Application of the Clamshell Thoracotomy in an Infant with a Large Mediastinal Tumor.

Historically it was recommended for emergency thoracotomy in thoracic trauma as the last resort when there was cardiopulmonary arrest. Nowadays, the only indications are lung transplantation and huge mediastinal masses. We report the use of a clamshell thoracotomy in a 7-month-old boy with a large anterior mediastinal mass extending into the bilateral thoracic cavities.

Head Scarf Pin in the Airway and Challenges in Rigid Bronchoscopy: A Case Series.

Accidental Foreign body aspiration, especially sharp metallic objects may lead to life threatening complications. A metallic object is identified readily on a Xray chest or CT chest and helps us in ascertaining its location. A straight pin with blunt head in the shape of pearl is used in wearing a head scarf also known as hijab to hold it in place. The head scarf pin (hijab pin) if accidentally aspirated into the airway may lead to grave complications. Hence timely intervention with suitable instrumentation is essential to prevent any lethal complications. We have reported four different cases of adolescent females who had accidentally aspirated hijab pin and challenges faced by us during removal.

Gluteal flap for omphalocele repair in a case of epigastric heteropagus: A novel approach for surgical management.

Epigastric heteropagus is an extremely rare congenital anomaly, in which there is asymmetrical conjoined twinning, with the parasitic twin attached to the epigastrium. A 2-day-old male, with epigastric heteropagus and omphalocele, was operated in our institution. After excision of the parasitic twin, omphalocele was covered with a gluteal skin flap available from the parasitic twin. Post-operative course was uneventful, except for infection along the edges of the skin flaps, which was managed conservatively. Only 44 cases of epigastric heteropagus twins have been reported previously in world literature. We present a novel surgical approach for the repair of the omphalocele in a case of epigastric heteropagus twins, probably the 45(th) case to be reported in the world literature.

Endoscopic Removal of a Recurrent Trichobezoar in an Adolescent: A Case Report.

Trichobezoar, a rare condition of intragastric hair accumulation is commonly associated with an underlying psychological condition. Removal of the bezoar either endoscopically or surgically (laparoscopy or laparotomy) with concurrent psychiatric assessment and treatment is the mode of treatment. We present a 10-year-old child with recurrent trichobezoar, who was managed surgically the first time, and subsequently endoscopic removal was done on recurrence of bezoar after 3 months. We also present the difficulties encountered during endoscopic bezoar removal.

Changing outcomes of stem cell transplantation in primary immunodeficiencies: Results from a tertiary-care charitable trust hospital in Mumbai.

Background: Hematopoietic stem cell transplantation in primary immunodeficiency disorders has come a long way since the first transplant in 1968. In India, pediatric stem cell transplantation long-term survival outcomes range from 62.5% to 75%, compared to 90% in high-income countries. Objective: We present single-center data of primary immunodeficiency transplants with immune-reconstitution evaluation after transplantation from a charitable trust hospital. Methods: Retrospective data of children transplanted for primary immunodeficiency disorders from March 2019 to March 2022 in a newly established transplant unit were collected. Data of pretransplant infections and comorbidities, surveillance for carbapenem-resistant Enterobacteriaceae, transplant characteristics, donor source, graft-versus-host disease, posttransplant infections, immune reconstitution, overall survival at 1 year, and immunodeficiency-free survival were collated. Results: Twenty-one patients underwent transplantation for primary immunodeficiency disorders. The median age at transplantation was 3 years and 5 months (range, 7 months to 17 years). Seventy-five percent of the cohort had organ involvement, with lung being the most common organ involved, followed by central nervous system. Fifty-two percent of children had peritransplant infections, with most of them recognized at the pretransplant assessment. Among 20 of 21 children with engraftment, 94% had complete chimerism initially, with 33% developing mixed chimerism over time. The median duration of immunosuppression was 3 months after transplantation, and only 1 child required systemic graft-versus-host disease treatment for more than a year. Immune-reconstitution showed good T-cell recovery at 3 months and naive T-cell production at 6 months. There was no regimen-related or sepsis-related mortality. Overall survival of the cohort was 95% at 1-year follow-up. Immunodeficiency-free survival was 86% after a median follow-up of 20 months. Conclusions: Immunodeficiency-free and graft-versus-host disease-free survival can be achieved in the majority of children with primary immunodeficiencies using enhanced supportive care and the latest transplantation algorithms.

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.

Subtotal Pancreatectomy for Congenital Hyperinsulinism: Our Experience and Review of Literature.

Congenital hyperinsulinism (HI) is characterized by profound hypoglycemia caused by inappropriate insulin secretion. HI is a heterogeneous disorder with at least two histologic lesions and several implicated genes. If HI is caused by a focal lesion, elective surgery is the treatment of choice because it leads to complete recovery without diabetes. On the contrary, near-total pancreatectomy though recommended for diffuse HI, long-term risks of endocrine and exocrine deficiencies are present. Between the years 2006-2011, three patients of HI were referred to and operated by a single surgeon. The preoperative diagnosis was confirmed by recurrent hypoglycemia, inappropriately high insulin levels, and augmented glucose requirements. The medical records of all three patients were reviewed to study their clinical features, medical and surgical treatment, and postoperative outcome (short- and long-term). There were three patients in this series (male/female ratio, 1:2), all presenting in the neonatal age. All patients failed medical treatment, and radiological imaging did not reveal any pancreatic lesion. All patients underwent subtotal (80 %) pancreatectomy. Two patients had diffuse type of HI and one focal HI. One patient had transient hyperglycemia for 3 months, which needed insulin supplementation. No patient has developed recurrent hypoglycemia, malabsorption syndrome, or any neurological sequelae until the last follow-up. Doing subtotal or near-total pancreatectomy in diffuse type of HI still remains controversial as one has a higher risk of recurrent hypoglycemia, while the other has a higher rate of insulin dependent diabetes mellitus. Subtotal (80 %) pancreatectomy may be considered as the primary modality of surgical intervention in diffuse type of HI, especially when the diagnostic facilities are limited or diagnosis is not known after preliminary investigations. This minimizes the chances of postoperative diabetes mellitus, and redo surgery can always be considered if there is recurrent hypoglycemia.

Tubular Colonic Duplication Presenting as Rectovestibular Fistula.

Complete colonic duplication is a very rare congenital anomaly that may have different presentations according to its location and size. Complete colonic duplication can occur in about 15% of all gastrointestinal duplications. Double termination of tubular colonic duplication in the perineum is even more uncommon. We present a case of a Y-shaped tubular colonic duplication which presented with a rectovestibular fistula and a normal anus. Radiological evaluation and initial exploration for sigmoidostomy revealed duplicated colons with a common vascular supply. Endorectal mucosal resection of theduplicated distal segment till the colostomy site with division of the septum of the proximal segment and colostomy closure proved curative without compromise of the continence mechanism. Tubular colonic duplication should always be ruled out when a diagnosis of perineal canal is considered in cases of vestibular fistula alongwith a normal anus.

Closure of large meningomyelocele wound defects with subcutaneous based pedicle flap with bilateral V-Y advancement: our experience and review of literature.

BACKGROUND: Meningomyelocele is a defect of the spinal cord, vertebrae, and the overlying skin and is the most common form of open spinal dysraphism. Rapid closure of the back defect in the early postnatal period is mandatory to reduce the frequency of infection-related complications of the central nervous system. Majority of the cases present with small defects, which can be closed primarily, with or without subcutaneous dissection. However, direct closure is not possible in 25% of the cases. Different types of local flaps (skin or muscle flaps) are widely used for covering the skin defects; and with varying results. PATIENTS AND METHODS: A prospective nonrandomized study was conducted in the department of pediatric surgery at a tertiary hospital, from September 2007 to October 2011. Overall 35 patients with large meningomyelocele defects that could not be closed primarily were included in the study. All patients were treated using subcutaneous tissue based pedicle flap with bilateral V-Y advancement. RESULTS: There were 27 neonates, 7 infants, and 1 child, with a male:female ratio of 1.19:1. There were 3 thoracolumbar, 14 lumbar, 14 lumbosacral, 3 sacral, and 1 multiple meningomyelocele defects with an average size of 8.5 cm (range 6.5-11 cm). Average intraoperative blood loss was 8 mL (range 6-10.5 mL). Average operative time which included flap reconstruction time, after closure of dura, was 38.6 min. Total seven patients had wound complications viz. fat necrosis (n = 2), flap necrosis (n = 2), hematoma (n = 1), cerebrospinal fluid leak followed by wound dehiscence (n = 1), wound infection which led to meningitis (n = 1). Average healing time for flap repair was 7.52 days. Overall 80% (n = 28) of the patients had good flap texture and contour with satisfactory cosmesis. CONCLUSION: Closure of large meningomyelocele wound defects with subcutaneous based pedicle flap with bilateral V-Y advancement is an effective technique. The main advantages of this technique are its simplicity, short operative time, good tolerance, early healing, and good cosmetic outcome with an excellent flap texture and contour match with minimal complications.

Solitary crossed renal ectopia with vesicoureteric reflux presenting with impaired renal function in a neonate.

Solitary crossed renal ectopia (SCRE) is an exceedingly rare anomaly of the urinary tract. So far, only 34 cases have been reported in the literature. It usually presents after infancy. Most of these cases are diagnosed incidentally while patients are undergoing evaluation for associated genitourinary, cardiovascular, hematological or vertebral abnormalities. We report the first case of SCRE presenting in neonatal age with impaired renal function and vesico-ureteric reflux.

Post meningocele repair urinary ascites in a neonate--a rare presentation.

A full-term female neonate with a large lumbosacral meningocele developed sudden abdominal distension and urinary retention after meningocele repair. An erect abdominal radiograph showed homogeneously opacified areas with a paucity of intestinal gas. Abdominal ultrasound revealed ascites with multiple internal echoes. With a provisional diagnosis of hollow viscus perforation, an emergency laparotomy was performed, which failed to reveal any leak from the urinary tract. However, fluid analysis confirmed the diagnosis of urinary ascites. The patient developed repeated episodes of urinary retention after catheter removal. She was started on clean intermittent catheterization (CIC) to ensure adequate bladder drainage. At 6 months of follow-up, ultrasound of the urinary tract, voiding cystourethrogram, and magnetic resonance imaging of the spine were all within normal limits. The CIC was discontinued, and the patient was observed. At present, she is voiding normally with a good stream. Failure to establish normal micturition after meningocele repair and CIC requirements suggested a neurogenic cause. Bladder rupture, secondary to spinal shock resulting in bladder atonia, could not be ruled out. The perforation (leading to urinary ascites) could be owing to bladder atonia and spinal shock rather than detrusor sphincter dyssynergia.

Single-stage Modified Duhamel procedure for Hirschsprung's disease : our experience.

INTRODUCTION: Primary single-stage pull-through for Hirschsprung's disease (HD) has been reported to give comparable surgical outcomes to staged operations with less morbidity. Herein, we present our experience with single-stage Modified Duhamel procedure for management of HD. PATIENTS AND METHODS: This was a review of 48 cases of HD who underwent single-stage Modified Duhamel procedure without a protective colostomy. RESULTS: The age at surgery ranged from 6 months to 10 years (median - 9 months, mean - 2.3 years). The average weight of the child was 7.2 kg (range, 4.9-22 kg). 38 (79.2%) patients had classical rectosigmoid HD, the rest being long segment HD (the proximal most level being the splenic flexure). The average duration of surgery was 175 minutes (range, 130-245 minutes). The average blood loss was 45 ml. The average hospital stay was 7.2 days (range: 6-10 days). The major postoperative complications (n=3) included postoperative adhesive intestinal obstruction, anastomotic leak and persistent constipation due to residual aganglionosis. Each required a re-exploration. Minor complications included surgical site infection (n=3) and post-operative enterocolitis (n=3), which were managed conservatively. Six patients had constipation for a limited period post-operatively. All patients have a satisfactory functional outcome and normal development and growth. CONCLUSIONS: For HD, we recommend that single-stage Modified Duhamel procedure should be the preferred approach in view of its low morbidity, satisfactory functional outcome and avoidance of stoma, multiple surgeries and economic benefit in view of decreased hospital stay.

Spontaneous bowel perforation in a neonate with anorectal malformation.

Gastrointestinal perforation in neonates with anorectal malformations is extremely uncommon. Delayed patient presentation is an important factor that demands special attention. We present a neonate with anorectal malformation and meconium peritonitis following spontaneous bowel perforation. A day 1 neonate was referred with features suggested of peritonitis. After adequate resuscitation and drainage under local anesthesia, patient was successfully operated for a sigmoid perforation and is now awaiting definitive surgery for the anorectal malformation.

Bleomycin: A worthy alternative.

CONTEXT: Lymphangiomas are developmental anomalies presenting mainly in the first two years of life. Surgical excision has been the mainstay of treatment; however a potentially disfiguring surgery along with presence of important structures in the vicinity and infiltration into surrounding structures makes the dissection difficult. AIMS: To study the safety and efficacy of Bleomycin as a sclerosing agent for lymphatic malformations in children. SETTINGS AND DESIGN: Prospective non comparative nonrandomized trial. MATERIALS AND METHODS: The study was carried out in 15 children between Day 5 of life to 12 years of age who presented between May2008 to May 2009. Bleomycin aqueous solution was injected intralesionally at a dose not exceeding 0.6 to 0.8 mg. /kg Body wt. The response to therapy was monitored clinically by measuring the length, breadth and area as well as by measuring the two largest perpendicular dimensions. The response was graded as excellent [total disappearance], good [>50% reduction] and poor [<50% decrease]. Those patients with diffuse lymphangiomas associated predominantly with hemangiomatous malformations, mediastinal, spinal or retroperitoneal extensions, visceral lymphangiomas, those with infections were excluded from the study. STATISTICAL ANALYSIS USED: None applicable. RESULTS: The reduction in the size of the mass usually took between two weeks to ten months. The average duration of follow up has been ten months. A significant response was seen in 8 out of the fifteen [53.33%] patients. 5 patients [33.33%] patients showed a good response to therapy and achieved >50% reduction in the size of their swellings. 2 patients [13.33%] showed a poor response to therapy and achieved less than 50% reduction in the size of the swelling. Complications of the therapy were few and far between. 2 patients developed fever after injection, one patients reported a transient increase in size of swelling, 2 patients have developed discoloration of the overlying skin and are currently being followed up for final outcome. None of the patients developed leucopenia or leukocytosis. All of the complications were managed with conservatively. Patients are on long term follow up to evaluate long term effects, if any.

Acute acalculous cholecystitis causing gall bladder perforation in children.

We report two cases of children who presented with acute abdomen due to gall bladder perforation and biliary peritonitis. Cholecystectomy with peritoneal lavage proved curative.

Outcome analysis of shunt surgery in hydrocephalus.

AIM: To study the clinical outcome of shunt surgeries in children suffering from hydrocephalus. METHODS: A prospective study of 50 children with hydrocephalus who underwent a ventriculo-peritoneal shunt insertion over a period of two years. These patients were then followed up for shunt related complications, shunt revisions and outcome. RESULTS: Twenty six of the 50 patients (52%) suffered from complications. The most common complications were shunt blockage (n=7) and shunt infection (n=6). These complications necessitated repeated shunt revisions. CONCLUSIONS: Infective complications of hydrocephalus are more likely to leave behind an adverse neurological outcome in the form of delayed milestones and mental retardation.