Biliary atresia (BA) is a cholangiodestructive disease affecting biliary tract, which ultimately leads to cirrhosis, liver failure and death if not treated. The incidence is higher in Asian countries than in Europe. Up to 10% of cases have other congenital anomalies, such as polysplenia, asplenia, situs inversus, absence of inferior vena cava and pre-duodenal portal vein, for which we have coined the term Biliary Atresia Splenic Malformation (BASM) syndrome. For these infants the aetiology lies within the first trimester of gestation. For others affected with BA, aetiology is more obscure and perinatal destruction of fully-formed ducts perhaps by the action of hepatotropic viruses has been suggested. Whatever the cause, the lumen of the extrahepatic duct is obliterated at a variable level and this forms the basis for the commonest classification (Types I, II, III). All patients with BA present with varying degree of conjugated jaundice, pale non-pigmented stools and dark urine. Key diagnostic tests include ultrasonography, biochemical liver function tests, viral serology, and (in our centre) a percutaneous liver biopsy. In some centres, duodenal intubation and measurement of intralumenal bile is the norm. Currently BA is being managed in two stages. The first stage involves the Kasai operation, which essentially excises all extrahepatic biliary remnants leaving a transected portal plate, followed by biliary reconstruction using a Roux loop onto that plate as a portoenterostomy. If bile flow is not restored by Kasai procedure or life-threatening complications of cirrhosis ensue then consideration should be given to liver transplantation as a second stage. The outcome following the Kasai operation can be assessed in two ways: clearance of jaundice to normal values and the proportion who survive with their native liver. Clearance of jaundice (<2 mg/dL or <34 µmol/L) after Kasai has been reported to be around 60%, whereas five years survival with native liver ranges from 40% to 65%.

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Aims and Objectives: Delayed management of anorectal malformation (ARM) increases the surgical and functional complications for the patient. We defined "delayed presentation of ARM" and reviewed our patients with ARM to find out the incidence and causes of delayed presentation. Materials and Methods: Patients satisfying the criteria of "delayed presentation of ARM" were involved. Detailed information of each patient including the mode of presentation, associated anomalies, plan of management and follow-up was obtained from the hospital records. Results: Between 2003 and 2006, 43 patients satisfied our criteria of "delayed presentation of ARM". There were 21 males and 22 females. Seventeen of these males presented with low-type ARM. Eleven of them were managed by a single-stage procedure. These "delayed presenters" had to live with constipation, inadequate weight gain and parental anxiety for a greater time. Analysis of the outcomes showed more functional complications in patients who had undergone failed perineal surgery previously. In females with low ARM, the procedure of choice was anterior sagittal anorectoplasty (ASARP). Single stage surgery provides good outcomes for most of low type of ARMs. High-type ARMs in males and females were managed by a staged procedure. Conclusion: "Delayed presentation of ARM" is a major group of ARM in our setup. The management and results of their treatment are not different from those of the early presenters. The most common cause of delayed ARM is wrong advice given by the health care providers followed by inadequate treatment elsewhere. Corrective surgeries taking second attempt in perineum always produces poor outcomes.

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Aims and Objective: To postulate a hypothesis to explain the embryogenesis of exstrophy bladder based on our clinical observations. Materials and Methods: In 27 cases of exstrophy, we measured the distance between the lowermost inguinal skin crease to the root of the penis (clitoris) (B) and the distance between the penis (clitoris) and the scrotum (labia majora) (C). These were compared with age, height and XP distance (distance between xiphisternum and symphysis pubis) matched control group of normal children. The distance between the lowermost inguinal skin crease and the penis (clitoris) (A) was measured in control group. Results: The observation was A = B + C. This implies that in exstrophy bladder, the position of the penis (clitoris) has moved cephalad from the lower border of A to the junction of B and C. Conclusion: Based on the observations, we postulate that abnormal origin of genital tubercle may be the cause of exstrophy bladder. The abnormal origin of primordia of the genital tubercle in more cephalad direction than normal causes wedge effect, which will interfere with the medial migration of the mesoderm as well as the midline approximation of mesodermal structures in the lower abdominal wall, thereby resulting in the exstrophy of bladder.

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Appendicitis epiploicae is described as a rare entity in an 8-year-old boy presented with the features of acute abdomen simulating acute appendicitis. Surgical exploration revealed a torsion of appendices epiploicae of the cecum. The excision of infarcted epiploicae with seromuscular inversion resulted in satisfactory recovery. Authors describe this uncommon cause of pediatric acute abdomen along with the review of pertinent literature.

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This report is to present and discuss an extremely rare association of situs inversus with duodenal atresia in an 11-day-old male neonate born full term and weighing 1.9 kg. The baby presented with recurrent bilious vomiting. Babygram revealed situs inversus and duodenal obstruction. Echocardiography showed dextrocardia with a small ASD. Exploration confirmed a duodenal diaphragm with a central perforation between the third and fourth part of the duodenum and situs inversus. The literature search revealed 20 cases reported so far.

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Rectal atresia is a rare condition in which the anus and sphincter muscles are normally developed, with usually no fistulous communication with the urinary tract. We describe an unusual case of membranous rectal atresia with recto-labial fistula. It was treated by blind perforation of rectal membrane with lay opening of fistlous tract.

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Aims and Objectives: The purpose of this study was to determine the percentage, etiology, associated injuries and outcome of the children with liver trauma. Materials and Methods: In a retrospective review all children below 15 years of age who had sustained abdominal trauma were included. the study duration was January 1994 to December 2004. Results: Out of a total number of 436 trauma patients 34 were identified to have liver trauma (including one death). The median age was 5.89 and range: 1 to 14 years). Boys accounted for 76.4% ( n = 26), and the most common cause was motor vehicle injuries, accounting for 41.17% ( n = 14). Nine children underwent surgery (26.4%). Head injuries were the most common associated injuries, and the mortality rate was 2.94% ( n = 1). Conclusions: The liver remained the second most commonly injured intraabdominal organ and nonoperative management is the preferred treatment for hemodynamically stable patients.

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Injuries to the pancreas by blunt trauma are uncommon. The association of pancreatic injury with acute portal vein thrombosis secondary to blunt trauma abdomen is furthermore rare. The early diagnosis of the pancreas with injury to the portal vein is challenging and difficult. These injuries are associated with high morbidity and mortality, particularly if the diagnosis is delayed. Accurate and early diagnosis is therefore imperative and computed tomography plays a key role in detection. We present a case of child with a rare combination of pancreatic laceration and acute portal vein thrombosis following a blunt trauma to the abdomen. With extensive literature search we found no such cases has been described previously.

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Jejunoileal atresia is one of the common causes of neonatal intestinal obstruction. Intestinal perforation with meconium peritonitis in the neonatal period, which carries a high mortality rate, is also common. The association of jejunal atresia with idiopathic ileal perforation is very rare.

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Blunt trauma to the parotid gland is extremely rare and requires considerable force. We present a unique case in which a child sustained parotid injury without any associated injury to the facial skeleton, parotid gland and ductal structures and managed successfully. A literature search revealed that this type of injury has not been reported previously.

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Aims and Objectives: The aim of this study is to find easier way of home care while obviating the colostomy before single stage pull through operation. Materials and Methods: From August 2005 to December 2006, eight cases of neonatal Hirschsprung disease were treated. Mean age 4.5 (2-6) day/old with absent anorectal inhibitory reflex, rectosigmiod disease in Barium enema, positive Acetylcholine esterase (Ache) staining, good response to rectal washout. They underwent botulinium toxin injection (5 unit /kg/quadrant) in four quadrant intrasphincteric. They were followed until pull through operation in 8-10 weeks post injection. Results: Four of 8 (50%) cases only needed rectal washout for three to five days post injection until pull through operation, two had decrease in number of rectal washouts /day and the remaining two underwent colostomy five days post injection because of no response. Conclusion: Botulinium toxin injection can help in palliative care in patients with Hirschsprung disease who are waiting for colostomy or definitive pullthrough. It gives an option of eaiser home care for these patients.

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Cystic pancreatic tumors are rare in children and the immature cystic teratoma of the pancreas is even rarer. A review of the world literature shows 18 documented cases involving all the age groups. The preoperative evaluation of this lesion is rather questionable, with definitive diagnosis taking place intraoperatively. We report the 19 ^th case, in a 5-month-old male child. The clinical presentation and preoperative diagnosis of this anomaly are discussed.

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Large solid ovarian lesions are considered malignant in nature in pediatric and adolescent age group. We present an adolescent girl who had large solid ovarian lesion, with negative tumor markers. She underwent laparotomy and right oopherectomy. Histopathology revealed that the lesion was massive ovarian edema. This is an extremely rare lesion of ovary and is benign in nature. Very few case reports are available in English literature. Hence we suggest that massive ovarian edema should be considred as one of the differential diagnosis in all the patients having large solid ovarian lesions with ngative tumor marker assay. Ovarian preservation with the help of frozen section analysis should always be considred in these patients.

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