Symptoms related to esophageal dysmotility are common following successful repair of esophageal atresia (EA) and tracheo-esophageal fistula (TEF). Esophageal manometry was performed in 27 survivors of EA/TEF at a mean age of 30.5 +/- 30.3 months and in 25 age-matched controls (mean age 36.8 +/- 22.6 months). The patients were also evaluated clinically to grade them into 3 result-oriented groups "Excellent", "Good" and "Fair", depending on pre-existing criteria evaluating the ability and ease of swallowing. Gastroesophageal reflux (GER) was evaluated with the help of a radionuclide scan. The mean esophageal and peak esophageal pressures and therefore also the lower esophageal sphincter (LES) pressure were lower in the patients (12.4 +/- 5.5, 34.9 +/- 20.4 and 12.2 +/- 6.8 mm Hg, respectively) compared to the controls (21.1 +/- 6.9, 62.3 +/- 19.3 and 16.8 +/- 4.3 mm Hg, respectively). The mean body pressures were highest with "Excellent" results and lowest with "Fair" results and the LES pressures followed a similar trend. Likewise, in the patients with GER, LES pressure was 12.0 +/- 7.1, 12.3 +/- 3.7, 11.0 +/- 5.7 and 6.9 +/- 5.6 mm Hg with nil, mild, moderate and severe GER, respectively. The pressure and contractility profile of the esophagus was abnormal in the majority of patients, even in the absence of symptoms.

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Fistulae between the hepatic artery and portal vein, known as hepatoportal arteriovenous fistula (HPAVF), most commonly occur secondary to trauma or malignancy. Congenital HPAVF is an extremely rare anomaly and only 11 pediatric cases have been documented to date HPAVF causes portal hypertension with the reversal of flow in the portal circulation, leading to hyperemia and congestion of the bowel, causing severe ascites, gastrointestinal bleeding, anemia, and malabsorption. The diagnosis is first made by Doppler sonography, then confirmed by angiography. HPAVF is most effectively treated by performing either ligation of the hepatic artery or percutaneous transcatheter embolization. We describe herein the case of an 8-month-old boy diagnosed to have congenital HPAVF by duplex Doppler sonography and confirmed by digital subtraction angiography, who was successfully managed by percutaneous transcatheter embolization of the feeding right hepatic artery. A review of the previously reported 11 cases of infants with this unusual anomaly, examining presentation, management, and outcome, is also presented.

Context:Spinal dysraphisms are congenital abnormalities of the spine due to imperfect fusion of midline mesenchymal, bony and neural structures. Imaging plays a vital role in their evaluation as significant portion of patients may present with concurrent anomalies that need to be corrected simultaneously to avoid repeat surgeries.Aims:The aims of the study were to evaluate Spinal dysraphisms using USG and MRI and to correlate imaging findings with operative findings in patients undergoing surgery.Settings and Design:Hospital based observational study conducted over a period of year.Materials and Methods:38 cases of both sexes and below 12 years of age with spinal dysraphism were studied. USG was performed in 29 cases where acoustic window was available for proper evaluation. MRI was performed in all cases. USG findings were compared with MRI findings and operative follow up was taken in 23 cases who underwent operative management.Statistical Analysis Used:Results were analysed using percentage and arithmetic mean.Results:39.47 % cases were male and 60.53 % cases were female. Neonatal period was the most common presenting age group. Closed spinal dysraphism (63.16%) was more common than open (36.84%). 79.31% cases showed full agreement between spinal USG and MRI examinations and 6 out of 20.69% showed partial agreement. On operative correlation, USG findings were confirmatory in 91.30% cases and MRI findings were confirmatory in 100% cases.Conclusions:USG can be used as the initial modality for evaluation of spinal dysraphism as well as for screening of suspected cases. MRI is indicated to confirm abnormal USG findings, which shows all concurrent abnormalities and also provides additional anatomical details relevant to surgical planning.

Aim:To determine the annual incidence of congenital malformations in Assam and to analyze the data.Materials and Methods:Data regarding babies born with congenital malformations in the state of Assam during the year 2006 were obtained through questionnaires and analyzed. The results were compared with similar Indian data.Results:The overall incidence of congenital malformation was 0.08%. This was considerably lower than similar published data from other states. Five hundred and eleven babies were born with congenital malformations, with 421 (82.4%) having major malformations. Males were affected more than females, 334 (65.4%) vs. 177 (34.6%). The gastrointestinal and genitourinary systems accounted for 26% and 25.8%, respectively. Malformation involving the central nervous system was more common in certain ethnic groups.Conclusions:The incidence of malformations in certain systems was at variance with the data from other states.