Rapunzel syndrome with a long tail.

A rare form of gastric trichobezoar extending into small bowel with varying gastrointestinal symptoms is known as Rapunzel syndrome. A case history of 6 years old boy is described. This is the twelfth patient with Rapunzel syndrome in the literature.

Gastric teratoma: a rare cause of gastrointestinal bleeding.

Gastric teratoma is a rare tumor of infancy, almost exclusively found in males. We report a 6-month-old male child with gastric teratoma who presented with gastrointestinal bleeding; he was treated successfully with local excision of the tumor.

Morgagni hernia with Down syndrome: a rare association -- case report and review of literature.

Morgagni hernia is a rare diaphragmatic hernia accounting for only 2% of the congenital diaphragmatic defects. A case of Morgagni hernia was diagnosed radiologically in a 12-months-old male with Down syndrome, with recurrent respiratory distress and chest deformity. The 2-dimensional echocardiography was normal. The diagnosis of Morgagni hernia was confirmed by barium studies. The patient underwent a corrective surgery at 18 months of age following which his symptoms subsided. Literature review revealed only 18 cases of Morgagni hernia with Down syndrome reported till date, with age of presentation varying from neonatal age group to 12 years of age. The mode of presentations varied from asymptomatic detection to severe respiratory distress. The possible mechanism of association and its clinical implication has been discussed. The case emphasises a need for diaphragmatic hernia to be looked for as a possible cause of respiratory distress in Down syndrome.

Infantile hemangioendothelioma.

Primary hepatic tumors are uncommon in children and account for only three per cent of the tumors in children. Infantile hemangioendothelioma is a rare benign hepatic tumor arising from mesenchymal tissue. Most of the cases present before six months. An unusual presentation and progression of infantile hemangioendothelioma is reported in a 19-month-old female child. The diagnosis was arrived at by radiological and histopathological examination. The patient underwent excision surgery, following which made an uneventful recovery. On follow-up at six months, patient was asymptomatic with no evidence of recurrence.

Tar syndrome with unilateral absent radius and associated esophageal atresia: a variant?

We report a male child with Thrombocytopenia and Absent Radii (TAR) syndrome but with single absent radius and associated tracheoesophageal fistula which has never been reported till date.

Anterior tibial artery pseudoaneurysm.

Traumatic pseudoaneurysms are rare in civilian trauma, more so in childhood. A case of anterior tibial artery pseudoaneurysm is described which was successfully treated non surgically by percutaneous coil embolisation, thus questioning the need for surgery in peripheral pseudoaneurysms.

Isolated jejunal varices.

Isolated jejunal varices are an uncommon manifestation of portal hypertension. A one and a half year old boy presented with recurrent, massive gastrointestinal bleeding from jejunal varices. The bleeding site was identified at exploratory laparotomy. Jejunal resection and anastomosis resulted in complete resolution of the bleeding and there has been no recurrent bleeding over an eight month follow-up period.

Intrathoracic enteric foregut duplication cyst.

A one month old male child presented with respiratory distress since day 10 of life. There was intercostal retraction and decreased air entry on the right side. Investigations revealed a well defined cystic mass in the posterior mediastinum with vertebral anomalies, the cyst was excised by posterolateral thoracotomy. Histopathology revealed it to be an enteric foregut duplication cyst.

Esophageal endoscopic sclerotherapy in children using 3% aqueous phenol.

OBJECTIVE: To study the efficacy of endoscopic variceal sclerotherapy (EST) in controlling acute variceal bleeding and preventing recurrence of bleeding from esophageal varices in children. METHODS: Ninety children (mean age 7.3 +/- 3.0 years) with portal hypertension [extra-hepatic portal venous obstruction (EHPVO) 83, cirrhosis 7] presenting with hematemesis and/or melena were subjected to EST using 3% phenol in water as sclerosant. RESULTS: Active variceal bleeding could be controlled in 31 of 34 (91%) cases. Varices could be obliterated in 87% of patients with a mean of 5.4 +/- 2.5 injection sessions. Pre-obliteration variceal rebleeding was observed in 15% of patients. Complications such as esophageal ulceration, stricture and perforation were observed in 32%, 4.5% and 1% of patients respectively. Strictures responded to dilatation whereas perforation responded to conservative treatment. Recurrence of varices was seen in 22% of patients at a mean interval of 5.8 +/- 1.9 months. The mortality in the emergency group was 9.5% and nil in the elective group. Ten percent of patients required surgical intervention. CONCLUSION: EST with 3% phenol in water is effective in controlling active bleeding as well as preventing recurrent bleeding from esophageal varices in children.

Colloid carcinoma of rectum in a 11 year old child.

The rarity of rectal carcinoma in children has prompted us to report this patient who presented with bleeding per rectum and constipation. Histopathological examination of biopsy revealed the growth to be a colloid carcinoma of rectum and it was inoperable on exploratory laparotomy. There are three factors which contribute to an overall poor prognosis of rectal carcinoma in children viz. delay in diagnosis, advanced stage of disease and poorly differentiated histology.

Crossed renal ectopia.

A 2 year old male child presented with mass on right side of abdomen. The clinical diagnosis of renal lump was made and rest of the examination did not reveal any abnormality. His intravenous pyelography showed non-functioning left kidney. Cystoscopy and ascending pyelography on left side showed normal ureteric orifices and crossing of the left ureter to opposite side. Ectopic kidney was removed without any damage to the lower pole of the right kidney.

Splenic cyst.

An 11 year old boy presenting with cystic lump in left hypochondrium was diagnosed to have splenic cyst and treated successfully by splenectomy. Large, infected cyst involving hilum was the indication.

Adenocarcinoma of the colon in a child.

A rare case of a 10 year old boy presenting with history of lower GI bleeding for one year and acute intestinal obstruction was diagnosed as adenocarcinoma of the colon on exploration. One year follow-up after radical colectomy did not show any recurrence.

Benign neonatal pneumoperitoneum--an enigma.

A case of neonatal pneumoperitoneum in absence of a bowel perforation or intrathoracic pathology has been reported. The cause of such a rare manifestation remains to be established.

Chylothorax--a conservative approach (a case report).

Lymphatic blockage due to a non-specific inflammation probably of a filarial origin caused dilatation and ectasia of lymph channels at thoracic inlet of a child. Transudation of lymph through these channels led to chylothorax. The present case report highlights the significance of conservative approach towards this complex problem.

Developmental anomalies associated with hypospadias.

Hundred and thirty patients with hypospadias were studied between 1983 and 1990, with an aim of analysing the associated anomalies in these patients. Urogenital anomalies were observed in 30% and extra-urogenital anomalies were noted in 16%. Undescended testis was the most frequently seen anomaly, having been noted in 6.9% of 130 cases. Vesico-ureteral reflux was present in 5.4% and inguinal hernia in 3.8%. The incidence of these anomalies increased in proportion to the severity of penile deformity. The present article emphasizes the need for systemic urological and endocrinal examination, especially in severe cases of hypospadias.

Neurogenic tumor of the urinary bladder (a case report).

Neurogenic tumors of urinary bladder are rare. A ganglionure fibroma arising from the trigons of urinary bladder in two year old boy presenting with hematuria, is described here.