ABSTRACT: Rokitansky Kuster Hauser Syndrome consists of varying degrees of Mullerian agenesis with associated anomalies, commonest being renal. Over the years, several methods of vaginoplasties have been described in literature. This paper described our experience with three cases of this syndrome; two of whom had associated rectovestibular fistula and the third, I double urethrae. A loop of sigmoid colon was used as a vaginal substitute in the first case, an ileal loop in the second, while the accessory urethra was used in the third case. All the girls have been followed up and showed good cosmetic results with an adequate vagina.

ABSTRACT: In this article retrospective study of 148 gastrointestinal perforations in children admitted in the pediatric surgical ward of Govt. Medical College, Jabalpur, MP, India, from 1978 to 1997 were analysed. Commonest etiology encountered in this series was the category of unknown , followed by enteric, appendicular, traumatic, viral, tubercular and miscellaneous. Commonest site of perforation was ileum, followed by perforation at unknown site, appendix, jejunum, colon and Meckel's diverticulum. The maximum number of cases were between the age of 10 and 12 years. Male to female ratio was 1.5: 1. Peak incidence was found to be in the rainy season. There was no mortality in appendicular perforations. In the rest of the cases, least mortality was in the group which underwent simple repair and peritoneal drainage followed by resection and anastomosis. Maximum mortality was seen in the group of patients in whom only peritoneal drainage was done.

ABSTRACT: The results of treatment have been analyzed in 367 consecutive cases of esophageal atresia (EA) with or without tracheo-esophageal fistula (TEF) over a period of 16 years (1976-1991) with respect to type of anomaly, Waterston's groups, gestational age, age at admission, weight, temperature and pneumonitis. Details of outcome were not available in 26 patients between 1976 and 1979; the results of treatment have been analyzed in 341 cases after 1980. Ninety per cent of the patients had EA with distal TEF. Survival was directly related to Waterston's group, gestational age, weight, temperature and absence of pneumonitis. None of the babies with core temperature less than 35 degree C survived. Survival was not determined by age at admission. Despite improved survival rates in recent years, the overall survival does not compare favourably with those reported from developed countries. The factors resulting in poorer results have been analyzed and remedial measures suggested.

ABSTRACT: In this retrospective study, the results of treatment have been analysed in twenty consecutive cases of congenital diaphragmatic hernia seen over a period of twenty years (1978-97) at Department of Surgery, Govt. Medical College, Jabalpur (MP), India. Cardinal features of respiratory distress, cyanosis and tachypnoea were mainly observed in neonates and were associated with high mortality. Non specific presentation was seen more often in late presentation. All patient underwent surgical treatment under general anaesthesia.

ABSTRACT: In this prospective study, routine bronchoscopy was performed in 26 newborn babies with esophageal atresia and tracheo-esophageal atresia and tracheo-esophageal fistula (EA & TEF) prior to thoracotomy for repair and the results were compared to 25 babies where repair was done without prior bronchoscopy. Preoperative bronchoscopy provided significant information and reduced the postoperative pulmonary complications but did not alter the survival rates in EA & TEF.

ABSTRACT: During an 11-year study period at the All India Institute of Medical Sciences, New Delhi, 'H'-type tracheoesophageal fistula was diagnosed in 10 patients representing approximately 2 percent of all babies seen with esophageal atresia and/or tracheo-esophageal fistula. The average age at presentation in this group was higher (2 months) than the common variety (2-3 days). Delay in diagnosis primarily stems from poor sensitivity of radiological studies. Once the diagnosis is made the treatment is straightforward. Bronchoscopic localization and cannulation of the fistula, just before definite surgery was possible in all the cases and is strongly recommended.

ABSTRACT: A 13-month old female child presented with Giant Megaureter with ureteral stenosis, with preservation of ipsilateral renal function. The diagnosis was established pre-operatively and an attempt to salvage the renal unit was done. The distinguishing clinical and roentgenographic features are discussed as well as the probable embryologic errors in development producing this condition.

ABSTRACT: Two hundred patients of esophageal atresia with or without tracheoesophageal fistula treated during a period of 8 years were studied with emphasis on magnitude of gap between two ends of esophagus. Patients were divided in two groups, one group (154 patients) with gap less than 2 cm and other group (46 patients) with gap more than 2 cm. Mortality and complications were noted with reference to distance between the two ends of esophagus. There was high morbidity and mortality in patients with long gap. Surgical technique, proper mobilization of proximal pouch and tension free anastomosis are prerequisite for a final outcome.

ABSTRACT: A case report of acute diaphragmatic rupture caused by blunt trauma to the abdomen by fall of a pillar over a five year old girl. Acute diaphragmatic rupture in pediatric age group is very rare and usually results from automobile accidents and penetrating injury. The presentation may be acute or chronic. A high index of suspicion is requires for the diagnosis of the condition. Diagnosis is made by proper history and X-ray. Treatment of choice is laparotomy and repair of the diaphragmatic tear in layers.

ABSTRACT: This paper addresses one of the most important problems confronted by the surgeon called upon to manage esophageal atresia, namely when this is associated with a long gap between the two esophageal segments. In recent times, there have many articles devoted to the management of this potentially difficult problem, with many differences in opinion emerging including disagreement as to its precise definition. To avoid repetition, reference is made to earlier publications on this subject, based upon experience at the Royal Children's Hospital , Melbourne (1-3) Although there are various definitions, Our current view is that the use of the term. 'long gap' should be limited to esophageal atresia with or without a proximal fistula- particularly the latter. It is not considered to be a suitable term for those patients with esophageal atresia with a distal fistula, with a significant gap between the esophageal segments. Regardless of the definition, it has become clear there are several options for the management of the long gap. This is not surprising, and is in keeping with the philosophy expressed in a recent Editorial (4), that this is the era of option-it is no longer possible to consider there is only one way to approach a specific problem.

ABSTRACT: A 3 year old girl with neuroblastoma with preoperative diarrhoea and hypokalemia developed sudden intra operative hyperkalemia. The possible etiology and treatment are discussed.

ABSTRACT: Total cleft sternum is a rare congenital malformation. We here in report a case of total cleft sternum repaired in neonatal period. Stress is given on the technique of repair and its advantages when performed in neonatal period.

ABSTRACT: Pre-and postoperative cortisol levels were measured in clotted blood of 27 neonates who underwent surgery for esophageal atresia with or without tracheo-esophageal fistula. The preoperative and 6 hours postoperative values were 40.6 plus minus 29.0 and 41.5 plus minus 33.0 meug/dl respectively (p-value not significant). These values were further analyzed with regard to various clinical and biochemical parameters. The results indicated that factors which did not significantly affect the postoperative cortisol response included age, color, temperature, weight, pneumonitis, respiratory distress, hemoglobin, blood sugar, duration of surgery, sedation, anesthetic complications, starvation and ventilatory support. The changes with regard to blood transfusion requirements were almost significant (p less than 0.06) while the amount of blood loss was directly proportional to increase in the cortisol response (p less than 0.02).

ABSTRACT: A rare association of undescended testes and progeria is presented with a review of the literature. This entity is of considerable interest because of its striking features of premature aging.

ABSTRACT: A rare case of diphallus with imperforate anus reported.

ABSTRACT: Eighty neonates with esophageal atresia and tracheo-esophageal fistula underwent end-to0end esophageal anastomosis. Of these, 15 (18.75 percent) developed anastomotic leakage; all but one had 'major' leaks. Patients with major leaks (n=14) developed respiratory distress with saliva or gastric juice in chest drain within one week of surgery. Only one of these was taken up for re-thoracotomy, others had respositioning of drains and some had extra tubes inserted. Only 2 out of 13 survived the catastrophe. One neonate had a minor radiological leak only and was successfully managed conservatively. Of the various risk factors studied, use of braided silk suture, and distance between esophageal pouches were found to be statistically significant. Livaditi's myotomy was associated with high leak rate, possibly because of learning phase. Retropleural approach was found to have no advantage over transpleural, once a major leak had occurred. We conclude that anastomotic leak following esophageal repair still has high morbidity and mortality and an aggressive surgical intervention is needed for improved outcome.