Diencephalic syndrome of emaciation in an adult associated with a third ventricle intrinsic craniopharyngioma: case report

OBJECTIVE AND IMPORTANCE: Diencephalic syndrome of emaciation (DS) is seen almost exclusively in infants and young children, and only two cases of DS in adults have been reported previously. We describe a case of DS associated with a third ventricle intrinsic craniopharyngioma in an adult patient. CLINICAL PRESENTATION: A 54-year-old man presented with profound emaciation, disorientation, memory loss, and psychological disorders. Computed tomographic scanning and magnetic resonance imaging of his brain disclosed a tumor within the third ventricle. The preoperative endocrinological examination indicated an elevated growth hormone level and a decreased somatomedin C level. INTERVENTION: The patient underwent partial resection of the tumor, which was adherent to the floor of the third ventricle but not to the ventricle wall, by an interhemispheric-transcallosal approach. The histological examination revealed a squamous papillary-type craniopharyngioma. The patient received 50 Gy of radiotherapy, which resulted in 90 to 95% reduction of the tumor size within 6 months, as indicated by the magnetic resonance images presented. The psychological disorders, memory disturbance, and severe emaciation improved gradually thereafter. CONCLUSION: Although extremely rare, DS can occur in an adult harboring a tumor in the anterior hypothalamus.     

Diencephalic syndrome of emaciation (Russell''s syndrome) long-term survival

Three cases of diencephalic syndrome are reported. Two of them, which have been observed postoperatively for more than 7 years, showed typical clinical and endocri-nologic features at the time of their first admission, but showed mass signs uncharacteristic of diencephalic syndrome after recurrence of the tumor. Recent data showed a normal baseline for plasma growth hormone but abnormalities in provocation tests. The significance of age in the manifestation of the syndrome is briefly discussed, especially in relation to the tendency toward normalization in the growth hormone level at a later age.     

Trigeminal neurinoma associated with suprasellar arachnoid cyst: case report

The first case of trigeminal neurinoma associated with suprasellar arachnoid cyst is presented. Coexistence of these two lesions may be accidental, but it is also possible that the arachnoid cyst was secondary to the neurinoma.     

Ruptured suprasellar dermoid associated with middle cerebral artery aneurysm: case report.

Dermoid tumor associated with cerebral aneurysm is extremely rare. We report here a case of ruptured suprasellar dermoid cyst associated with middle cerebral artery (M1) aneurysm and stenosis of adjacent arterial trunks. The characteristic feature of this association is the tight adhesion of the vascular lesion to the tumor capsule.     

Seatbelt syndrome associated with an isolated rectal injury: case report

Background

Spontaneous splenic rupture considered a relatively rare but life threatening. The three commonest causes of spontaneous splenic rupture are malignant hematological diseases, viral infections and local inflammatory and neoplastic disorders. We describe a unique and unusual case of inflammatory myofibroblastic tumor of the tail of pancreas presented with massively enlarged spleen and spontaneous splenic rupture.

Case presentation

A 19 years old male patient with no significant past medical history presented to emergency room with abdominal pain and fatigue. Massively enlarged spleen was detected. Hypotension and rapid reduction of hemoglobin level necessitated urgent laparatomy. About 1.75 liters of blood were found in abdominal cavity. A large tumor arising from the tail of pancreas and local rupture of an enlarged spleen adjacent to the tumor were detected. Distal pancreatectomy and splenectomy were performed. To our knowledge, we report the first case of massively enlarged spleen that was complicated with spontaneous splenic rupture as a result of splenic congestion due to mechanical obstruction caused by an inflammatory myofibroblastic tumor of the tail of pancreas. A review of the literature is also presented.

Conclusion

Inflammatory myofibroblastic tumor of the tail of pancreas should be included in the differential diagnosis of the etiological causes of massively enlarged spleen and spontaneous splenic rupture.     

Seatbelt syndrome associated with an isolated rectal injury: case report

Seatbelt syndrome is defined as a seatbelt sign associated with a lumbar spine fracture and a bowel perforation. An isolated rectal perforation due to seatbelt syndrome is extremely rare. There is only one case reported in the Danish literature and non in the English literature. A 48-year old front seat restrained passenger was involved in a head-on collision. He had lower abdominal pain and back pain. Seatbelt mark was seen across the lower abdomen. Initial trauma CT scan was normal except for a burst fracture of L5 vertebra which was operated on by internal fixation on the same day. The patient continued to have abdominal pain. A repeated abdominal CT scan on the third day has shown free intraperitoneal air. Laparotomy has revealed a perforation of the proximal part of the rectum below the recto sigmoid junction. Hartmann's procedure was performed. The abdomen was left open. Gradual closure of the abdominal fascia over a period of two weeks was performed. Postoperatively, the patient had temporary urinary retention due to quada equina injury which resolved 10 months after surgery. The presence of a seatbelt sign and a lumbar fracture should raise the possibility of a bowel injury.     

A suprasellar atypical teratoma presenting as an intrasellar mass: a case report

Intracranial atypical teratomas are midline brain tumors with both germinomatous and teratomatous elements. When they occur in the suprasellar region, they usually present with a classic triad of visual disturbances, diabetes insipidus, and hypopituitarism. We report the case of a suprasellar atypical teratoma that presented as an intrasellar lesion and caused secondary amenorrhea in a 15-year-old girl.     

Atypical osteochondroma of the lumbar spine associated with suprasellar pineal germinoma: A case report

BACKGROUNDOsteochondromas are the most common benign bone tumor, accounting for 36% of benign bone tumors. Often found within the appendicular skeleton, osteochondromas of the spine are rare, comprising 4% to 7% of primary benign spinal tumors. CASE SUMMARYWe report a case of a solitary lumbar osteochondroma in an 18-year-old male with a history of a suprasellar pineal germinoma treated with combined chemotherapy and radiation. He underwent mass excision and partial laminectomy with the ultrasonic bone scalpel (Misonix, Farmingdale, NY, United States) at the L5 Level without the use of adjuvants. The patient returned to work and full activities without back pain at 3 mo postoperatively. CONCLUSIONOsteochondromas are common tumors of the appendicular skeleton but rarely occur within the spine. This case discussion supplements current osteochondroma literature by describing an unusual presentation of this tumor.     

Severe scoliosis associated with Costello syndrome: a case report

Costello syndrome is characterised by dwarfism, unique cutaneous lesions, a distinct facial gestalt, and mental retardation. There have been no detailed reports of severe spinal deformities requiring surgical treatment as a complication of Costello syndrome. We report a case of a 10-year-old girl with progressive scoliosis associated with Costello syndrome. She underwent anterior release and posterior surgical correction and fusion from T5 to L2 using a third generation hook and rod system plus spinous process wiring. Congenital portal vein deficiency and coagulopathy were other major complications. At 15-month follow-up, the patient had good balance and no evidence of instrumentation failure.     

Stevens-Johnson syndrome associated with oral minoxidil: a case report

Minoxidil is frequently used in patients with advanced renal disease who have been unresponsive to other antihypertensive agents. We describe a case of a 50-year-old man with chronic renal failure who was hospitalized complaining of a 2-week history of sore throat that had progressed to severe oral lesions and multiple pustular blisters on many sites of his body. The patient had been placed on oral minoxidil for uncontrolled hypertension one week prior to the onset of symptoms. The diagnosis of minoxidil-induced Stevens-Johnson syndrome (SJS) was established, minoxidil was discontinued and the patient subsequently improved. Although minoxidil-induced SJS is extremely rare, clinicians should be aware of this potentially severe adverse effect. This report emphasizes the importance of monitoring patients who are taking oral minoxidil for any signs or symptoms associated with SJS.     

Vogt-Koyanagi-Harada syndrome associated with renal failure: a case report

Vogt-Koyanagi-Harada (VKH) syndrome is a rare entity characterized by depigmentation of the skin and eye lashes, chronic granulomatous iridocyclitis and exudative retinal detachment, as well as aseptic meningitis and encephalopathy. We describe a 22-year-old male intravenous drug addict, infected with hepatitis B and C virus, suffering from this syndrome, associated with progressive renal sclerosis, malignant hypertension, heart failure and chronic myeloproliferative disorder. The association with these various diseases is discussed and relevant cases are reviewed.     

Peutz-Jeghers syndrome associated with intestinal intussusception: a case report

A 12-year-old boy visited our hospital with complaints of severe upper abdominal pain and vomiting. Marked tenderness was present in the upper abdomen, and a movable hard tumor the size of a fist was felt there. Because abdominal computed tomography scans revealed invagination, an emergency laparotomy was performed. The small intestine was invaginated over an area measuring 30 cm in length. After the invaginated intestine was restored, a mass was felt in the small intestine, which was resected over an area measuring approximately 10 cm in length. The extracted mass was histopathologically found to be a hamartoma. The patient was diagnosed to have Peutz-Jeghers syndrome (PJS) based on the presence of pigmented spots detected on the oral cavity, lips, and toes of both feet. PJS is an autosomal dominant genetic disease associated with melanin pigment spots on the oral mucosa, lips, nasal alae, palm, and soles, as well as hamartomatous polyposis in the alimentary canal. Polyps are often a cause of invagination and ileus in affected patients. Gastrointestinal cancer, uterine cancer, and breast cancer are also seen in patients with PJS. A long-term follow-up is required to prevent invagination and ileus in children and cancer in adults.     

Cervical myelopathy in an adult due to atlantoaxial subluxation associated with Down syndrome: a case study

 Atlantoaxial subluxation resulting in cervical myelopathy in patients with Down syndrome has been well documented. The risk of cervical myelopathy is reported to decrease with age, and reports of adult-onset cases are limited. We report an adult patient with Down syndrome who developed cervical myelopathy and was successfully treated by surgery. Because there is little information on this condition with adult onset, we report the features of the radiographic findings. Particular care over the long term should be taken in those who have atlantoaxial subluxation combined with odontoid hypoplasia or accessory ossicles, as they are at particular risk of cervical myelopathy even during adulthood. Received: March 27, 2002 / Accepted: October 3, 2002 Offprint requests to: A. Seichi     

Lobar emphysema with pneumothorax in an adult: report of a case

A 31-year-old woman was transferred to our hospital for treatment of a right pneumothorax. She had presented initially with moderate dyspnea and coughing at a local clinic, where a chest radiograph showed a collapsed right lung. Chest computed tomography showed overinflation of the middle lobe and a large bulla. We diagnosed congenital lobar emphysema of the middle lobe with pneumothorax and performed middle lobectomy by video-assisted thoracic surgery via four ports (5–12 mm in size). The patient had an uneventful postoperative course and was discharged from hospital 5 days after surgery.     

Malignancies associated with renal transplant: first case report of a nephroblastoma occurring in an adult host kidney

The increased incidence of malignancies after renal transplantation has been well documented. We describe the first reported case of a nephroblastoma (Wilms tumor) occurring in the host kidney of an adult after renal transplantation.     

Werner's syndrome associated with meningioma: case report

We present two cases of Werner's syndrome associated with intracranial meningioma. Characteristic clinical features of Werner's syndrome include short stature with slender extremities, premature senility, juvenile cataract, skin changes, a tendency to diabetes mellitus and familial occurrence. A 44-year-old female, who had been treated for diabetes mellitus, was diagnosed as having Werner's syndrome because of various characteristic features. A falx meningioma was incidentally found on CT scan, and was surgically removed. Her diabetes mellitus improved. The second case was a 28-year-old male was diagnosed as having Werner's syndrome, diabetes mellitus, juvenile cataract, together with diabetes insipidus, and liver dysfunction. He developed severe headache, gait disturbance and then became unconscious with right hemiparesis. He was found to have a parasagittal meningioma by CT scan and angiography. After removal of the tumor, diabetes mellitus, diabetes insipidus and liver dysfunction improved. The reported incidence of neoplasms associated with Werner's syndrome is about 10%. The majority of associated tumors were mesenchymal in origin. Ten meningiomas, 1 neurinoma and 2 gliomas are reported as associated tumors in the central nervous system. Most of the associated meningiomas were asymptomatic and found incidentally at autopsies or CT scans. Diabetes mellitus associated with Werner's syndrome is generally mild with high immunoreactive insulin value and is controllable by diet therapy and oral antidiabetic drugs. Daily profile of blood sugar improved after the removal of tumor in our cases. In 50 gm glucose tolerance test, tendency of delayed appearance of peak value, which is common in Werner's syndrome, was not altered in our cases. Discussion is made as to the association of Werner's syndrome with meningioma and diabetes mellitus.     

Cystic hygroma in an adult: a case report

Cystic hygromas are benign lesions arising due to an abnormality in lymphatic development and only rarely present in adults. Adequate radiological imaging prior to surgery is important as incomplete excision often leads to recurrence. Several adjunctive therapies have been shown to be beneficial in recurrent or inaccessible lesions but these are not in common use. We describe the case of a cystic lymphangioma appearing in adulthood that presented a diagnostic and therapeutic challenge.