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Aims: (1) To describe the technique of Extended Portal Dissection in patients with Biliary Atresia. (2) To analyse the short-term outcome in terms of stool colour in the patients undergoing Kasai's operation with either Limited or Extended Portal Dissection. (3) To perform a comparative analysis between these patients. (4) To arrive at a conclusion regarding the merit of Extended Portal Dissection. Methods: 41 cases of Biliary Atresia from Jan 2000 to April 2007 were analysed retrospectively. Two groups were formed - Group A which included patients between 2000 to 2003 who underwent Limited Portal Dissection (LPD), and Group B included patients of Biliary Atresia from 2004 till April 2007 who underwent Extended Portal Dissection (EPD). Data was collected regarding the number of patients in each group, sex distribution, Age of patients at the time of surgery in 3 subgroups - 1. less than 1 month. 2. 1-3 months 3. More than 3 months, Histopathology report for the size of the biliary channels and the liver architecture, and the colour of stools within 1 month post-operatively. A comparative analysis was then performed between the 2 groups to assess whether the Extended Portal Dissection was beneficial in the short-term outcome following Kasai's portoenterostomy. Results: Of the 41 patients, Group A had 19 and Group B, 22 patients. Age distribution at the time of surgery showed 1 (5%) patient less than 1 month of age in Group A and no patient in Group B. 10 (53%) patients were in the age group 1-3 months in Group A and 9 (41%) in Group B, whereas 8 (42%) in Group A and 13 (59%) in Group B were more than 3 months of age. Group A had 12 (63%) males and 7 (37%) females versus 16 (73%) males and 6 (27%) females in Group B. A Liver biopsy histopathology report showing > 150 microns of Biliary channels was found in 15 (79%) in Group A and 16 (73%) in Group B. Cirrhosis on histopathology was also evaluated and was found to be present in 14 (74%) in Group A and in 18 (82%) in Group B. On analysis of the post-operative recovery upto 1 month following the surgical correction of Biliary Atresia, it was found that 3 patients in each group were lost to follow-up. On evaluating the stool colour in the post-operative phase, 22 patients of 41, i.e. 54% showed improvement in the colour of stools and passed yellow stools within 1 month post-operatively. Of these 8 (50%) belonged to Group A and 14 (74%) from Group B. Conclusions: Group A included patients of Biliary Atresia who underwent portal dissection limited to the fibrous area of the hilar plate as described by Kasai. In the patients of Group B, Extended portal dissection involved dissection of the hilar plate of about 4-5 mm in depth at the porta and lateral dissection. Group B had more patients over 3 months of age, had less patients with histopathological biliary channels more than 150 microns and more patients with liver cirrhosis as compared with Group A. Inspite of the above findings, the short-term outcome in terms of stool colour was better in Group B versus Group A. This suggests that Extended Portal Dissection is beneficial in improving the outcome of patients with Biliary Atresia. [ABSTRACT FROM AUTHOR]

Aims: 1.To compile data on patients of congenital adrenal hyperplasia (CAH). 2. To evaluate the results of management of patients with CAH. 3. To suggest improvised methods of treating these patients. Materials and Methods: Data was collected retrospectively from files of patients who were treated in our hospital from 1990 to 2006. The records were reviewed with respect to age of presentation, sex distribution, mode of presentation, type of CAH, medical therapy, operative procedure and outcome. The follow-up period ranged from 6 months to 16 years. Results: A total of 75 patients were treated in our center from 1990 - 2006. Majority of the children presented during infancy (45%), 28% presented during the neonatal period, 15% between 1 to 5 years of age, and late presentation between 5 to 10 years was seen in 12%. The most common mode of presentation was as Ambiguous Genitalia (55%), Vomiting and Diarrhoea being 36%. 9% of children (males) presented with precocious puberty. Additionally, hyperpigmentation was noticed in most of the patients and hypertension was seen in a couple of male CAH patients. The Male: Female ratio was found to be 1:3. Majority of the patients were Salt-losers (85%), while only 15% were simple virilizers. Except for 4 (5%) patients of 11Beta hydroxylase deficiency, all others (95%) had 21 hydroxylase deficiency. Medical therapy (steroids) were started in all patients as soon as the diagnosis of CAH was made. A high percentage of patients (36%) were lost to follow-up (LTFU) or refused treatment. Of the 64% patients on regular follow-up, Recession Clitoroplasty was done in 37%, One-stage Clitorovaginoplasty was done in 37%, Two-stage reconstruction was done in 7%, 3% underwent Hysterectomy and salpingo-oophorectomy, and 17% of patients have not yet been operated upon. 4 (8%) of the female CAH patients preferred to be reared as males. The external genitalia appearance was found to be satisfactory in all patients, though on detailed examination, 13% girls operated in the past had narrowed introitus, which was treated with vaginal dilatations. Conclusions: CAH is a complex physical and psychological problem due to imbalance of steroids. This condition needs to be managed adequately by the pediatrician/pediatric endocrinologist as well as pediatric surgeon. Early detection and replacement of steroids is the most vital step in the management. Patients who presented late or were non-complaint to medical therapy have several problems as regards the external appearance in spite of being phenotypically females. Surgical correction plays a major role in restoring the ambiguity of the genitalia. In order to improve the overall outcome of these patients who can achieve a normal life, the rate of non-compliance to treatment and LTFU has to be reduced by joint clinics, support groups, counseling and public education. [ABSTRACT FROM AUTHOR]

Aim: 1. To present an unusual type (rhabdoid) of soft-tissue sarcoma of extremity. 2. To emphasize the importance of rapidity of management in treatment of soft tissue sarcomas, especially in infants. Case report: Two month female child admitted with rapidly increasing swelling on the right forearm since one month. MRI showed a soft tissue lesion on the volar aspect of forearm measuring 7.4x3.6x2.7 with no bony involvement. Wide excision of the mass was done immediately after working up the child for anaesthesia. Histopathological examination revealed undifferentiated sarcoma-rhabdoid phenotype. Patient was advised chemotherapy and radiotherapy but due to some family problems, the parents requested discharge and consented to undergo chemotherapy and radiotherapy within the next week. But he was lost to follow-up for a month and came back with a large recurrent mass with erosion of the bone. Chemotherapy was initiated; there was a slight decrease in the size of the mass, followed by surgery which resulted in above elbow amputation. In spite of having wide clear margins on gross examination, the histopathology showed positive margins. Immediate post-operatively child started on chemotherapy, but due to hepatocellular damage had to be discontinued. While waiting for the child to recover, she developed axillary nodes which proved to be metastatic undifferentiated sarcoma on FNAC. No further chemotherapy or radiotherapy could be given. Result: Due to the aggressive disease and rapid progression of the tumor complicated by liver failure due to chemotherapy, the child succumbed to the tumor. Conclusions: 1. Malignant Rhabdoid tumor is a rapidly progressive and highly malignant neoplasm. 2. Chemotherapy is usually not well tolerated by infants. Delay of even one week in the multimodality management of this type of tumor is detrimental. 4. Undifferentiated soft tissue sarcoma, especially rhabdoid type is an uncommon occurrence in the extremities, first described in the kidney. [ABSTRACT FROM AUTHOR]

Aim :To present a rare entity of Allgrove's syndrome also known as Triple A syndrome which presented to us in siblings. Case Report: Siblings 3 and 4 years old presented to us with primary complaints of persistent non bilious vomiting after each feed since 6 months of age. Since the children were not gaining weight and showed failure to thrive, mother brought the children to us from a village in Uttar Pradesh after prolonged treatment for vomiting in her hometown. On enquiry, there was history of alacrimia as well since birth. Examination confirmed the alacrimia. The other positive feature was hyperpigmentation of the gums and the extremities. With these features, persistent vomiting, alacrimia and hyperpigmentation child was investigated further with a suspicion of Triple A syndrome. Investigations revealed a raised Serum ACTH level, depressed Serum cortisol levels and Achalasia cardia on Barium swallow. Both children were started on steroids and subjected to Heller's cardiomyotomy with partial fundoplication. Pneumatic dilatation was not done as facilities were not available, nor these children were suitable candidates for pneumatic dilatation. Both children had an uneventful recovery post-operatively and were sent home on 10th POD on full feeds. A follow-up Barium swallow revealed a normal cardio-esophageal junction. Result: The hyperpigmentation of the gums and extremities decreased with steroid treatment and the vomiting episodes settled completely following Cardiomyotomy. Conclusion: Triple A syndrome, or Allgrove's syndrome is an uncommon entity wherein the incidence is not known. Anecdotal reports mentioned in literature describe this syndrome. The diagnosis of this syndrome can be established by knowledge of this entity and clinical suspicion in patients who present with persistent vomiting or neurologic dysfunction which is a more common presentation as reported in the literature. [ABSTRACT FROM AUTHOR]

Aim: 1. To emphasize the need for treatment of selected patients with haemangiomas, especially of the face, and 2. To establish the efficacy of Injection Bleomycin as sclerosant for haemangiomas. Case Report: 6 month female child presented with increasing swelling over the entire right side of face since 15 days of age. On examination there was a large haemangioma involving the right temporal region, right upper eyelid causing eversion of the eyelid, the preauricular region, the parotid region, the infra and retroauricular region and extending upto the right upper neck, submandibular region. Patient was selected for Injection Bleomycin therapy and subjected to six courses of the sclerotherapy regime three weeks apart the dose being 0.66 units/kg/dose diluted in 1:1 normal saline. Result: The haemangioma started regressing with two doses of Injection Bleomycin and after completion of 6 doses regressed almost completely. The upper eyelid eversion also settled remarkably well without scarring. There was a small residual lesion in the infra-auricular region which also regressed after two more injections of Bleomycin. Conclusion: Hemangiomas which are rapidly progressing and those of the face must be managed at the earliest. Traditionally steroids have been used for treating these lesions which apart from the adverse effects do not cause permanent regression of hemangioma. The use of Bleomycin has given excellent cosmetic results without recurrence in many patients, as shown by this classical case. [ABSTRACT FROM AUTHOR]

Aims: 1. To report the presence of calculi in ureterocele. 2. To highlight that a common pediatric surgical problem such as urinary calculi can have surprises. Case report: Increased frequency of micturition and dysuria of one month duration were the presenting features in a two year old male child. Clinical examination was unremarkable. Investigations revealed 2 bladder calculi, which was confirmed on ultrasound. No other pathology was detected on ultrasound. Intra operatively, during a suprapubic cystolithotomy, bladder calculi could not be found. On extending the bladder incision, bilateral ureteroceles came as a surprise. Both the calculi were found in the left ureterocele. The right ureterocele was incised and the left one was excised. Both the calculi were removed and left ureteric reimplantation was done. Patient recovered uneventfully. After 3 months detailed investigations were done with DTPA Renal scan, an IVP and a MCU. Result: Child is free of urinary tract infection, though has been continued on chemoprophylaxis in view of a Grade I right vesico-ureteric reflux. The post-operative ultrasound does not show any upper tract dilatation and renal scans show bilateral single systems and good renal function. Conclusions: Urinary calculi are managed by all the pediatric surgeons. Association of calculi in ureterocele in children is a rare entity and has not been extensively reported in the literature. In the absence of any symptoms and an ultrasonography which fails to diagnose ureterocele, calculi in a Ureterocele could be misdiagnosed as bladder calculi as in this case. [ABSTRACT FROM AUTHOR]

Aim: To highlight the uncommon presentation and site of congenital esophageal stenosis in a neonate with associated microcephaly. Case Report: 3 day neonate presented with history of non bilious vomiting after feeds and frothing since birth. The baby was hospitalized elsewhere and after a failed attempt to negotiate an infant feeding tube the baby was transferred to us. The baby on examination had microcephaly and micrognathia. Dye study done showed stenosis at the distal third of oesophagus at T8. Child was taken up for surgery and an obvious stenotic ring about 0.5 cm was found at the junction of middle and lower third of the esophagus. No cartilaginous remnants could be palpated. An end to end esophago-esophageal anastomosis after resection of the stenotic segment was done across a transanastomotic feeding tube. Baby had a stormy post-operative course and feeding problems for about 3 weeks, but eventually could be fed orally and discharged a month after the surgery. A post-operative dye-study did not demonstrate a leak or stricture at the anastomotic site. Histopathology of the resected segment showed fibro muscular stenosis. A 6 month follow-up study confirmed a suspected gastro-esophageal reflux and child is awaiting fundo-plication for symptomatic gastro-esophageal reflux. Result: The baby had a stormy post-operative course and took about a month to settle. The developmental milestones are delayed and evaluation of the effects of microcephaly is being done. Conclusion: The unusual features of this case of CES are - i) Neonatal presentation. ii) Absence of tracheo-bronchial remnants which is the cause of early (neonatal) presentation, as per the literature. iii) Type III Congenital Esophageal Stenosis. iv) A very tight stenosis presenting like esophageal atresia / tracheo-esophageal fistula. v) Associated Microcephaly and micrognathia and probable mental retardation. [ABSTRACT FROM AUTHOR]

With the availability of several protocols in the management of Wilms' tumor, there is dilemma in the minds of the treating oncologists or pediatric onco-surgeons as to whether the child should receive upfront chemotherapy or should be operated upon primarily. It is necessary for us to understand why do we follow either of the protocols, NWTS which follows the upfront surgery principle or the SIOP which follows the upfront chemotherapy principle in all stages of the disease. While deciding which protocol to follow, it is imperative to know the pros and cons of the treatment strategies and also to study the outcome patterns in both the treatment regimes which is what this article highlights. In an attempt to compare all the differences in both the major protocols, it was realized that most of our patients in the Indian scenario present with advanced disease and thus poorer outcomes if intensive and appropriate treatment strategies are not utilized. Hence, it is imperative that we should study our own patients through the Indian Wilms' tumor study group and adopt the policies which improve the overall event free survival on a nationwide basis.