Gall Bladder Duplication with Choledochal Cyst: A Rare Entity.

A 4-year-old male child presented with complaints of abdominal pain and vomiting along with yellowish discoloration of the eyes. Investigations were suggestive of acute pancreatitis and double gall bladder (GB) with dilated common bile duct (CBD) with intraluminal calculi and Type II choledochal cyst. He underwent surgical resection of double GB with dilated CBD with hepatico-docho-jejunostomy. On follow-up, the patient was asymptomatic. Our case highlights the importance of preoperative diagnosis to deal with increased operative difficulty and complications.

A Rare Case of True Pancreatic Cyst.

Pancreatic cysts can be true or pseudocysts. True pancreatic cysts in children are rare clinical entities. We present a 23-month-old boy with a cystic lesion in the distal body and tail of the pancreas which on histopathology was found to be a rare true congenital simple cyst of the pancreas.

Type VI Choledochal Cyst - An Emerging Rare Entity (11th Pediatric Case of Type VI) with a Review of Literature.

Choledochal cysts (CCs) are abnormal dilatations of the biliary system. Usually, CCs are classified into five types. The sixth type (Type VI) is an emerging and rare type, reported the first case in 1991. We report this rare CC, Type VI seen in our experience for the first time. We have also reviewed the literature; only 26 cases of Type VI were found, including adults and children, ever since the first case has been reported in 1991. To the best of our knowledge, this is the 11th pediatric case report of a Type VI choledochal cyst.

Unusual presentation of superior mesenteric artery syndrome in a child.

Superior mesenteric artery syndrome (SMAS), also known as Wilkie's syndrome, is a rare cause of upper gastrointestinal tract obstruction. We report a case of a 10-year-old girl with persistent abdominal pain for over 3 months, who on extensive investigations was diagnosed with SMAS. She underwent a surgical procedure to bypass the obstructed portion of the intestine for relief of her symptoms.

Comparison between Suction Rectal Biopsy and Full-Thickness Rectal Biopsy in the Diagnosis of Hirschsprung's Disease.

AIM: The aim of this study is to compare suction rectal biopsy (SRB) with full-thickness rectal biopsy (FTRB) in suspected cases of Hirschsprung's disease (HD). MATERIALS AND METHODS: Between 2014 and 2018, we enrolled 41 consecutive children with suspected HD. We analyzed demographics, sex, age, clinical symptoms, radiological images, and biopsy reports. All the children had undergone X-ray of the abdomen and pelvis and contrast enema. All of them have undergone both SRB and FTRB, and their results were compared. RESULTS: Out of 41 children, 26 were male and 15 were female. The children were aged from 5 days to 12 years. All of them presented with delayed passage of meconium, abdominal distension, and severe constipation. They all were on oral laxatives. The sensitivity and specificity of SRB are 80.95% and 90.00% when compared to FTRB which has 100% and 100%, respectively. CONCLUSION: FTRB is the gold standard test for diagnosing HD. SRB may be a good screening test in suspected HD cases. SRB is not as equal and effective as FTRB for diagnosing HD.

Multidisciplinary Management for Intestinal Obstruction by Gel Ball Ingestion.

Foreign body ingestion is common in young children. Very few cases require surgical exploration for removal. We report a case of superabsorbent gel ball ingestion causing intestinal obstruction, managed by a combination of endoscopic and open surgical removal under intraoperative ultrasound guidance.

Mediastinal Extension of Pancreatic Pseudocyst: A Rare Pediatric Presentation.

Pancreatic pseudocysts are cystic cavities which are localized collection of pancreatic secretions, rich in amylase and other enzymes, present in and around pancreas, encased in a false epithelial lining of fibrous or reactive granulation tissue. Extension of a pancreatic pseudocyst into the mediastinum is rare. We are reporting a case of a 5-year-old child with mediastinal pancreatic pseudocyst which was successfully drained by cystojejunostomy.

Risk Prediction Scoring System to Predict the Postsurgical Outcomes of Biliary Atresia.

AIM: To find out association between liver function, liver histopathology and outcomes of biliary atresia (BA) following Kasai Portoenterostomy (KPE). MATERIALS AND METHODS: This is a retrospective study of children who underwent KPE at a single institute by single surgeon. The patient records analyzed and data of complete blood counts, liver function tests, coagulation profile and histopathology reports collected. The outcomes recorded as alive and jaundice free, alive but jaundiced, and deceased. Statistical analysis done using SPSS 23. OBSERVATIONS: Total of 148 children operated during January 2000 to December 2018. Of these, 26 matched inclusion criteria. The parameters assessed were percentage of direct bilirubin, ratios of Aspartate transaminase (AST) to Alanine transaminase (ALT); Gamma glutamyl transferase (GGT) to AST; GGT to ALT and Aspartate transaminase to platelet ratio index (APRi). Among histopathology reports, fibrosis grade and bile ductular size noted. Among 26, 16 alive and ten are deceased. Among 16 alive, all are jaundice free. Of the parameters, ratio of AST to ALT, APRi and grade of fibrosis found statistically significant and further analysis showed if AST to ALT ratio < 2.1, APRi < 1.8 and grade of fibrosis < four, irrespective of age at surgery, had 96.2 % probability of successful KPE. Based on these observations, a scoring system and risk prediction model constructed based on Receiver operating characteristic (ROC) curves which are first in BA management. RESULTS AND CONCLUSION: Although numbers are sufficient for statistical analysis, we further intend to validate the scoring system in a prospective trial. BA children can be subjected to risk prediction model and KPE performed in those who have a score less than seven and offered to those with score between eight and 16 out of 20. KEY MESSAGE: The scoring system and risk prediction model can guide in the management and post-operative follow up of children with biliary atresia.

Balloon venoplasty as primary modality of treatment in children with Budd-Chiari syndrome.

INTRODUCTION: Budd-Chiari syndrome (BCS) is a rare condition affecting children. It is characterized by occlusion of venous outflow from liver at the level of hepatic veins (HV) or inferior vena cava (IVC). The management of BCS in children revolves around forming new collaterals for venous outflow or by elimination of blockage in the venous outflow tracts. These can be achieved by balloon venoplasty (BV), transjugular intrahepatic portosystemic shunting (TIPSS) or open shunt surgeries. AIM: The aim of this study is to evaluate the management of BCS in children with balloon venoplasty as primary modality of treatment. MATERIALS AND METHODS: This is a retrospective study which includes children diagnosed with BCS managed by balloon venoplasty by a single surgeon at a single institute. Once confirmed, the child was posted for balloon venoplasty and liver biopsy. When venoplasty was successful, child was subsequently heparinized and dose titrated. Routine follow up was mandated and dose adjustments were continued during follow up. In case of unsuccessful venoplasty, depending on the liver biopsy report, shunt procedure or liver transplantation is offered to patients. An algorithm was then designed for management of BCS in children. RESULTS: A total of 35 children who underwent evaluation of symptoms associated with Budd-Chiari syndrome were included in the study. Of all the children, 14 are alive and symptom free, 9 are deceased and 12 lost to follow up. Hepatic vein was the most common site of obstruction (85%), followed by both IVC and HV (15%). Overall, in 35 children, 26 had a successful balloon venoplasty, in 3 venoplasty was not done (2 spontaneous resolution and 1 died awaiting), in 6 it was unsuccessful (3 technical failures: 1 underwent TIPSS and 2 lost to follow up, 3 clinical failures: portocaval shunt for failed venoplasty). CONCLUSION: Budd-Chiari syndrome is a rare condition affecting children. Balloon venoplasty as a primary modality of treatment for BCS is a promising option for management in children. Early and aggressive use of radiological intervention can help achieve recanalization in children. TYPE OF STUDY: Clinical research paper. LEVEL OF EVIDENCE: Level IV.

Rare Association of Isolated Microphthalmia with Anorectal Malformation.

Anorectal malformations (ARMs) have coexisting congenital anomalies. These can affect the overall prognosis. Anomalous craniofacial associations are less common. Recently, we managed two patients of ARM associated with unilateral microphthalmia, without any other major systemic anomalies. This was found to be a rare association on extensive literature search.

Role of Chemoports in Children with Hematological/Solid Tumor Malignancies - Technical Implications and Complications: An Institutional Experience.

AIM: The aim of this retrospective analysis was to identify the variously related complications and to study preventive and therapeutic measures for these complications. MATERIALS AND METHODS: A total of 72 catheters were inserted in 69 patients (mean follow-up of 1140 days) from December 2002 to May 2017. Sixty-four children were diagnosed to have hematological malignancies, and five children had solid tumors. The youngest child was 2 months of age, and the oldest was 15 years, 5 months. Records were analyzed retrospectively for the age, indication, route of insertion, and postoperative complications. A protocol-based insertion and postinsertion handling by trained nursing staff/doctors were instituted, including a periodic training program for those concerned. RESULTS: Chemoport-related complications were infection in 3 (4.16%), necessitating port removal in one patient. The rest were managed by antibiotic-lock therapy. The other problems were catheter tip occlusion in 1 (1.38%) and extravasation in two patients (2.77%) leading to a sterile collection around the port chamber. An unsightly scar in 4 (5.55%) and granuloma formation at scar site in 1 (1.38%) patient were noted. CONCLUSION: Totally implantable chemoports are preferred in children with solid and hematological malignancies because of decreased pain, the rate of infection, and ability to maintain patency for the long term. Despite significant advantages over other types of central venous access, chemoports have their own complications. It was also noted that the rate of complications could be minimized by periodic training of all the personnel concerned and following protocol-based handling of ports.

Pediatric Budd-Chiari Syndrome: A Case Series.

OBJECTIVE: To study the diagnostic methods and treatment outcomes in children with Budd- Chiari syndrome. METHODS: Case records of 25 patients with Budd-Chiari syndrome were evaluated retrospectively. These patients were investigated with imaging techniques and underwent balloon angioplasty or surgical management. RESULTS: 21 patients underwent balloon angioplasty, of which 17 had good medium- to long-term results, while only one out of four patients who underwent a portocaval shunt survived. CONCLUSIONS: The balloon angioplasty has satisfactory outcome in the treatment of acute Budd-Chiari syndrome. In failed cases, the surgical therapy may be attempted, but the outcomes do not appear rewarding.

Role of ano rectal myomectomy in children with chronic refractory constipation.

BACKGROUND: Chronic refractory constipation (CRC) is an uncommon type of constipation. These children have persistent symptoms even after treatment with high dose laxatives, which may cause abdominal distension, vomiting, cramping and bloating. We conducted this study to assess the diagnostic and therapeutic role of anorectal myomectomy in children with CRC. MATERIALS AND METHODS: This study includes 107 patients who fit the criteria of CRC. Complete bowel preparation with polyethylene glycol solution, enemas and antibiotics was carried out before surgery in all patients. The anorectal myomectomy was carried out under general anaesthesia with the patient in the high lithotomy position. The patients were followed up from 6 months to 13 years postoperatively. The success of myomectomy was based on the daily and complete passage of stools without the need for medication or enemas. RESULTS: A total of 99 patients were included in the study, of which, 86 (86.86%) patients showed a good response to anorectal myomectomy. Of these, 32 patients had normal histology, 14 had histology suggestive of Hirschsprung's disease, 8 had hypoganglionosis, 10 had ultra-short segment Hirschsprung's disease and 22 had hypertrophic nerves with immature ganglia. Poor response was seen in 13 (13.13%) patients of whom 5 had normal histology, 5 had Hirschsprung's disease, 2 had hypoganglionosis and 1 had ultra-short segment Hirschsprung's disease. CONCLUSION: Anorectal myomectomy is an effective and technically simple procedure in selected patients with CRC for both diagnostic and therapeutic purposes.

Outcome of Biliary Atresia After Kasai's Portoenterostomy: A 15-year Experience.

BACKGROUND: To study the outcome of Biliary atresia after Kasai's portoenterostomy and clinical and biochemical factors affecting the outcome. METHODS: Medical record review of patients of biliary atresia operated from January 2000 to December 2014. The following data were collected and analyzed - sex, age at surgery, liver function tests, associated congenital anomalies, and clearance of jaundice (at 3 months). Final outcome was classified as alive, dead, or jaundice-free at last follow-up (minimum 1 year). RESULTS: 121 patients (61.9% males) were included; 32 (26.5%) were lost to follow-up at 1 year. At last follow-up, out of the 89, 42 (47.2%) were alive, 29 (32.6%) were jaundice-free, and 47 (52.8%) had died. The native liver survival rate at last follow up was 43.8%. 42 (47.2%) patients had complete clearance of jaundice at 3 months post-procedure. Jaundice-clearance rate was significantly high in patients alive (83.3% vs 16.7%, P<0.001)) as compared to those who died later. CONCLUSION: Jaundice clearance at 3 months post surgery is a good early indicator of long term success.

Rare case of transverse testicular ectopia - Case report and review of literature.

This case report has been reported in line with the SCARE criteria; Consensus-based surgical case report guidelines of International Journal of Surgery 2016. INTRODUCTION: Transverse Testicular Ectopia (TTE) is a rare condition which manifests with unilateral undescended testis and contralateral hernia. Till now around 100 cases have been described in the literature. The management depends on the anatomy of the vas, vessels and testis found on surgical exploration. An algorithm exists for its management and we propose a modified algorithm for management of TTE. CASE PRESENTATION: Five year male presented with complaints of unilateral undescended testis on the right and hernia on the left. Clinically the right testis was impalpable and left testis palpable in the left hemiscrotum and fluid hernia on the same side. DISCUSSION: Transverse testicular ectopia is a rare condition presenting with UDT and contralateral inguinal hernia. Although more than 100 cases have been described in the literature so far, those managed with a transeptal contralateral orchidopexy are two cases to the best of our knowledge. TTE was first described by Von Lenhossek in 1886. The mean age of presentation is around 4 years and most of the cases are diagnosed on surgical exploration. The management of TTE remains controversial even though an algorithm has been described for its management due to its varied presenting scenarios. CONCLUSION: TTE is a rare condition which requires high index of suspicion for diagnosis preoperatively. Whenever suspected we recommend an USG and/or MRI prior to diagnostic laparoscopy and proceed with orchidopexy. Diagnostic laparoscopy is both helpful in diagnosis and management. Transeptal contralateral orchidopexy gives good tension free fixation of testes in the scrotum.