Nasopharyngeal glial heterotopia with delayed postoperative meningitis.

A male infant, who underwent radical resection of a large glial heterotopia at the nasopharynx at 8 days, developed delayed postoperative bacterial meningitis at 9 months. Neuroradiological examination clearly demonstrated that meningitis had occurred because of the intracranial and extracranial connections, which were scarcely seen in the perioperative period. A transsphenoidal extension of hypothalamic hamartoma is possible because the connection started from the right optic nerve, running through the transsphenoidal canal in the sphenoid bone and terminating at the recurrent mass in the nasopharyngeal region.

An ectopic adreocortical adenoma of the renal sinus: a case report and literature review.

BACKGROUND: Ectopic adrenal tumors are very rare, especially in the renal sinus in adults. An unusual case of ectopic adrenal cortical adenoma in the right renal sinus is reported here. CASE PRESENTATION: This patient was a 37-year-old woman. She was admitted to our hospital for hypertension and bilateral limb weakness. Computed tomography (CT) revealed a mass in right renal sinus. It was initially considered a tumor of the renal pelvis. Further computed tomographic angiography (CTA) showed the mass to be located outside the renal pelvis. After adequate preoperative preparation (blood pressure control and serum potassium supplement), the patient underwent laparoscopic resection of retroperitoneal tumor. During the procedure, a soft tissue tumor 3.4*3.0 cm(2) in size with a golden color was found in the right renal sinus. The final immunohistochemistry examination showed an ectopic adreocortical adenoma. CONCLUSION: Ectopic adrenal tumors are rare in the renal sinus and difficult to diagnose and treat. Large and functional tumors should be treated with complete resection. The procedure is sometimes difficult for tumors located deep in the renal sinus. The decision to perform an open or minimally invasive surgery should be made according to the surgeon's experience.

[Heterotopic gastric mucosa in the rectum: Report of a case]. / Mucosa gástrica heterotópica en el recto: reporte de un caso.

BACKGROUND: Ectopic gastric mucosa has been described in different locations of the digestive tract, but that of the rectum is the least frequent. CLINICAL CASE: The case is described of a 48 year-old woman being investigated by the gastrointestinal department due to rectal bleeding and rectal tenesmus. Colonoscopy showed a diverticular cavity 3 cm, which was reported by histology as fundic-type heterotopic gastric mucosa. Barium enema and abdominopelvic CT showed a diverticular image at level of the right posterolateral wall of the rectal ampulla. Trans-rectal diverticulectomy was performed with primary closure of the resulting mucosal defect. The surgical specimen showed areas of gastric epithelium with no signs of atypia. CONCLUSIONS: It is not known whether the origin of heterotopic gastric mucosa occurs during foetal development or is the result of abnormal regeneration under inflammatory conditions. It is usually clinically asymptomatic or presents as haematochezia, especially in cases where gastric acid is being produced. In these cases there must be an initial treatment with proton pump inhibitors, although the definitive treatment is always surgical or endoscopic excision of the mucosa.

Kaposi sarcoma related to an ectopic hypothalamic adrenocorticotropic hormone-secreting adenoma: case report.

OBJECTIVE: We aim to report a case of Kaposi sarcoma (KS) with Cushing's syndrome caused by endogenic glucocorticoid-induced immunosuppression. CLINICAL PRESENTATION: A 43-year-old woman presented with delirium, hirsutism, fatigue, and hypertension. At the time of presentation, physical findings showed a Cushingoid appearance, with moon-like facies, hirsutism, and hyperpigmentation. Laboratory findings showed the following: adrenocorticotropic hormone, 86.7 pg/mL (normal range, 0-46 pg/mL); baseline cortisol level, 50 microg/dL (normal range, 6.2-19 microg/dL); potassium, 2.2 mEq/L (normal range, 3.5-5 mEq/L); and midnight cortisol level, 33 microg/dL. Serum cortisol levels failed to suppress after low and high doses of dexamethasone; these findings confirmed the diagnosis of ectopic adrenocorticotropic hormone production. Magnetic resonance imaging revealed a 12 x 15-mm, round, hypothalamic mass lesion in the center of the median eminence. INTERVENTION: Endoscopic biopsy from the floor of the third ventricle was performed, and pathological examination of the lesion showed a diffuse adrenocorticotropic hormone-secreting adenoma. The patient developed diffuse skin lesions that were proven to be a KS by skin biopsy while she was prepared for transcranial surgery. After surgical removal of the adenoma, she became hypocortisolemic and required cortisol replacement. Within 1 month after surgery, all KS lesions disappeared spontaneously. CONCLUSION: Excessive cortisol may induce immunosuppression. KS is one of the most common malignant tumors of patients with immunosuppression. To the best of our knowledge, this is the first case of Cushing's syndrome with KS caused by endogenous glucocorticoid-induced immunosuppression.

[65-year old female patient with persistent hypercalcemia]. / 65-jährige Patientin mit persistierender Hyperkalzämie.

A 65-year old patient presented with newly diagnosed hypercalcemia and elevated parathyroid hormone levels. Exploration of parathyroid glands was performed under the diagnosis of primary hyperparathyroidism and was combined with thyreoidectomy due to concomittant multinodular goiter. However, no parathyroid adenoma was identified despite careful exploration. Therefore, the operation was terminated and an ectopic adenoma was suspected. A (99m)Tc-sestamibi scintigraphy with SPECT, neck ultrasound, neck CT scan with contrast media and selective venous sampling were performed for further localization of the adenoma. A single adenoma localized in the submandibular area of the left jaw was suspected and confirmed during surgical reexploration. Postoperatively, parathyroid hormone levels were below detection level and the patient required short-term calcium supplementation for symptomatic hypocalcemia.

Treatments of hamartoma with neuroendoscopic surgery and stereotactic radiosurgery: a case report.

Hypothalamic hamartoma is a non-neoplastic tumor manifesting as gelastic seizure, precocious puberty, and abnormal behavior. Treatment of it is very complicated due to its location. We report a case of hypothalamic hamartoma treated by neuroendoscopic surgery and stereotactic radiosurgery. A 5-year-old girl presented with violent behavior, precocious puberty, gelastic seizure and atonic seizure. She was diagnosed with hypothalamic hamartoma by CT and magnetic resonance imaging at 11 months of age. Tumor size did not change, but tumor intensity had changed on the MR image at 5 years of age. Magnetic resonance spectroscopy revealed decreased N-acetylaspartate and increased choline and creatine in the tumor. After neuroendoscopic biopsy, she underwent linear accelerator stereotactic radiosurgery. But her symptoms remained unchanged for 6 months. She then underwent partial resection and laser coagulation of the tumor by a neuroendoscopic approach. After the procedure, the frequency of her seizures was remarkably decreased, and her violent behavior improved. The transventricular neuroendoscopic approach to the hypothalamus is less invasive than the radical surgery. Neuroendoscopic surgery can be one of the treatments of choice for hypothalamic hamartoma.

Transposition of the lingual thyroid: A new alternative technique.

BACKGROUND: In symptomatic lingual thyroid, surgical transposition of the gland with its vascular supply intact seems to have superior results to those obtained by surgical ablation and autotransplantation. However, the procedure should be simple, reproducible, reliable, and cause less morbidity as well as providing simple access to and evaluation of the gland postoperatively. METHODS: We present the case of a 33-year-old female with lingual thyroid who was treated by transposing the whole gland to the lateral pharyngeal wall through a lateral pharyngotomy incision. The transposed lingual thyroid was nourished by a random tongue muscle pedicle flap. RESULT: At the 5-month postoperative stage, iodine scanning reviewed the radioactivity uptake of the transposed gland. Even though the patient was not on postoperative thyroid hormone supplement, her thyroid function gradually returned to normal after initially showing hypothyroid postoperatively. CONCLUSION: This new technique for transposition of lingual thyroid is simple and reliable and should be considered as an alternative method in the management of symptomatic patients.

Heterotopic pancreas: a difficult diagnosis.

Heterotopic pancreas is a rare disease. We evaluated 17 patients treated surgically at our hospital. Epigastric pain (77%), abdominal fullness (30%), and tarry stools (24%) were the three most frequent symptoms and signs. The lesions were diagnosed as gastroduodenal tumors by gastroduodenoscopy (67%) or upper gastrointestinal series (71%). Among these, only one gastric submucosal tumor was considered to be heterotopic pancreas preoperatively. Three patients were found to have gastric tumor by abdominal ultrasound. Computed tomography, small-intestinal series, barium enema, endoscopic retrograde cholangiopancreatography, angiography, and cholescintigraphy did not help in disclosing lesion. In about half of the patients, the lesions were located at the stomach. Tumor size varied from 1 to 3 cm. Surgical excision relieved symptoms. These findings indicated heterotopic pancreas is still a difficult disease for diagnosis, regardless of the improvements of diagnostic tools and techniques.

[Atopic stomach polyp as the cause of invagination of Meckel's diverticulum]. / Dystoper Magenpolyp als Ursache der Invagination eines Meckel'schen Divertikels.

We report a 16 year old boy with suspected appendicitis. Besides leucocytosis a roll was palpable in the right lower quadrant. The enema with water soluble contrast medium revealed an invagination. Intraoperatively, reduction was possible. The cause was an inverted Meckel's diverticulum. In this diverticulum we found a large perpendicular hyperplasiogenic gastric polyp as the cause of inversion and invagination.

[Gastrointestinal hemorrhage secondary to heterotopic jejunal pancreas]. / Hemorragia digestiva secundaria a páncreas heterotópico yeyunal.

A case of heterotopic pancreas located in the small intestine is herewith presented. The 19-years male patient had been admitted three times since 1991 for an anemic syndrome secondary to melena. The origin of the hemorrhage was not determined with the studies performed (intestinal transit, opaque enema, esophagogastroscopy, colonoscopy and gammagraphy with technetium 99). On the last admission a vascular lesion low flow was observed on arteriography of the upper mesenteric artery on the mesenteric edge of the proximal jejunum. Following laparotomy a small tumor was found in the jejunum and 5 cm of the intestine including the tumor was resected. The anatomic pathologic results demonstrated a heterotopic pancreas.

Alimentary tract duplications in infants and children.

This is a review of 30 duplications of the alimentary tract in 28 patients treated at the Surgical Unit of the Children's Department of the Medical University of Pécs, Hungary, and at the Department of Pediatric Surgery of the Medical Academy of Dresden, Germany, from 1964 to 1989. The ages of patients ranged from 1 day to 13 years, 80 percent were less than 2 years of age at initial presentation. There were 6 thoracic, 20 abdominal and 2 thoraco-abdominal duplications. Distended abdomen, vomiting, bowel obstruction and palpable abdominal mass were most frequently encountered. Plain thoracic and abdominal x-rays, ultrasonography, barium esophagogram, barium meal and enema were the most common diagnostic procedures. Emergency operative intervention was required in 18 patients. One infant died of an unrelated disease. Twenty-three duplications were cystic and 3 tubular. One patient had an appendiceal duplication, and another patient a flat lumenless duplication located on the perineum close to the anal opening. The surgical procedure--removal of the duplication--should not be more radical than necessary to eliminate the potential complaints and prevent recurrence. During surgery the common blood supply shared between the duplication and the native bowel must be carefully protected to avoid undue sacrifice of normal bowel.