Surgical and anaesthetic outcomes of paediatric splenectomies at a tertiary care institution in South India: a retrospective cohort.

PURPOSE: Splenectomies though well-established in the successful management of several resistant haemoglobinopathies, have not been studied in detail in the paediatric population to assess the outcomes. We conducted this review to primarily assess the surgical and anaesthetic outcomes of paediatric splenectomies and secondarily highlight factors predictive for a high-risk splenectomy. METHODS: A 5 year retrospective chart review was made, and patient follow-up was done jointly using the hospital electronic medical records and telephonic calls. A p value of < 0.05 was considered significant. RESULTS: Among the 69 splenectomised children, 61% were male and the overall mean age was 10.2 years. The cohort consisted of patients with thalassemia (46%), ITP (30%), haemolytic anemia (19%) and 1 child each with lymphoma, splenic cyst and Kassabach Meritt syndrome. Most (96%) were electively operated and 23% were performed laparoscopically. 61% received intravenous analgesia and the mean volume of fluid administered intra-operatively was 21 ml/kg. There was no documented OPSI, and there was one mortality. The mean follow-up period was 43 months and the overall survival rate was 98.5%. CONCLUSIONS: Splenectomy was associated with a promising overall outcome. A greater pre-operative transfusion requirement, a larger sized spleen and increased fluid administration intra-operatively, were associated with a worse outcome.

Laparoscopic Cystogastrostomy in Children with Pancreatic Pseudocysts: A Preliminary Experience of Eight Cases.

Introduction: Pancreatic pseudocysts (PPCs) and walled-off necrosis (WON) in children following acute pancreatitis are uncommon. The various modalities of therapy possible are conservative treatment, external drainage, endoscopic stenting, and internal surgical drainage procedures. There are no existing guidelines for the management of PPC in children. We evaluate the outcomes of laparoscopic cystogastrostomy (LCG) performed at our center. Materials and Methods: Eight children (median age: 10 years) underwent LCG for large PPC (median size: 12.5 cm). There were seven patients with PPC and one with WON. Seven underwent LCG by a transgastric approach and one underwent LCG by a retrogastric approach. Results: Seven out of the eight patients had complete resolution of symptoms and the PPC. The median follow-up period was 32 months (interquartile range: 9.5-55.5 months). There were no conversions. There was one patient with a WON who developed a recurrence. Conclusion: LCG is a safe and effective treatment option for large PPC/WON in children. A posterior retrogastric approach, when indicated, is a safe approach with a comparable outcome.

Surgical management of the acute paediatric scrotum: a three-year single centre experience.

BACKGROUND: Acute scrotal pain is a common emergency presentation in paediatric surgery. Torsion of the testicular appendage (TTA) is the most common cause for pain, with testicular torsion (TT) being the sinister pathology to exclude. Outcomes are time dependent, and a delayed scrotal exploration could result in testicular loss. METHODS: We performed a review on a large retrospective cohort of 449 surgical scrotal explorations at a large referral paediatric surgical centre over three years. RESULTS: Only about a quarter of children with testicular pain presented within 4 h. TT is commonly associated with nausea and an abnormal lie. Two children with a classical 'blue dot' sign were later found to have a testicular torsion. 19% of all children with a TTA were also seen to have Bell clapper anomaly (BCA). Recurrent testicular pain was associated with 84.7% (p < 0.001) of BCA. Intra-operative diagnosis of TTA correlated with histopathology in 84.6% (p=0.021). The sensitivity of intraoperative diagnosis was 90.9% with a specificity of 75.3%. CONCLUSION: Routine histopathology for a classic TTA may not be required especially in resource poor situations. All children presenting with recurrent episodes of testicular pain must be considered for surgical scrotal exploration. And in view of the incidence of BCA in this cohort, all scrotal explorations for acute scrotal pain should include an assessment for BCA.

Paediatric mucormycosis: tailoring surgical strategies to compliment antifungal chemotherapy. Different strokes for different folks.

Children manifesting soft-tissue fungal infections are uncommonly seen, more so the subgroup of invasive soft-tissue mucormycosis. Invasive fungal infections in various organs respond differently and are often complicated by an immune-compromised host. Repeated and aggressive clearance of disease till an infection-clear margin is obtained is the mainstay of surgical therapy. This is coupled with appropriate antifungal therapy and the management of any underlying medical conditions. From our experience, we propose a surgical algorithm for therapy of soft-tissue mucormycosis in children.

Transureteroureterostomy as an adjunctive antireflux procedure in children undergoing bladder augmentation for neurogenic bladder with major reflux.

INTRODUCTION: Transureteroureterostomy (TUU) provides urinary drainage of both renal systems to the bladder via a single ureter and is useful in selected situations of complex urological reconstructions. Herein we discuss its use, advantages and complications in children with neurogenic bladders and high-grade (4/5) reflux who have undergone augmentation cystoplasty. PATIENTS AND METHODS: Children with neurogenic bladder complicated by unilateral or bilateral high-grade vesicoureteric reflux (VUR), who underwent TUU along with augmentation cystoplasty (BA), were selected from two institutions. Eighteen children with an average age of 5 years at presentation were identified from a retrospective chart review. RESULTS: All had bilateral hydroureteronephrosis (HUN) of which there were 30 refluxing megaureters. While BA reduced bladder pressure, VUR was managed by refluxing to non-refluxing TUU in six cases with unilateral VUR and unilateral reimplantation with TUU to the reimplanted ureter in 12 cases of bilateral VUR, thus minimizing reimplantation to 12 of 30 ureters. The average time of follow-up was 51 months. Follow-up cystograms showed complete resolution of VUR in all. HUN improved/stabilized in all but one child. Serum creatinine remained normal in all but two cases. Other advantages of TUU include the use of the distal ureter as a catheterizable channel and ease of undiversion when the ureter has been diverted as a ureterostomy. An unusual complication of a TUU site stricture is discussed and the innovative technique of using a cecal patch to salvage the anastomosis is detailed. CONCLUSION: We conclude that a TUU is a safe and useful adjunctive procedure in children undergoing BA for neurogenic bladder with high-grade VUR, minimizing the need for ureteric reimplant in an unhealthy bladder.

An unusual presentation of gall bladder papillomatosis in association with metachromatic leukodystrophy.

A 5-year-old boy with metachromatic leukodystrophy, debilitated by spastic quadriparesis presented to us with massive ascites and respiratory distress. A subtotal cholecystectomy was performed on him from another centre for a gall bladder mass a year before he came to us. Imaging revealed a polypoidal frond-like mass arising from the gall bladder fossa which was supplied by a hypertrophied branch of the right hepatic artery. A decision was made to offer surgical resection preceded by embolisation of the feeding vessel. At surgery, a polypoidal frond-like mass in communication with the peritoneal cavity was seen arising from the remnant gall bladder bed with over 4 L of mucoid ascites. The mass along with the remnant gall bladder was removed. Biopsy revealed villous papilloma of the gall bladder. The child is well and asymptomatic at 5-month follow-up.

Idiopathic female pseudohermaphroditism with urethral duplication and female hypospadias.

Female hypospadias is a rare anomaly of the female urethra where it opens on the anterior vaginal wall anywhere between the introitus and the fornix. It is often associated with other genitourinary anomalies such as Cloacal malformation, female pseudohermaphroditism, nonneurogenic neurogenic bladder and urethral duplication. Idiopathic female pseudohermaphroditism is extremely rare, and most cases occur secondary to adrenogenital syndrome or maternal androgen exposure. We report a unique case of a 1-year and 4-month-old girl who presented with ambiguous genitalia and renal failure secondary to a non-neurogenic neurogenic bladder. On further evaluation, she was found to have urethral duplication with a hypospadiac female urethra. She initially underwent a vesicostomy and was further planned to undergo an appendicular Mitrofanoff at an older age. The mainstay of treatment in these cases includes relief of bladder outlet obstruction and recovery of renal function by adequate urinary drainage. Clitoral reduction, if cosmetically warranted, may be planned at puberty.