ABSTRACT: Beckwith Wiedemann syndrome is characterized by Exomphalos, macroglossia and gigantism. It is associated with life threatening hypoglycemia in the neonatal period and an increased risk of solid organ tumors in the long term. Although exomphalos is a common condition seen in our country, this entity has not been previously reported from India. Authors report such a case and its outcome. Exomphalos is the commonest abdominal wall defect seen in our country. Although there is no published series on the outcome of exomphalos in neonates from our country, exomphalos major has a high risk of operative mortality in the neonatal period. Beckwith Wiedemann syndrome is an association of examphalos, gigantism and macroglossia. Life threatening complication of hypoglycemia in the neonatal period and a long term risk of solid organ tumors are further problems.

[ABSTRACT]   Full text not available   

ABSTRACT: Orchidopexy in a child below 2 years of age is a standard modality of treatment for undescended testis. Extended retroperitoneal dissection with inguinal dissection is a safe and effective procedure for inguinal as well as high-undescended testis. It leads to increased cord length without vascular compromise for its scrotal placement and later function. This is important for subsequent fertility. This study evaluates prospectively, the results of extended retroperitoneal dissection, performed in 60 cases of undescended testis with regard to permanent testicular position, and testicular atrophy.

[ABSTRACT]   Full text not available   

ABSTRACT: A 1-year old girl who presented with abdominal distension and tachypnoea was found to have a large, palpable abdominal mass extending over the right hemiabdomen. Abdominal ultrasound showed a huge, mixed multicystic and solid mass in the right hemiabdomen and epigastrium. Surgery revealed a massive mesenchymal hamartoma of the liver (MH) arising from the right lobe. Massive intraoperative hemorrhage during mobilization of the tumor necessitated subtotal excision of the tumor after clamping its base. The available literature on MH of the liver is reviewed.

[ABSTRACT]   Full text not available   

ABSTRACT: A case of Melanotic Neuroectodermal Tumor of Infancy, presenting as tumor arising from the maxilla in a 5-month old baby, is presented. On exploration, the characteristic melanotic pigmentation in the tumor tissue, which was confirmed by histopathological examination of the resected specimen, clinched the diagnosis. The rarity of the condition is highlighted and the relevant literature reviewed.

[ABSTRACT]   Full text not available   

ABSTRACT: Melanotic Neuroectodermal Tumor (MNET) is an interesting neoplasm seen in children under 1 year of age. The clinical behavior of the tumor is quite different from that of other tumors of neuroectodermal origin. All the 5 cases of MNET that are reported in this study were under the age of 14 months. Four were males and 1 female. In 3 cases the tumor was located in the anterior maxilla, in 1 it was in the mandible, and in another in the skull. A local excision of the tumor with curettage of the bony wall was undertaken in 4 cases and hemimandibulectomy was done in 1. Four of them remined well, but in one case, the tumor recurred within 6 weeks of primary resection. The recurrent tumor was re-excsised and chemotherapy with Endoxan and Doxorubicin was administered. The histopathological picture of the recurrent tumor did not show any difference from the other 4 tumors, except that it was highly cellular with scanty stromal tissues. A review of the literature reveals that though the tumor is generally considered benign, malignancy can occur in 1.9 percent cases and in tumors of head and neck region, the incidence of malignancy is 2.3 percent.

[ABSTRACT]   Full text not available   

ABSTRACT: Pulmonary agenesis is a rare anomaly. Seven cases with unilateral pulmonary agenesis were presented at the Park Children's Center for Treatment and Research between 1989 and 1995. Five were boys and 6 had right sided agenesis. All presented in the first year of lie. Four patients had associated anomalies. Two patients had associated anorectal malformations, of these 2, 1 had a radial ray defect and a partial sacral agenesis. One patient had a radial ray defect without any anorectal malformation. Another patient had an intrathoracic right upper limb and absence of ribs on the right side. One patient died of pneumonitis of the solitary lung. Two patients from a neighboring state and a neighboring country are lost to follow-up. The clinical data (including the follow-up of 4 patients) and a review of the literature are presented.

[ABSTRACT]   Full text not available   

ABSTRACT: A retrospective review of abdominal tuberculosis in 25 children who underwent laparotomy is presented. There were 17 boys and 8 girls with the mean age of 6.6 years. Intestinal obstruction (80) was the commonest presentation. Pulmonary tuberculosis was present in only 2 cases. The commonest finding on laparotomy was a plastered gut mass. An analysis of management with review of literature is presented.

[ABSTRACT]   Full text not available   

ABSTRACT: Infantile hypertrophic pyloric stenosis is an acquired condition presenting between 3-12 weeks. It has rarely been reported in association with other gastrointestinal anomalies. Such an association can present in the postoperative period, after an abdominal surgery in a newborn, creating diagnostic and therapeutic dilemma. We report 2 such cases of gastric outlet obstruction which evolved in the postoperative neonate and in which the correct diagnosis took some time to be established. In a postoperative neonate, if there are symptoms or signs of an evolving gastric outlet obstruction, pyloric stenosis must be considered.

[ABSTRACT]   Full text not available   

Full text not available   

Full text not available   

Full text not available