nuclei showing vescicular nuclear chromatin and prominent nucleoli. Hypocellular areas were myxoid with few hyalinized blood vessels. Deeper part showed small foci of invasion into the surrounding soft tissues. No Verocay bodies were noted. There was no evidence of junctional activity or progressive dermal maturation. There was no evidence of atypia, mitoses or necrosis in the tumor. No psammoma bodies or sheets of mature adipose tissue were seen. The sections from the tumor were reviewed after depigmentation with 0.5% potassium permanganate (KMnO4) and the findings were confirmed. Positivity with Masson's Fontana (MF) stain and negativity with Periodic Acid Schiff (PAS) and iron stains confirmed the melanin nature of the pigment. Immunohistochemical marker study was done after depigmentation of the section which revealed positivity with S-100, HMB-45, and Melan A, and negativity with cytokeratin (CK) and Epithelial Membrane Antigen (EMA) [Figure , ]. Reticulin stain and Laminin immunostain revealed a biphasic staining pattern with prominent areas of pericellular staining. Proliferattion markers including Ki67 and p53 were done which revealed positivity in <1% of cells. With the above findings, diagnosis of non psammomatous melanocytic schwannoma was offered. Since some cases of MS are associated with Carney's syndrome, a detailed mucocutaneous examination was performed to rule out lentigines, an echocardiogram was performed to rule out myxomas and biochemical evaluations were done to rule out endocrine hyperactivity. All these investigations were negative and hence Carney's syndrome was ruled out. Since our patient showed ulceration of overlying skin and focal invasion in deeper portions of the tumor, he was advised for close follow-up and adjuvant radiotherapy. However, our patient did not show any evidence of recurrence or metastasis after a period of 10 months.


A 50-year-old woman who had undergone uneventful cataract surgery with intraocular lens (IOL) implantation in her left eye 1 year back was planned for small incision cataract surgery (SICS) with IOL implantation in the right eye under peribulbar anesthesia. Her systemic hypertension was well-controlled. A test dose of lignocaine was given 1 day before surgery which did not show any hypersensitivity reactions.
On the day of surgery, the patient developed lid edema with tight lids, conjunctival chemosis, and axial proptosis immediately after peribulbar injection (3 ml of 2% lignocaine + 1 ml of 0.5% bupivacaine + 1500 IU of hyaluronidase). The peribulbar block was given with a 26-gauge needle in the inferotemporal orbit. A diagnosis of peribulbar hemorrhage was made; the surgery was deferred, and the patient was managed with intravenous dexamethasone and mannitol, oral acetazolamide, and cold compress.
The chemosis and periorbital edema had partially resolved by 24 h. However, at 48 h postperibulbar injection, she developed increasing proptosis, lid edema, and tight eyelids with increasing chemosis []. Anterior segment examination under slit lamp showed a brisk pupillary response. The posterior segment revealed no abnormalities. In view of increasing proptosis, orbital cellulitis was suspected, and the patient was started on intravenous cefotaxime although the patient was afebrile and had no systemic symptoms. Computerized tomography (CT) scan of orbit was planned.
In view of atypical symptoms of absence of systemic signs, a review of literature was done which prompted us to consider a diagnosis of delayed hyaluronidase allergy. An intradermal test with bupivacaine, lignocaine, and low dose of hyaluronidase 15 IU/0.1 ml was done. There was no reaction at the site of lignocaine and bupivacaine injection, but the patient developed an immediate hypersensitivity response in the form of erythema and wheal within a few seconds that disappeared in a minute, thus confirming the diagnosis of hyaluronidase hypersensitivity []. She received intravenous dexamethasone 8 mg immediately followed by oral prednisolone 60 mg which was tapered over 2 weeks. She responded well to steroids with rapid resolution of lid edema and chemosis by 24 h prompting us to stop intravenous antibiotic and continue only oral steroid. CT scan of the orbit was not performed. Complete resolution of the proptosis was observed by 4 days [].
She developed a delayed hypersensitivity response with induration (10 mm × 12 mm) at the site of intradermal injection after 48 h which was treated with local application of hydrocortisone cream. This established the diagnosis of both immediate and delayed hypersensitivity to hyaluronidase in this patient. She underwent an uncomplicated phacoemulsification with foldable IOL implantation under topical anesthesia 1-month later.


A 45-year-old man was transferred to the emergency room with altered mental status after he was discovered lying on the ground. On physical examination, he showed a flexion response to pain stimuli in all extremities. His pupils were isocoric and demonstrated a normal light reflex. The initial Glasgow Coma Scale revealed E1V1M3. His blood pressure, pulse, respiration rate, and body temperature were 190/100 mm Hg, 119/min, 30/min, and 35℃, respectively. On laboratory examination, white blood cell, hemoglobin, hematocrit, and platelet count were 5290/mm3, 10.6 g/dL, 28.7%, and 82000/mm3, respectively. Serum blood urea nitrogen was 53 mg/dL (normal 10-38), and creatinine was 5.7 mg/dL (normal 0.6-1.2). Serum sodium was 108 mmol/L (normal 135-145), serum potassium was 3.2 mmol/L (normal 3.5-5.3), and chloride was 66.7 mmol/L (normal 98-110). The serum osmolality was 234 mOsm/kg (normal 185-295), and the blood sugar level was 89 mg/dL. Arterial blood gas analysis showed metabolic acidosis. His family disclosed that the patient had a history of heavy alcoholism.
The initial brain CT scan showed a traumatic epidural hemorrhage in the right temporal region (). As the patient was severely dehydrated and undernourished, he was started on hydration with normal saline (120 cc/hr), aiming for a sodium level of 120 mmol/L. Although his renal function recovered and his vital signs stabilized, his mental status did not improve. We performed a craniotomy to evaluate the hematoma and placed an intracranial pressure monitoring system at that time. Serum sodium and potassium were 121 and 2.5 mmol/L 24 hours after initial presentation, respectively. On the second day postoperatively, his mentation recovered to obeying to verbal commands. Serum sodium and potassium were 130 and 2.4 mmol/L, respectively. On the 4th day postoperatively, the patient was found to be stuporous. Serum sodium and potassium were 145 and 2.8 mmol/L, respectively. Brain MR images showed multiple contusions in the right temporal and left frontal regions (). Electroencephalograghy findings were consistent with diffuse cortical dysfunction without epileptic discharge. The patient's mental status did not improve any further. On the 10th day postoperatively, diffusion weighted imagery showed high signal intensity with decreased apparent diffusion coefficient values in pons and both thalamus and frontoparietal cortex. These lesions showed high signal intensity on T2-weighted images. This finding suggested a diagnosis of pontine and extrapontine myelinolysis (). Daily levels of serum sodium and potassium are shown in . The patient was gradually weaned from the ventilator. His eyes spontaneously opened, but there was no improvement in cognition or lower cranial nerve palsies. He was fully dependent on a caregiver's support at the 6-month follow-up.


A 46-year-old male presented to our hospital with a Glasgow coma scale score of 3 and an open head injury. His son found him at home in possession of a nail gun. The patient was in a drunken state. Neurological examination revealed grade 0 motor reflexes; light pupillary reflex: 3 mm fix/5 mm fix and a comatose mental state. The scalp was ripped by the nail and the brain was exposed.
Brain computed tomography (CT) revealed comminuted fracture of the left temporal bone with inward displacement of bony fragments and foreign body in the suprasellar cistern to the right medial temporal lobe, with intracranial hemorrhage in the left temporal lobe along the moving pathway of the foreign body (). His vital signs were very unstable, and required orotracheal intubation with mechanical ventilator application. The patient was transferred to the intensive care unit, after initiation of antibiotic therapy. Surgery was planned for the removal of the penetrated nail, after the vital signs were stabilized. Preoperative CT angiography revealed no gross evidence of vascular injury ().
General anesthesia was employed. The patient was positioned supine with the head turned to the right on a horseshoe-shaped head holder. Intra-operatively, we found burned scalp (1st degree) and torn dura. We removed multiple fractured temporal bony fragments. We attempted cerebral endoscopy for removal of the nail, after bleeding control. However, continuous minimal bleeding blocked the operative sight. The nail was found by stylet with assisted neuronavigation (Medtronic StealthStation® S7™, Louisville, CO, USA) and removed with forceps (). A drainage catheter of intracerebral hematoma was placed and dural closure was accomplished with Durafoam™ (Codman, Wokingham, UK). Greenplast™ (Greencross, Seoul, Korea) were applied to prevent cerebrospinal fluid leakage.
The patient's vital signs stabilized after removal of the nail. Neurological examination revealed Grade 3 motor reflexes; light pupillary reflex: 6 mm fix/6 mm fix; and a semicomatose mental state. Hydrocephalus had developed a month later. After ventriculoperitoneal shunt operation (), neurological examination revealed Grade 3 motor reflexes; light pupillary reflex: 5 mm fix/5 mm fix; and stuporous mental state with a Glasgow coma scale score of 6.


A thirteen-year-old girl was admitted to our medical center due to a traffic accident. She was in a stuporous state, and Glasgow coma scale was checked by E2V1M4. We immediately checked brain computed tomography (CT). On the initial brain CT, a small amount of acute SDH and anterior falx SDH were seen on the right convexity (). She was admitted to the general ward and was diagnosed of SDH combined with diffuse axonal injury. We carefully observed her, and her mental state was improved to an alert state one week after the initial trauma. Her follow-up CT, which was performed 1 week after the initial trauma, was shown to be clear of hemorrhage (). Her general condition and mental state recovered as time passed. But ten days after the initial trauma, she suffered a sudden attack of seizure and her mental state decreased to a comatose. On follow-up brain CT, a reappearance of convexity and falx SDH showed with severe and diffuse brain swelling (). There was no subarachnoid hemorrhage. Immediate decompressive craniectomy and hematoma removal were performed. One day after the operation, we performed an angiography to identify the existence of traumatic pseudoaneurysm. From angiography, we found the traumatic pseudoaneurysm in A2 artery. For her previous craniectomy, anti-thrombotic agent could not be treated premedication. Eleven days after the initial trauma, endovascular embolization was performed (). During the operation, stent insertion was preferentially done for instability of vessel. Coil packing for aneurysm was also performed. After endovascular embolization, we administered the patient with anti-thrombotic agent, aspirin, and plavix. On 1 year postoperative follow-up angiography, there was no recanalization at embolized traumatic pseudoaneurysm and her neurological symptom was improved to alert state ().


A 15-month-old female child presented with high-grade fever and progressive bilateral leg weakness with difficulty in balance for 2 weeks. There was no history of trauma, and the birth history was unremarkable. The patient was the first child of consanguineous parents.
On clinical examination, power in the left lower limb was 3/5 and 4/5 in the right lower limb. Examination of the back revealed a very small, abnormal tuft of hair in the midline in the mid lumbar region suggesting the presence of occult spinal dysraphism []. Thorough examination of the tuft of hair did not reveal any apparent sinus opening.
Magnetic resonance imaging (MRI) of the brain and full spine was carried out which revealed the presence of two Type 1 SCM in the thoracolumbar regions. The malformation started as a Type 1 SCM from T4 to T6, separated by a bony spur. The two hemicords then entered the same dural sheath but before uniting into a single, normal spinal cord, divided into another Type 1 SCM from T9 to T11, separated by another bony spur. The two hemicords then fused into a single dural sheath before continuing as a single spinal cord from T12 onward []. The MRI also showed a DST at L1 [] and an epidural abscess from T12 to L3 level []. Preoperative workup revealed C-reactive protein (CRP) of 2.8. The child was also evaluated for other anomalies, and computed tomography scan of kidney, ureter, and bladder revealed a duplicate collecting system with two separate renal pelvises and ureters in the right kidney.
The child underwent surgery through a limited exposure, incorporating the tuft of hair. Upon dissecting the lumbar fascia, a thick-walled, pearly white, elongated sinus tract was soon visible []. The tract was traced to the dura, where it fused onto it, communicating directly with the intradural space. The entire tract was excised, including the attached dura of the thecal sac, which was then primarily repaired. The extradural space showed extensive purulent collection along with long (2–3 cm) strands of thick hairs and sebaceous material. The extradural space was further explored through one level laminectomy and was extensively irrigated until the irrigant appeared clear and no further hairs could be delivered. The DST and dermoid cyst were distant from either of the SCM. Samples were sent for culture, sensitivity, and histopathological analysis. No attempts were made to relieve the SCM at this stage.
Culture from the pus showed growth of Staphylococcus aureus which was also resistant to all beta-lactams. Histopathology revealed a sinus tract with collagenous tissue without any lining, along with separate keratin flakes and a small bony chip. The cystic lesion showed skin tissue lined by keratinizing squamous epithelium. The lumen had keratin flakes and a granular layer was seen. Focally, hair follicles were also present. However, no other germ cell component was seen. The features favored those of a dermoid cyst. The child made an uneventful postoperative recovery and was kept on broad-spectrum antibiotics which were then changed to specific Gram-positive coverage, extending to a full 2 weeks of administration. No new neurological deficits were noted, and she was subsequently discharged. At subsequent follow-ups, the child regained full function in both the lower limbs.
Upon completion of her antibiotic course and normalization of CRP, she was readmitted 4 weeks later for elective repair of her multilevel SCM. Both malformations were approached through a single linear midline incision, and after respective laminectomies, the bony spurs at each level were individually drilled and removed. The operative findings correlated with the MRI interpretation and in between the two Type 1 SCM, there existed a single dural sheath. The canal was spacious and no attempts were made to reconstruct the two dural sheaths into one, at either level. The child once again made an uneventful recovery.


A 45-year-old Hispanic female patient with a history of multiple episodes of recurrent diverticulitis, irritable bowel syndrome, and endometriosis presented for an elective laparoscopic-assisted sigmoid colon resection. She had a BMI of 28.0, and she had no known underlying lung conditions or diseases. Past surgical history included a cesarean section and laparoscopic hysterectomy with endometrial excision occurring many years ago. She was not on any prescription medications, had no known drug allergies, and was a lifelong nonsmoker and a nondrinker.
A four-port laparoscopic sigmoidectomy was performed with pneumoperitoneum introduced directly through a 5-mm Endoview catheter and maintained in the range of 12–15 mmHg as per protocol. The sigmoid colon revealed evidence of chronic diverticulitis with adhesions to the left pelvic side wall and a subacute inflammatory mass. An intracorporeal colorectal end-to-end anastomosis (EEA) was constructed without difficulty, and no leak was demonstrated with an air infusion test. The patient was returned to the Post-Anesthesia Care Unit for recovery in stable condition.
The patient’s initial post-operative course was unremarkable. She was entered into a routine enhanced recovery protocol including early ambulation and feeding. Analgesia was obtained with intravenous acetaminophen and non-steroidal inflammatories, limiting the use of parenteral narcotics. Deep vein thrombosis prophylaxis was provided with subcutaneous heparin and intermittent compression stockings.
On the second post-operative day, it was noted that her PO2 dropped to 89%, which responded well to 2 L of nasal oxygen. Early in the morning of the third post-operative day, the patient suddenly became tachycardic and developed a temperature of 102.0°F. Her white blood cell count was 16,600 at that time. Because of the sudden quick change in her status, the patient was sent for an emergent computed tomography (CT) of the abdomen and pelvis, which revealed no evidence of an acute intra-abdominal process; however, a large right pneumothorax was identified (). A pigtail catheter was placed in the right pleural space under sedation and local anesthesia with immediate resolution of the patient’s symptoms. A post-procedure chest X-ray revealed expansion of the right lung ().
Blood and urine cultures were negative. The patient was then discharged on the sixth post-operative day, tolerating a regular diet without any respiratory complications, on oral amoxicillin/clavulanate and with instructions to follow-up at outpatient care.


A 42-year-old male presented for evaluation of refractory hypertension, with which he had struggled since the age of 15 years. He reported intermittent episodes of flushing, diaphoresis, anxiety, and headache. Blood pressure was 148/82 and heart rate was 94 beats per minute on a regimen of hydralazine and atenolol. On examination, there was notable fullness in the neck bilaterally, especially along the lateral aspects, although no discrete mass was palpable. Laboratory evaluation revealed markedly elevated 24-h urine normetanephrine and total vanillylmandelic acid. Levels of electrolytes, thyroid stimulating hormone, parathyroid hormone, and calcitonin were all within normal limits. Both computerized tomography (CT) and magnetic resonance imaging (MRI) of the neck showed bilateral carotid body tumors about 3 cm in size each, partially encasing both external carotid arteries ( and ). There was also evidence of a 1.2 × 1.1-cm2 PGL of the vagus nerve (i.e. glomus vagale). CT-abdomen showed three separate left adrenal masses and two right adrenal masses, measuring between 1.5 and 3.9 cm (). A nuclear medicine I-123 meta-iodobenzylguanidine (MIBG) scan showed increased tracer uptake in both adrenal glands, consistent with bilateral PCCs (). Within 1 month of diagnosis, the patient was started on phenoxybenzamine and subsequently underwent bilateral laparoscopic adrenalectomy. Pathology confirmed bilateral multinodular PCCs with focal vascular invasion, but lymph nodes were free of involvement. His blood pressure normalized, and he was discharged on hydrocortisone and fludrocortisone. Metanephrines normalized after adrenalectomy, and the patient no longer required any antihypertensives. Several months after adrenalectomy, the patient underwent resection of both PGLs, which were nonsecretory and pathologically confirmed to be PGLs. On recent follow-up, his blood pressure remains well controlled, and repeat metanephrine levels are within normal limits. Follow-up CT-chest/abdomen/pelvis shows no evidence of PCC recurrence. Repeat CT-neck shows no recurrence of bilateral carotid body PGLs, and the glomus vagale remains stable in size.
The patient underwent genetic counseling and testing. He tested negative for the MEN2a/b, VHL, and NF1 mutations but further testing revealed a novel mutation of the SDHD gene involving a substitution of thymine to guanine at nucleotide 236 in exon. Among hundreds of observed SDHD mutations, this particular mutation was identified only in one previous patient in Japan who was affected with the PGL/PCC syndrome, although details about that patient’s presentation are not available.
The patient’s first-degree family members were evaluated; his father had passed away and was not available for gene testing but his brother tested positive for the same SDHD gene mutation. He also had multiple PGLs including a right adrenal PCC, left peri-adrenal PGL, left peri-aortic PGL, and bilateral carotid body PGLs. The PCC and abdominal PGLs were first resected followed by the right carotid body PGL; the left carotid body PGL was left intact due to its sub-centimeter size and nonfunctioning status.
The University of Pittsburgh Medical Center does not require ethics approval for reporting individual cases. The patients provided written informed consent for their information and images to be included in this article and for publication in an international medical journal.


A 55-year-old postmenopausal female patient presented with mass per abdomen since 2 years and pain for 2 days. There was no history of bleeding per vagina. No signs of virilization were noted clinically. Per-abdomen tender mass was felt in left hypogastric and iliac fossa. Ultrasonography showed multicystic lesion measuring 12 cm × 11 cm in the left ovary []. Intraoperatively, left ovarian cyst showed torsion with rupture of cyst wall. Total hysterectomy with bilateral salpingo-oophorectomy was done.
On gross examination, ovary weighed 550 g, measured 13 cm × 11 cm × 7 cm, showed solid and cystic areas on cut section []. Microscopy showed round to solid tubules lined by pseudostratified columnar epithelium with elongated nuclei resembling sertoli tumor-like pattern along with conventional endometrioid tumor [Figures –]. Utero-cervix and rest of adnexa were unremarkable. A histopathological diagnosis of SEC of left ovary was made.
Immunohistochemistry (IHC) showed tumor cells strongly immunoreactive for epithelial membrane antigen (EMA), cytokeratin (CK) but negative for inhibin [Figures –], thus confirming the diagnosis of SEC of left ovary.


A 26-year-old woman with a large secundum atrial septal defect and mild pulmonary hypertension was evaluated for transcatheter closure. Transesophageal echocardiography revealed a large defect (31 mm × 29 mm × 30 mm) with deficient aortic rim [Figure and ]. The patient was taken to the catheterization laboratory for transcatheter closure under general anesthesia.
The maximum defect size on balloon sizing using stopped-flow technique was 33 mm, and a 36 mm Figulla Occlutech device (Occlutech GmbH, Jena, Germany) was selected. A 16 F Cook sheath (2F larger than the manufacturer's recommendation) was taken to ensure easy manipulation of a large device. The device was loaded onto the delivery system and delivered across the defect using the right upper pulmonary vein technique. The device, however, did not align the septum and protruded through the defect. As the device was being pulled into the sheath, it became obvious that it had not been locked properly at the time of loading and embolized into the right ventricle [].
A 15 mm Amplatz gooseneck snare (eV3 Endovascular Inc., Plymouth, MN, USA) was introduced via the same 16F sheath and the device was grasped by its screw on the right atrial disk. Once held, the snare was pulled with a constant gentle pressure and the device was brought into the right atrium []. A multipurpose catheter was passed from left femoral vein to orient the device so that the screw on the disc becomes coaxial with the snare []. We tried pulling the device into the sheath but the screw slipped out of the snare and device embolized to right ventricle again. We recaptured the device in the same manner and brought it back into the right atrium. This time instead of pulling it into the sheath, we lowered the sheath down into the inferior vena cava and pulled the bare right atrial disc into the inferior vena cava []. This served as a good stabilizing maneuver but still not good enough to slenderize the entire device into the sheath. A second smaller snare (5 mm) was passed through the same sheath and the large snare was pushed upwards over the slenderized right atrial disc while small snare was used to hold the right atrial screw firm []. The entire device could now be slenderized and pulled into the sheath and successfully removed []. Balloon assisted technique was used and the same 36 mm Occlutech device was successfully deployed [Figure and ]. The patient was discharged 24 h later on Aspirin (5 mg/kg/day) with check echocardiogram showing an optimally placed device and no evidence of any complications related to embolization or retrieval. On follow-up at 6 weeks, the device was stable with no residual shunt and reduced right ventricular volume.


A 5-year-old male with a history of complex congenital heart disease that included heterotaxy, dextrocardia with atrial and visceral situs inversus, unbalanced atrioventricular canal (AVC) with mildly hypoplastic left ventricle, ventriculoarterial concordance with aortic atresia and arch hypoplasia, left-sided superior vena cava (SVC), and interrupted left-sided IVC with azygos continuation [] presented to our institution with hypoxia, persistent cough, and respiratory distress. His prior palliative procedures included Norwood/Sano procedure as an infant followed by a Kawashima repair at 6 months of age, with subsequent lateral tunnel fenestrated Fontan completion using Gore-Tex material at 2.5 years of age. Chylous effusions complicated his postoperative course and he subsequently had a right pulmonary artery stent placed. At 4.5 years of age, he underwent cardiac catheterization and fenestration closure with a 4-mm Amplatzer septal occluder (ASO) (St. Jude Medical, St Paul, MN) and occlusion of an aortopulmonary collateral using an Amplatzer vascular plug II.
Shortly after the fenestration closure, the patient began having respiratory symptoms consistent with plastic bronchitis but the family had relocated and had no cardiology follow-up for several months. Six months after the closure, he presented to our institution with plastic bronchitis, on medical therapy including steroids, beta-agonists, and chest physiotherapy. In the interim, the patient had been hospitalized twice with hypoxia, respiratory distress, persistent cough, and intermittent facial edema. Chest X-ray showed prominent perihilar markings, bilateral small pleural effusions, and mild pulmonary edema. His initial echocardiogram at the time of presentation demonstrated mildly diminished ventricular function with an ejection fraction (EF) of 53%, mild AV valve regurgitation, and a widely patent lateral caval Fontan connection and superior cavopulmonary connection. The ASO device appeared to be in good position with no residual shunting. The device had been in place for too long to consider device retrieval. Therefore, he was referred for cardiac catheterization to create a Fontan fenestration.
Cardiac catheterization was performed under general anesthesia. The interrupted IVC with azygos continuation left transhepatic and transjugular access as two primary considerations for vascular access. Transhepatic approach was eliminated in this case because of a right dominant hepatic venous system leaving transjugular approach as the only available approach.
Baseline mean Fontan pressure and transpulmonary gradient were 17 mmHg and 10 mmHg, respectively. The transpulmonary gradient was measured using mean Fontan pressure and pulmonary capillary wedge pressure (17-7 = 10 mmHg). Pulmonary vascular resistance was elevated at 4.86 Wood units*m2. Systemic oxygen saturation was 79%. Hemodynamic measurements and Fontan angiography demonstrated stable fenestration device position without any residual shunting []. No additional baffle leaks or venovenous collaterals were noted. The previously placed Amplatzer device served as the chosen landmark. Using transesophageal echocardiography (TEE) and fluoroscopic guidance, we identified the location just inferior to the device. This location was chosen based on the available atrial space identified by TEE relative to the position of the device. Fontan baffle puncture was successful using a pediatric transseptal needle, but we could not advance the 7F transseptal sheath across it. The sheath then was exchanged with an adult transseptal sheath and an 18-gauge adult transseptal needle (Cook Medical, Bloomington, IN) was used to cross the baffle []. The transseptal needle was minimally modified by slightly increasing the curvature of the distal end of the needle to account for the angle of approach from the left internal jugular vein. Care was taken to not increase the needle curvature to the extent of losing the ability to apply sufficient force against the baffle. The selected baffle site allowed us to apply sufficient force to cross the baffle with sufficient space to not injure the posterior wall of the right atrium once crossed. The dilator crossed, but the sheath would not cross the newly created fenestration. Therefore, the fenestration was further dilated using a Sterling 4 mm × 2 cm balloon (Boston Scientific, Marlborough, MA) followed by Dorado 7 mm × 4 cm balloon (BARD Peripheral Vascular Inc., Tempe, AZ). A Palmaz Genesis Blue 19 mm × 9 mm premounted stent was subsequently advanced across the Fontan baffle. Under TEE and fluoroscopic guidance, the stent was successfully deployed across the baffle. A residual waist was left intentionally in the stent, leaving a “dumbbell” shape to prevent stent dislodgement []. Postprocedure TEE and angiography confirmed stable stent position with right to left shunting across the stent []. The poststent mean Fontan pressure and transpulmonary gradient were 18 mmHg and 11 mmHg, respectively. There was no immediate improvement in the transpulmonary gradient and oxygen saturation. The patient had recovered well from the procedure and was discharged the next day.
At 6-month follow-up, the severity and frequency of his pulmonary symptoms had improved without additional medical therapy. His oxygen saturation was 83% on room air. Follow-up echocardiography showed stable stent position with right to left shunting and a mean gradient of 5 mmHg. A follow-up catheterization performed 2 years later demonstrated a baseline saturation of 83%. The following hemodynamic data are included: A mean Fontan pressure of 7-8 mmHg, transpulmonary gradient of 5-6 mmHg, ventricular end diastolic pressure of 2 mmHg, and normal pulmonary vascular resistance of 1.99 Wood units*m2.


A 13-year-old Asian girl weighing 24 kg was admitted with progressive thoracolumbar kyphoscoliosis deformity since 2 years. She underwent tracheostomy 6 years before, related to severe bronchopneumonia management. Since then, she continued to require ventilatory support at night. Genetic study confirmed PD and she was started on ERT. Her motor milestones were delayed with progressive deterioration. At admission, she was on tracheostomy and required ventilatory support during sleep with BIPAP mode. She was not able to stand and walk.
She was conscious, oriented with normal higher mental functions. She had thoracolumbar kyphoscoliosis extending from D1 to L4 level. She had wasting of the lower limb and paraspinal muscles with 3/5 motor power. She had varus deformity of the left foot, bilateral fixed flexor deformity of lower limbs. Upper limb showed normal muscle power with no wasting. On auscultation, chest was clear with no added sounds. Oxygen saturation was 95% on room air. Blood gases were within normal limits. Chest X-ray showed normal lung fields. Her cardiovascular system and electrocardiogram (ECG) were normal. Echocardiography showed normal ventricles with ejection fraction of 54% with no evidence of hypertrophy or obstructive cardiomyopathy. Serum creatinine kinase was raised. She was prepared with chest physiotherapy and salbutamol nebulisation.
General anaesthesia was planned. No pre-medication was given. Monitoring included ECG, non-invasive blood pressure, pulse oximetry (SpO2), end-tidal CO2, central venous pressure, depth of anaesthesia monitoring (Entropy͹), temperature and urine output. After adequate pre-oxygenation, anaesthesia was induced with dexmedetomidine infusion 1 μg/kg over 10 min, ketamine 50 mg and morphine 2 mg. Anaesthesia was maintained with dexmedetomidine infusion at 0.5 μg/kg/h, oxygen in air (50:50%) and sevoflurane 1-2% as required. The child remained haemodynamically stable. Body temperature was maintained around 36°C by active forced hot air blankets. Motor evoked potential (MEP) monitoring was used during correction of kyphoscoliosis. The total blood loss was about 1.1 L. Under central venous pressure monitoring, 450 ml of blood along with 2.3 L of Ringers lactate were infused. At the end of the surgery, child was conscious; breathing appeared shallow and hence was shifted to paediatric intensive care unit for elective post-operative ventilation. Post-operative pain was treated with fentanyl infusion.
On the 2nd post-operative day, she developed bilateral mild pleural effusion which was treated with diuretics and antibiotics. On the 5th post-operative day, patient was weaned off from ventilator. However, she continued to require nocturnal ventilatory support. Rest of the hospital stay was uneventful and she was discharged on the 10th day.


A 33-year-old male patient, American Society of Anesthesiologists physical status 1, underwent fissurectomy under spinal anaesthesia with a 25-gauge Quincke spinal needle (BD, Becton Dickinson S. A), in a single attempt. His post-operative period was uneventful, and he was discharged the next day. The patient returned on the fifth post-operative day with complaints of headache, which had started on the second post-operative day. The headache was present all over the head and was not associated with nausea, vomiting or any other neurological signs. It increased in intensity on assuming erect posture and was relieved on assuming supine position.
A diagnosis of PDPH was made, and AEBP was planned in the operation theatre. In the theatre, after establishing standard monitors, under all aseptic precautions and patient in lateral position, epidural space (at L2 -L3) was identified with loss of resistance to air. Autologous blood (20 ml) was drawn aseptically from the left cephalic vein and was injected into epidural space after confirming negative aspiration. At the end of the epidural injection, the patient became apnoeic and unresponsive. Immediately, the position of the patient was changed supine and bag-mask ventilation was initiated. Within minutes, he became fully conscious and responsive, with no neurological deficit.
On evaluation over the next 24 h, it was found that the patient had only partial relief of headache. Next day, his headache increased in intensity and was orthostatic in nature. Although he had no other neurological symptoms, due to the transient unresponsiveness that followed the first AEBP, we did not attempt a second AEBP and decided to evaluate him with magnetic resonance imaging (MRI) of the brain.
Imaging revealed acute thrombosis of entire superior sagittal sinus and bilateral major cortical veins with features of increased intracranial pressure (ICP) (effacement of parietal lobe sulci) [Figure and ]. Fundus examination revealed papilloedema. Therefore, the patient was started on low molecular weight heparin 40 mg twice daily. After 2 days, warfarin 3 mg was added to achieve a target International Normalised Ratio of 2-3. Repeat MRI brain showed resolving signs of CVT. There was complete resolution of the headache. Protein C, Protein S and antithrombin III levels were within normal limits.


A 67-year-old woman sought medical attention complaining of progressive weight loss and loss of appetite over the last 10 months, which was treated with antidepressants because of the suspicion of a mood disorder. In the meantime, she noticed darkening of the skin, but her mucosa, palms, and soles were spared. At the time she was hospitalized, she had lost 21 kg and presenting daily fever (38°C) accompanied by rigor and marked weakness. She brought the results of a normal colonoscopy and an upper digestive endoscopy, which had recently been performed. The physical examination disclosed a pale and cachectic patient weighing 43 kg (BMI of 17) with normal hemodynamic and respiratory parameters. The skin of the neck, axillary, and inframammary regions was darkened and thickened with a velvety appearance, which is consistent with the clinical diagnosis of acanthosis nigricans (AN) ().
Peripheral lymphadenopathy was not found and physical examination of the lungs and heart was normal. However, although the abdomen was flat and flaccid, a hardened and painless mass was easily palpable in the left flank. The abdominal computed tomography confirmed the presence of a voluminous and heterogeneous mass, measuring 13.4 × 8.6 × 7.3 cm, predominantly hypoattenuating and with heterogeneous contrast enhancement. It anteriorly displaced the renal hilum, which was apparently without invasion ().
The laboratory work-up revealed microcytic hypochromic anemia, thrombocytosis, normal renal function, and moderate hyponatremia. The skin biopsy showed thickened epidermis by marked hyperkeratosis and papillomatosis. The papillomatosis resulted from finger-like projections of the dermal papillae to the surface, which was lined by thin epidermis. In between these projections, the epidermis was thicker than the epidermis overlying the papillae. There was no melanocytic proliferation or significant hyperpigmentation of the epidermal basal layer. The dermis had no significant inflammation. The pathological findings were consistent with AN ().
The patient was submitted to a laparoscopic left total nephrectomy followed by tumor removal through a Pfannenstiel incision. The surgical specimen comprised an irregular-contour monoblock weighing 597 g and measuring 14.5 × 13.0 × 8.3 cm, which, at the cut surface, showed a tumor mass occupying nearly 85% of the renal parenchyma, extending until the renal sinus but apparently sparing the vascular structures. The remaining renal tissue showed the corticomedullary limit, which was partially preserved in less than 10% of the kidney ().
The microscopic examination showed that the morphology was consistent with renal clear-cell carcinoma (), Fuhrman 4 nuclear grade with necrosis extending into nearly 40% of the neoplasia. The tumor was restricted to the kidney but invasion was present within the fat tissue of the renal sinus, as well as in the lymphatics and the venules within the tumor. The surgical margins were free of tumor.
The post-operative period was uneventful and the patient was discharged on the seventh post-operative day with skin bleaching (). She was referred to an oncological center for follow-up.


A 35-year-old HPV-18-postive female, without any previous personal or family medical history of interest, underwent a routine follow-up examination for cervicitis in December 2013. The gynaecological examination revealed a high-grade squamous intraepithelial lesion. A cone biopsy was performed, and a diagnosis of early infiltrating cervical adenocarcinoma was made. The maximum tumour thickness was 0.7 mm extending 2 mm horizontally and adjacent to an in situ adenocarcinoma (5 mm in the largest diameter) located 2 mm from the upper border of the endocervix. The clinical stage was pT1A2.
Given these findings, together with the fact that the patient was nulligravida but with reproductive desires, a radical trachelectomy plus sentinel node biopsy was planned. In accordance with established protocol, pelvic magnetic resonance imaging was performed (January 2014) prior to surgery, revealing a solid, slightly heterogeneous lesion in the right ovary measuring 27 × 24 mm and an intracapsular lesion (10 mm × 8 mm) in the left ovary. Radiologically, both lesions were suggestive of ovarian leiomyomas. No pathological findings were observed at the cervical level, and no evidence of lymphadenopathy was observed.
Given these highly unusual radiological findings – bilateral involvement of the ovaries due to leiomyomas – the multidisciplinary tumour board recommended a biopsy of both ovaries. A bilateral ovarian laparoscopy (January 29, 2014) revealed a solid 25-mm lesion in the right ovary and a 15-mm lesion in the left ovary. Based on the pathological examination of the intraoperative biopsy, the preliminary finding was a ‘right ovarian cyst with areas of carcinoma’. Consequently, trachelectomy was ruled out and instead a right adnexectomy was performed. Postoperatively, the patient progressed well without complications. The definitive pathological findings were an infiltrating low-grade NET tumour (Ki-67 = 5%) of likely gastrointestinal origin.
Complementary diagnostic tests included a colonoscopy (negative) and a gastroscopy. The gastroscopy revealed a tumour measuring approximately 4 cm in diameter with central ulceration located in the greater curvature of the stomach. The biopsy revealed a well-differentiated, non-gastrin-producing G2 NET (Ki-67 = 7%). In addition to the endoscopic tests, somatostatin receptor scintigraphy (octreoscan) and 18FDG-positron emission tomography (18FDG-PET/CT) were performed to assess disease dissemination: both tests revealed pathological uptake in the body-fundus junction, in the gastrohepatic lymph nodes, and in the left ovary. Gastrin levels were normal. Chromogranin levels were 105 ng/ml, while urinary 5-hydroxyindoleacetic acid (5-HIAA) levels were 21 mg/day. No relevant biochemical alterations were detected. Fig. and fig. show the results of the 18FDG-PET/CT scans.
Based on these findings, the case was presented to the multidisciplinary NET board, summarized as follows: 35-year-old female with a history of endocervical microinfiltrating adenocarcinoma leading to an incidental diagnosis of a well-differentiated G2 gastric NET with metastatic nodal involvement in a single locoregional area with bilateral involvement of the ovaries, treated previously with right adnexectomy. Given the patient's young age and lack of comorbidities, the tumour board – following clinical guidelines – recommended surgical treatment of the affected structures (left ovary, stomach, and gastrohepatic lymph nodes). In late March 2014, a complete laparoscopic gastrectomy was performed, with esophagojejunal anastomosis and D1 lymphadenectomy expanded to include nodal stations 7–9. No complications arose during the postoperative period.
The postoperative pathological report showed the following: complete gastrectomy with a well-differentiated G2 NET invading the entire gastric wall into the serosa, with metastasis in 1 out of 17 dissected nodes. On immunohistochemistry, the tumour was positive for AE1–AE3, chromogranin, synaptophysin, CDX-2, and negative for gastrin. Given these findings, the case was again presented to the tumour board, which recommended left adnexectomy. Consequently, a laparoscopic hysterectomy with left adnexectomy was performed in May 2014 without complications. In the postoperative period, the patient progressed favourably with acceptable gastrointestinal tolerance except for clinical signs of occasional dumping. Pathological findings from the surgical specimens indicated metastasis in the left ovary from a well-differentiated G2 tumour, no evidence of disease in the uterus, and foci of mild dysplasia in the endocervix.
After both surgeries, a thoracoabdominal-pelvic CT and an octreoscan (fig. ) were performed in June 2014, with image fusion. The images showed that the patient was free of macroscopic disease, and laboratory results for chromogranin A and 5-HIAA acid levels were normal. In July 2014, the patient had recovered from both surgeries and was in good general condition with tolerable symptoms associated with early onset menopause and total gastrectomy (occasional episodes of dumping in addition to hot flushes).
Currently, the patient is free of disease, with excellent performance status (100%). Regular follow-up examinations are scheduled to monitor her condition.


A 43-year-old male residing at sheep-raising province, presented with low grade fever, dyspnea, and chest pain since 18 days. He also complained of difficulty in breathing, 2 days prior to admission. There was no history of cardiac disease or congestive cardiac failure or tuberculosis. The patient had a history of prolonged close contact with sheep and to dogs. On examination, the patient has dyspnea with a heart rate of 96/min, respiratory rate of 20/min without respiratory muscle retractions. His blood pressure was normal. There was no pallor, icterus, and evidence of cardiac tamponade. Organomegaly was not palpable in abdomen. Heart sounds were muffled but there was no murmur. Breath sounds were normal. Laboratory exam revealed a hemoglobin of 10 g/dl, total white cell count of 13,000/mm3, serum albumin was 3.2 g/dl and serum globulin 3.7 g/dl. X-ray chest showed a normal cardiothoracic ratio. Ultrasound scan of abdomen showed normal liver parenchyma. Echocardiography showed thick pericardium with mild pericardial effusion and dense fibrin strands and multiple cystic legions in pericardial cavity [Figures –]. Echocardiography also revealed a large cystic mass in right ventricle attached to interventricular septum []. There was no evidence of tamponade. Heart valves were normal. Computerized tomographic (CT) scan of abdomen showed no hydatid cyst in the liver. The patient scheduled for open cardiac surgery. Median sternotomy was done. Pericardiotomy revealed dense pericardial fibrin strand associated with some vesicles and fragment of ectocyst []. There was a fistula tract from right ventricle's hydatid cyst to pericardium []. After pericardiotomy and dividing fibrin attachment, and removal of daughter cysts, hypertonic saline soaked pad was spread around the pericardium and cyst to minimize the risk of dissemination in case of an accidental spillage of hydatid fluid. Cardio pulmonary bypass with aortic and bicaval cannulation were established and superior vena cava and inferior vena cava were snared. From crater of fistula tract, all content of cyst with ectocyst were removed. The mouth of cyst was left opened to pericardium without capitonnage. One mediastinal chest-drainage tube was placed. The cyst was carefully aspirated after injecting 0.5% cetrimide, a scolicidal solution and then enucleated. Oral albendazole in a dose of 15 mg/kg/day was given 2 days preoperatively and continued for 4 weeks postoperatively. On follow-up after a month, X-ray chest and echocardiography showed complete resolution of the pericardial and right pleural effusions.


A 20-year-old, right-handed man sustained acute unstable complex fractures of the head and neck of the right radius following a traffic accident, consisting of a radial head fracture and medial displacement, radial neck fracture, and a rupture of the lateral ligament complex. On admission, closed elbow trauma was observed, in which severe soft tissue swelling was noted but the neurovascular status was normal. An apparent restriction was noted in the flexion–extension arc of the elbow. The initial radiographs revealed complete fracture-dislocation of the head and neck of the radius, with a large fragment of the radial head lateral to the shaft of the radius and posterior to the elbow joint (). On the basis of the above findings, an open procedure was required for the patient.
In the operating room, an open reduction and mini T-shaped plate internal fixation was performed under general anesthesia. An arthrotomy of the elbow joint was performed through a posterolateral approach. This surgical technique (the exposure process of the radial head) was done in accordance to that described by Shmueli et al. After making a longitudinal incision in the capsule of the elbow joint, damage to the lateral ligament complex was observed, and two large radial head fragments were found to be dissociative. Some small fragments in the elbow joint were removed. The articular surface of the radial head was lustrous but not integral. After extracting the radial head manually, a reconstruction of the articular surface of the radial head was performed in vitro. After refreshing the fracture site, these fragments were fixed with a mini T-shaped plate and screws (Synthes [Shanghai] Medical Trading Co., Ltd, Guangzhou, People’s Republic of China) (), and then the damaged periosteum was interruptedly sutured using absorbable sutures. Finally, one small hole was drilled at the epicondyle. Nonabsorbable sutures were passed through this and through the lateral ligament complex (radial collateral ligament and annular ligament) to fix the ligament securely to the bone. Medial dislocation of the radial head was successfully eliminated. As the reconstructed radial head appeared to be properly aligned on fluoroscopy, a long plaster fixation in the neutral forearm position and 90° of elbow flexion were applied. The postoperative radiographs of the elbow showed that the fixation device was stable and nondisplaced (). After 12 hours, the neurovascular status was rechecked, and the patient was strictly instructed to keep the limb elevated.
Three weeks later, the reconstructed radial head appeared by fluoroscopy to be in its proper location on fluoroscopy. No obvious rotation or separation was observed. Then, the plaster fixation was removed and an attempt was made to start early active exercises for flexion–extension and pronation–supination. Weight bearing was not allowed until the first clinical and radiological follow-up after 1.5 months. After 3 months, full loading was performed.
Bone union occurred without osteonecrosis or heterotopic ossification. Additionally, a radiograph taken 6 months after surgery revealed no avascular necrosis, collapse, or deformity of the radial head. After the metalwork was removed at 1 year (), no comment was made regarding the complications associated with the use of the internal fixator, and the patient was symptom-free with full range of motion in the elbow joint. The carrying angle was 12°. The Mayo Elbow Performance Score was 97 (excellent) (). This study was reviewed and approved by the review board of the First Affiliated Hospital, Sun Yat-sen University, and an exemption for informed consent was obtained from this Investigational Ethical Review Board.


A 22-year-old, stuporous, but previously healthy man, 182 cm in height and 70 kg in weight, was admitted to the emergency room with an accidental injury from a fall. An emergent brain CT demonstrated acute traumatic subdural hematoma, thus an emergent decompressive craniectomy and hematoma removal was performed under general anesthesia without any remarkable incidences. After the first operation, the patient had not made favorable progresses, therefore several further operations such as brain abscess drainage, external ventricular drainage catheter insertion, cerebral lobectomy, ventriculoperitoneal shunt were performed during a 4 month period. Finally, the patient had cranioplasty.
The vital signs of the patient scheduled for cranioplasty were a systolic/diastolic blood pressure of 110/70 mmHg, heart rate 104 bpm, respiratory rate via tracheostomy 20 /min and the patient was still in a stuporous state. Preoperative laboratory data were within the normal range, and the chest X-ray revealed improvement of focal pneumonia and an arterial blood gas analysis in the tolerable range (ABGA; pH 7.418/PaCO2 39.5 mmHg/PaO2 104 mmHg with 2 L/min oxygen inhalation). An electrocardiogram (ECG) performed before the surgery showed sinus tachycardia, and transthoracic echocardiography revealed a preserved global left ventricular systolic function (ejection fraction; 69%). The preoperative brain CT sacn showed a marked shrinkage of the left cerebral hemisphere and an increase in ventricular size (). The general anesthesia was induced with 125 mg thiopental sodium and 50 mg rocuronium, without any premedication. The vital signs were monitored using noninvasive blood pressure (NIBP), ECG and a pulse oximeter. The tracheostomy tube was changed for a 7.5 mm cuffed tracheostomy tube. Following the induction of anesthesia, the right dorsalis pedis artery was cannulated with a 22 gauge catheter, and the right femoral vein was cannulated with an 18 gauge catheter. General anesthesia was maintained with balanced anesthesia using oxygen-air-sevoflurane (0.5–1.5%) and supplementary remifentanil (4–6 µg/kg/h) infusion. Cranioplasty was carried out under controlled ventilation, keeping within 10% fluctuation of the vital signs and ETCO2 with intermittent injections of ephedrine, phenylephrine and esmolol. The ABGA was checked immediately after skin incision, and the results were a pH of 7.436, PaCO2 35.1 mmHg, PaO2 219.1 mmHg, and base excess –0.5. Intravenous fluid replacement consisted of 500 ml plasma solution and 50 ml lactated Ringer solution, and the estimated blood loss was 200 ml. After 2 hours of operation, the dura was closed, and the bony fragment was wired into place. Subsequently, drainage catheters were introduced into both the subgaleal and epidural spaces. By the time the scalp sutures were completed, a closed negative suction through the catheters had been applied. Approximately 30 seconds after suction drainage, sudden asystole of ECG, the disappearance of ETCO2 wave and a flat line arterial wave form were noticed. Instantly, the suction drainage was stopped, and we started external cardiac massage for approximately 20 seconds. Two times injections of 500 µg epinephrine were administered under the diagnosis of cardiac arrest. The cardiac rhythm was resumed to 120 bpm, and systolic blood pressure was regained to 150 mmHg immediately. The ABGA was checked after resuscitation, and the results were in the normal range (pH 7.418, PaCO2 39.5 mmHg, PaO2 103.3 mmHg and base excess 0.2). The patient was transferred to the NICU with a controlled AMBU bagging.
He went into a coma and showed partial spastic movement of extremities, suggesting a sudden increase in intracranial pressure (ICP). Emergent brain CT () revealed extensive diffuse brain swelling. Due to the fact that there were no specific causes of brain edema during the operation or general anesthesia, the negative vacuum suction drainage was presumed to be the main cause, resulting in the patient being diagnosed with PHBS. The patient received agents to reduce ICP, in addition to general supportive care with controlled ventilation. He improved gradually in the NICU, and reached a stuporous state with self-respiration 3 months postoperatively.


A 36-year-old female was referred with renal impairment, creatinine clearance 0.4 mL/s and proteinuria 4.7 g/day. Physical examination was unremarkable. Biochemistry showed normochromic normocytic anaemia, slight hypoalbuminaemia and hypercalcaemia. Anti-neutrophil cytoplasmic antibodies, anti-nuclear antibodies, anti-glomerular basement membrane, anti-phospholipid antibodies, and viral screening were negative. IgG-kappa M-protein was found in plasma (4.8 g/L) and urine (<0.02 g/L), while plasma immunoglobulins and liver enzymes were normal. Bone marrow, skeleton scintigraphy and MR scan were normal. An ultrasound showed normal-sized kidneys. Kidney biopsy presented focal interstitial nephritis, light grade interstitial fibrosis but no glomerulonephritis, vasculitis or tubular damage, and negative Congo staining ().
The diagnoses were monoclonal gammopathy of unknown significance (MGUS) and idiopathic interstitial nephritis. Despite corticosteroid treatment, renal insufficiency progressed and, after 17 months, haemodialysis was initiated.
Four years later, the patient received a kidney transplantation with a standard immunosuppressive regimen: thymoglobulin induction, prednisolone, mycofenolat mofetil and cyclosporine. The optimal plasma creatinine after transplantation was 0.112 mmol/L. Unfortunately, 4 years after transplantation renal function declined to a plasma creatinine of 0.219 mmol/L. A kidney-graft biopsy at this point showed global nodular glomerulosclerosis and deposition of kappa light chains within glomeruli, vessels, and tubular basal membranes, plus C3c in the vessels, characteristic of light-chain deposition disease (LCDD) with no rejection signs or amyloid deposits (a and b).
P-IgG kappa M-protein was 0.86 g/L and Bence-Jones proteins were present in the urine. A bone marrow re-examination presented clonal plasma cell infiltration of kappa type with a kappa/lambda ratio of 19.8.
Presenting M-protein, clonal bone marrow infiltration and renal graft LCDD changes, the patient was diagnosed with multiple myeloma []. Reevaluation of native kidney biopsies revealed a weak-positive reaction for kappa light chains in glomerular nodules, suggesting that LCDD might have contributed to the renal insufficiency. LCDD has a high recurrence risk and poor graft function prognosis after kidney transplantation [, ]. Diagnosing LCDD might be difficult in the early stage. Therefore, patients with a pre-existing MGUS should be carefully evaluated and followed prior to and after kidney transplantation [, ].


A 53-year-old man with macular edema secondary to branch retinal vein occlusion presented with a central macular thickness (CMT) of 525 µm, a best-corrected visual acuity (BCVA) of 48 ETDRS letters and an intraocular pressure (IOP) of 13 mm Hg in the right eye. He was scheduled for intravitreal implantation of Ozurdex®. The implant was injected via the pars plana route 3.5 mm from the limbus following the topical anesthesia with proparacaine. However, the implant accidentally caused a posterior capsular tear and was injected into the crystalline lens because of an involuntary head movement of the patient during the procedure (fig. ). Slit-lamp examination revealed that the implant had penetrated the posterior lens capsule and was lodged in the posterior cortex of the lens with a resultant mild posterior cortical cataract. Since the anterior segment was normal, and the resultant cataract and implant itself did not make a substantial effect on BCVA, the decision was made to observe the patient with intralenticular implant, preserve the therapeutic effect and avoid reinjection.
CMT and BCVA were 253 µm and 58 letters at the first month, 262 µm and 56 letters at the third month and 254 µm and 48 letters at the sixth month (fig. ). Macular edema resolved within time, while BCVA did not show significant improvement due to an increase in lens opacification (fig. ). IOP was within normal limits during follow-up. The patient underwent phacoemulsification surgery at 7 months after the injection with implantation of posterior chamber IOL into the capsular bag. A gentle hydrodissection-hydrodelineation was performed, and low phaco parameters were used during the surgery of the cataract with a damaged posterior capsule. Posterior capsular fibrosis was encountered in the area of the posterior capsular defect during the surgery. After the cataract surgery, the IOL was well centered with a no anterior chamber reaction, and BCVA improved to 63 letters with a normal IOP and a normal foveal contour. The fellow eye was completely normal.


A 72-year-old hispanic male with a past medical history of pulmonary fibrosis controlled on oral steroids and home oxygen therapy, hypertension, diabetes mellitus, and no prior smoking history presented to the emergency department for progressive dyspnea over three weeks. He reported having cough that is productive of yellow sputum with no hemoptysis, associated with fever, night sweats, and six-pound weight loss over one week prior to the admission. In addition, the patient reported having a rapidly growing scalp lesion over two months with accompanying pain but no pruritus or discharge. He denied exposure to smoke or tuberculosis. On physical examination, vital signs indicated a temperature of 96.8°F, blood pressure of 153/87, heart rate of 82, respiratory rate of 22, and oxygen saturation of 86% on room air. On head examination, there was a large, ulcerative, well-circumscribed lesion, measuring 2.5 cm in diameter on the right parietal scalp with no cervical lymphadenopathy. On chest examination, he had fine bilateral diffuse crackles. The remainder of the exam was normal.
Diagnostic laboratory values showed a leukocytosis with a white blood count of 23,200 Cells/mcL, hemoglobin of 12.3 g/dL, platelets of 211,000/mcL, blood urea nitrogen of 17 mg/dL, and creatinine of 0.8 mg/dL.
Arterial blood gas on 21% FiO2 showed pH of 7.44, PO2 of 51, and PCO2 of 39, indicating a gradient of 50 mmHg. A chest X-ray showed bilateral interstitial infiltrates []. The patient was admitted to the medical floor, started on intravenous corticosteroids, oxygen, and nebulized treatments with symptomatic improvement. Initial workup included an echocardiogram showing severe pulmonary hypertension and a computed tomography (CT) scan of the chest showing diffuse fibrotic changes bilaterally with multiple densities of different diameters [Figures and ]. For further evaluation, a CT-guided biopsy of the lung revealed a poorly differentiated malignant neoplasm with foci of necrosis; immunohistochemical stains indicated adenocarcinoma [Figures and ]. Genotype testing was negative for epidermal growth factor (EGFR) mutation, RAS mutation, and alk translocation. Excision of the right scalp lesion was performed, and the pathology revealed poorly differentiated malignant neoplasm favoring adenocarcinoma, as confirmed by immunohistochemical stains [Figures and ]. During his hospital stay, the patient developed severe hypoxic respiratory failure requiring intubation and transfer to the intensive care unit. The etiology of his acute decompensation was determined to be septic shock secondary to healthcare associated pneumonia; hence, the patient was started on vasopressors and intravenous antibiotics. With the acute change of clinical status, prognosis was poor; his condition deteriorated and he expired after 2 weeks of hospitalization.


A 23-year-old man with a history of viral cardiomyopathy had undergone placement of a HeartWare LVAD as a bridge to transplantation (BTT) in August 2013. His postoperative course was complicated by subsequent right ventricular (RV) failure and was placed on continuous milrinone therapy. As an outpatient, he was on a regimen of warfarin and ASA/clopidogrel therapy. In August 2014, the flows on his LVAD device were increasing, and he presented to our institution. He was noted to have elevated lactate dehydrogenase (LDH) of 1442 units/dL as well as tea-colored urine []. The patient was compliant with his medications as an outpatient. His international normalized ratio (INR) on admission was 2.74. He had only mild heart failure symptoms of dyspnea on exertion. He was admitted to the Intensive Care Unit with the presumptive diagnosis of LVAD pump thrombosis and placed on argatroban infusion titrated to a goal partial thromboplastin time of 60–90 s.[] His other medications included bumetanide, carvedilol, digoxin, spironolactone, and milrinone infusion, which were all continued. A transthoracic echocardiogram [] and a computed tomography of the chest did not reveal an obvious thrombus. On the 3rd day of admission, there was a significant increase in LVAD flows and power as well as a steadily increasing LDH (peak >12,900) despite argatroban therapy within the therapeutic range. All options were discussed with the patient including LVAD exchange that he declined at that time. The decision was made to discontinue argatroban infusion, start a heparin drip, and administer tissue plasminogen activator (tPa) intravenously. Initial tPa dosing included a 10 mg IV bolus followed by 1 mg/min for 20 min and finally 1 mg/h for 24 h. There was an immediate improvement in the device parameters after administration of the initial tPa bolus (flow 10.1–>8.2 and power 10 w–>3.5 w). LDH levels continued to fall over the following days at which time the heparin drip was discontinued, and subcutaneous enoxaparin and warfarin were instituted. The patient was discharged after 13 days of stabilization to await, as an outpatient, a suitable transplant donor.


A 44-year-old, (Indian) female was referred to our rheumatology clinic by an ophthalmologist with a history of fluctuating redness in both eyes with ocular pain. She was diagnosed as nodular scleritis and referred for systemic evaluation. On inquiry, she also gave a history of polyarthritis, lasting for 2-3 weeks, 10 years back, it had responded to symptomatic treatment. She also gave a history of intermittent claudication in the right arm, especially during activities such as washing clothes or utensils, for the past 10 years. She was a nonsmoker and had taken oral nonsteroidal anti-inflammatory drugs for arm pain from her general practitioner (GP) without much relief. On inquiry, she also gave a history of recurrent headaches. She did not have any sinusitis, nasal discharge, hemoptysis, skin rash, and fever or weight loss.
On ocular examination, her visual acuity was 20/20 oculus uterque and there was evidence of nonnecrotizing nodular scleritis in her right eye [] without any evidence of thinning bilaterally. There was no evidence of retinal vasculitis on dilated fundoscopy.
On examination, her right radial pulse was absent without any subclavian bruit. The blood pressure in the right upper limb was not recordable, whereas in the left upper limb it was 140/80 mmHg, and both lower limbs 150/90 mmHg. Her investigations revealed hemoglobin 12 g/dl, white cells – 5600/cu mm, erythrocyte sedimentation rate (ESR) of 90 mmHg, and C-reactive protein (CRP) was 6.3 mg/l (normal <6). Rheumatoid factor, anti-cyclic citrullinated peptide, antineutrophil cytoplasmic antibody (ANCA), and antinuclear antibody were negative, urine examination and biochemistry were normal. Serum venereal disease research laboratory was also negative. These reports add further evidence, in addition to the history and examination, to the lack of any other collagen vascular disease whatsoever.
In view of the above findings, a working diagnosis of Takayasu's disease was made, which was further confirmed on a computed tomography (CT) aortogram []. It showed thickening of the right brachiocephalic artery, with complete occlusion of the right subclavian artery, origin of the right vertebral artery was not seen.
The patient was started on oral prednisolone 1 mg/kg body weight along with injection methotrexate 25 mg/week, with folate supplements. Prednisolone was tapered by 5 mg/week after a month till 20 mg/day, then 2.5 mg/week till 7.5 mg daily, calcium supplements and alendronate 35 mg/week. Her attacks of recurrent scleritis subsided [], but right arm claudication persisted for which she underwent right subclavian artery stenting, with moderate symptomatic improvement.


A 26-year-old Hindu male reported to psychiatry outpatient department with a complaint of difficulty in walking, weakness of both lower extremities from last 1-month. He was receiving treatment from our institution since last 2 years with a diagnosis of the bipolar affective disorder. He was maintained on lithium carbonate 800 mg and olanzapine 10 mg/day from last 14 months. He was euthymic since last 3 months. Hence, he was maintained only on lithium 800 mg/day from last 3 months. On examination, the patient was conscious, oriented, power and tone of both lower limbs reduced, deep tendon reflexes were reduced and plantar responses remaining flexor. No other abnormal finding was elicited on physical examination. Routine laboratory tests including liver functions test, serum B12 and folate assays, thyroid function tests, serum electrolytes, electroencephalogram, magnetic resonance imaging brain were within normal limits. Serum lithium level was 0.84 mEq/L. Nerve conduction studies revealed large fiber axonal degeneration predominantly motor type. He had no previous medical history of hypertension, diabetes, heavy metal exposure, alcohol intake, epilepsy, neurological deficits, or any other drug intake. No features consistent with lithium toxicity, that is, confusion and lethargy, slurred speech, urinary incontinence, worsening tremor were present. There is no family history of any neuropsychiatric illness. The lithium carbonate was discontinued. He was started on quetiapine 400 mg/day. After a stoppage of lithium, the patient improved gradually within next 4 weeks. Over the next 6 weeks difficulty in walking, weakness of both lower extremities disappeared completely. There was no deterioration in affective symptoms.


A 5-year and 9-month-old boy with previous history of being small for age, previous Proteus mirabilis urinary tract infection when 3 years old, and acute bronchitis with secondary bacterial infection when 5 years old presented to the children’s emergency department with symptoms of cough for 1 week duration associated with fever for 1 day.
He complained of neck pain for 3 days before admission, but it resolved on admission. There was no history of trauma. There was no sick contact, no recent travel, no chest pain, and no pretreatment with antibiotics.
Initial examination demonstrated oxygen saturation of 96% in room air with pulse rate of 143 beats per minute and respiratory rate of 40 breaths per minute, and reduced air entry on the left base with crepitation on chest examination.


A 4-month-old 6 kg female child was scheduled for bilateral lensectomy for congenital cataract. She was delivered by caesarean section at term but developed respiratory distress and convulsions on the 4th day of life. She needed mechanical ventilation for 1 week and received peritoneal dialysis. She had been diagnosed earlier with IVA and was on formula feeds with carnitine and glycine supplements. Gas chromatography and mass spectrometric examination of urine showed massive elevation of isovalerylglycine. A month earlier, the parents had noticed opacities in both eyes and had sought treatment.
On examination, she was playful and normothermic, heart rate was 110/min, respiratory rate 26/min and systemic examination was normal. Her blood investigations revealed normal values of haemoglobin, platelets and haematocrit. Blood urea was 101 mg/dL, serum creatinine 0.8 mg/dL, serum Na+/K+, 148/6.8 mEq/L and bilirubin 8.8 mg/dL. Arterial blood gas analysis (ABG) values were obtained [] which showed metabolic acidosis. Patient was started on 10% dextrose 24 ml/h, intravenously with 8 hourly monitoring of ABG and blood sugar levels. She was scheduled for surgery on the 5th day after optimisation of acidaemia [].
After pre-oxygenation with 100% oxygen and premedication with injection glycopyrrolate 5 µg/kg, ondansetron 0.08 mg/kg, midazolam 0.03 mg/kg and pentazocine 0.3 mg/kg, intravenous (IV), the baby was induced with sevoflurane 2–4% in oxygen and trachea intubated with number 3.5 uncuffed polyvinyl chloride tube. Anaesthesia and relaxation were maintained with sevoflurane, atracurium 0.5 mg/kg bolus and top up doses. Electrocardiogram, oxygen saturation, non-invasive blood pressure, skin temperature and blood sugar levels were monitored intraoperatively. Trachea was extubated after thorough oropharyngeal suction and reversal with injection neostigmine 0.05 mg/kg and glycopyrrolate 8 µg/kg. The patient was monitored closely in the post-operative period. Blood sugar, ABG and electrolytes values done after 2 h were optimal [].


A 57-year-old male in Beijing, China underwent carotid artery stenting via transfemoral approach for severe (>90%) asymptomatic right internal carotid artery (ICA) stenosis (Fig. a). A neurosurgical team at an outside facility performed the procedure using a 5-mm SpiderFXTM distal filter EPD and ev3 ProtégéTM 8 × 30 mm RX stent (Covidien; Plymouth, MN, USA). During deployment, this stent (referred to as Stent 1) inadvertently migrated cephalad and became lodged onto the EPD. Despite multiple attempts, the EPD was unable to be retrieved with the capture sheath. The physicians placed another ev3 7 × 40 mm ProtégéTM RX stent in the right ICA to compress the EPD and guidewire against the arterial wall. We refer to this as Stent 2, which was placed at the C1–C2 vertebral level, superior to Stent 1 (Fig. b). The patient was discharged on aspirin and clopidogrel, with the retained guidewire sutured in place at the groin. At time of discharge, the ICA stenosis had not been resolved.
One month after discharge, the patient began experiencing substernal chest pain radiating to the neck with right suborbital pain. It was discovered that the guidewire had fragmented into three pieces. He was referred to our cardiovascular surgical team at the Anzhen Stroke Center for further management.
Upon presentation, we found moderate (55%) stenosis in the right common carotid artery (CCA) and severe (90%) stenosis in the ICA inferior to Stent 1. The most superior of the three guidewire fragments (Fragment A), still attached to the EPD, was compressed by Stent 2 and extended into the CCA (Fig. c and d). The middle piece (Fragment B) was adhered to the right CCA wall by local fibrin deposits. The inferior fragment (Fragment C) extended from the right femoral artery to the thoracic aorta, where it punctured the proximal aortic arch wall (Fig. a and b). Thrombus had also formed along the entire 1-meter length of Fragment C.
Given the risks of dislodging emboli during surgery, we initially considered employing two additional stents – one to manage the stenosis and another to compress and stabilize Fragment B. However, this would not resolve the fragmented guidewire. With the patient, we agreed upon CEA and surgical removal. Due to the extremely superior cervical location of the EPD, it was not likely to be amenable to surgical retrieval. A second surgery was scheduled for a later time to remove Fragment C.
We performed CEA at the right ICA, after which Stent 1 was cut open longitudinally and excised. Since Stent 2 was located more superiorly (C1–C2 vertebral level) and had accrued significant thrombotic material since the initial stenting procedure, we were unable to safely remove it. Thus, Stent 2 and the EDP were left intact. Fragment A was connected to the EDP and compressed against the arterial wall by Stent 2. We cut Fragment A near the stent and removed the freed portion of this fragment. Stent 2 was anchored to the artery using 7-0 Prolene sutures.
Intraoperative ultrasound confirmed the location of Fragment B in the right CCA (T1 vertebral level). We extended the CCA incision inferiorly to visualize the length of Fragment B, which was then removed intact using an artery clamp. Fig. a shows Fragments A and B, and Stent 1 after removal.
The endarterectomy incision was repaired with polytetrafluoroethylene carotid patch, and intact vasculature was confirmed via fluoroscopy.
After a 1-week recovery in the intensive care unit, the patient returned to the operating room for removal of Fragment C, which was piercing the aortic arch. Via median thoracotomy, a purse-string suture was made around the point of penetration. A 6-Fr sheath was used to entrap thrombotic material as the guidewire was removed from femoral artery without incidence.
After surgery, the patient reported alleviation of his symptoms. Carotid vasculature remained patent with sufficient flow, and Stent 2 was still in place 30 months post-operatively (Fig. b). While the patient will require close follow-up, no complications have been reported to date, over 3 years after surgery. Informed consent was obtained from the patient prior to creation of this manuscript.


A woman aged 35 years presented to our department with 1-year history of clicking noise in her throat on swallowing [Figures and ]. This was associated with pain but no difficulty on swallowing. She denied of any previous history of trauma. She did not have any respiratory symptoms or jaw-locking. Taking into consideration, the patient's symptom logy; it was suspected to be a case of the salivary gland sialolith. On sialography and ultrasonography, no such finding was evident.
On clinical examination, a trigger point was palpable on anteroinferior aspect of masseter muscle. Patient had an associated sleep disturbance and loss of interest in surroundings. Beck's depression index was used to assess the depression associated with pain. Patient was found to have moderate depression. She was given trigger point injection with 0.5 ml of 0.5% injection bupivacaine. However, patient had no relief. For alleviation of pain associated depression, tricyclic amitriptyline antidepressant 75 mg was used for 3 weeks. Patient got no relief in pain and clicking. On further examination of throat, CT scan was done [] greater horn of hyoid bone was found large in size with abnormally long greater cornua. While swallowing, a significant click was as greater cornua were impinged, caught, and slipped back the spine. Subsequently, the diagnosis was made as “clicking hyoid.”
Patient was explained of the diagnosis and offered the options of conservative and surgical modalities of treatment. However, patient elected the conservative one. To commence the conservative treatment, hyoid bone was palpated digitally as advised by Brown. The patient was given local lignocaine plus steroid injections weekly for 1 month on the tip of the affected cornua. However, patient reported with no relief. Thereafter, the patient was planned for surgical resection of greater cornua under general anesthesia. An informed written consent was obtained from the patient for the surgery.
Superior limb of Mcfee incision was given. Skin, subcutaneous tissue, platysma were incised. Blunt dissection was done through deep cervical fascia. Middle pharyngeal constrictor was bluntly dissected from the greater cornua to expose it []. After the exposure, greater cornua was resected from its junction to the body []. Layer wise closure was done. The patient was followed up with an uneventful healing with complete resolution of clicking and pain [].


A 32-year-old adequately nourished male was admitted for complaints of shortness of breath for 1 month which had exacerbated for the past 2 days. No other accompanying complaints were present. He used to work as a tailor with a mixed diet usually on his platter. Examination revealed a well built and nourished male with a body mass index of 25.3 kg/m2 who was tachypneic with the presence of tachycardia and mild fever. Oral thrush was present and scrapings were sent for fungal culture. There were no apparent risk factors for the presence of HIV or any other immunosuppressive states. Chest X-ray revealed few scattered inspiratory crackles and the saturation monitor was showing a SpO2 of 87%. High flow O2 was initiated and the patient was then shifted to the Intensive Care Unit for further management. Blood gases showed a PO2-47 mmHg and PCO2-24 mmHg. Chest roentgenogram showed bilateral perihilar infiltrates. Given the clinical picture of the patient, Pneumocystis jiroveci pneumonia (PJP) was high on cards, given the apparent immunosuppressed state. Empirical antibiotics, fluconazole and oral trimethoprim-sulphomethoxazole [TMP-SMX], was started along with steroids. Lactate dehydrogenase was 916 U/L (high) and high-resolution computed tomography of the thorax revealed ground glass opacities in both lung fields, more so being perihilar. Other routine blood work was normal. The patient was tachypneic on high flow O2 and required noninvasive ventilation intermittently. Bronchoalveolar lavage was done and it revealed pneumocystis organisms. Acid-fast bacteria and routine bacterial and fungal culture were negative. The fungal scrapings yielded a growth of Candida albicans sensitive to fluconazole. Thus, the diagnosis of PJP was confirmed.
To identify the cause of the immunosuppressed state, his HIV ELISA was done but was negative. A repeat sample yielded the same result. Since the clinical suspicion of HIV was so high, a Western blot and a HIV-RNA polymerase chain reaction were ordered as well. Both turned out to be negative. Now, it was confirmed that the patient did not have the dreaded disease. CD4 and CD8 counts were ordered, as a suspicion for idiopathic CD4+ lymphocytopenia (ICL) was high. The CD4 turned out to be 110/mm3 and the CD4% was 14.3. The CD8 counts were 312/mm3 (normal) with a CD4:CD8 ratio, inverted (0.35; normal: 1.4–1.7). This test was repeated after 6 weeks and the results were the same.
The patient was screened for other causes of immunodeficiencies through history and laboratory reports. He denied any history of immunosuppressive drug intake, did not experience repeated infections in the childhood, his immunoglobulin and sugar levels were normal, and the titers for HTLV1 and 2 were negative. Thus, idiopathic CD4+ lymphocytopenia was labeled as the cause of his immunodeficiency and was given a prophylaxis of TMP-SMX and fluconazole post-treatment.


A 79-year-old man was referred by his General Practitioner to our Radiology Unit in the August 2015 to perform a swallow study. He complained of fluctuating dysphagia episodes following solid food ingestion over the last 5 months, with mild weight loss. No other GI symptoms were present. His past medical history included a lung resection of the right apical lobe due a cancer 11 years before, without adjuvant chemotherapy. Moreover, he was suffering with diabetes and blood hypertension treated with metformin and irbesartan, respectively.
At videofluoroscopic swallow examination, the surface of mucosa appeared irregularly nodular in the oropharynx, through the entire esophagus, as well as in the fundus and gastric body (). Upper endoscopy was promptly performed which perfectly confirmed the radiologic findings (). In detail, nodular lesions were detected between tongue origin and epiglottis, and diffuse submucosal nodules, with a diameter varying from 3 to 8 mm, through the entire esophagus. Normal intervening mucosa was noted. Similar nodules were observed in the gastric body and fundus, whilst the mucosa of gastric antrum, duodenal bulb and descending portion of duodenum was macroscopically normal. Multiple biopsies were taken in the esophagus, stomach, as well as in the duodenum.
Histological assessment showed diffuse lymphoid infiltration of small lymphocytes, forming nodular aggregates, in both esophageal and gastric mucosa, and the immunohistochemistry study revealed CD20+, CD5+ and cyclin D1 overexpression (). The proliferative index assessment, by using the Ki67 (clone MIB-1), showed a positivity in 20% of cells. Helicobacter pylori infection was absent. Therefore, a diagnosis of MCL was made. Following a standard staging procedure, including total body computed tomography and bone marrow biopsy, a stage III disease was diagnosed with mediastinal and axillary lymph nodes involvement. Human immunodeficiency virus infection, a condition predisposing to Burkitt’s lymphoma throughout the GI tract [], was absent. Chemotherapy including rituximab (375 mg/m2) and bendamustine (70 mg/m2) was started. Following 3 chemotherapy cycles the dysphagia markedly improved, and a repeat endoscopy showed the complete regression of esophageal lesions with an evident reduction in the nodular pattern of gastric body mucosa (). Chemotherapy is ongoing.