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.

Acceptability and outcomes of foreskin preservation for phimosis: An Indian perspective.

AIM: Understanding the Indian perspective, effectiveness, and acceptability of prepuce conservation in children with phimosis. Circumcision is performed and recommended far too often for nonphysiological phimosis. Will a less radical approach be acceptable in the subcontinent? METHOD: A two-arm study with the first arm as KAP (knowledge, attitude, and practice) study (n = 502). The second arm recruited deserving boys (n = 47) with symptomatic phimosis (see inclusion criteria). Betamethasone ointment was applied twice daily over the foreskin and gently massaged to stretch the phimotic band. Those who failed were offered lateral preputioplasty or circumcision. Religious beliefs influence attitude and practice and these were looked at with subgroup analysis. RESULTS: Most (85%) knew that circumcision was not the only treatment for phimosis. Though many parents (93%) knew the importance of foreskin cleanliness, few practiced it. The success of the steroid application was 81% (n = 38/47). Eight underwent preputioplasty. Minor discomfort as morbidity was noted. All parents were able to completely retract the foreskin of their children by the end of one month and were happy about the cosmetic result. CONCLUSIONS: KAP data on foreskin health is not available in the subcontinent, and this is a landmark study. Religious belief and community identity play a strong role in decisions related to foreskin preservation. Prepuce hygiene and knowledge about the usefulness of the foreskin is poor. The combination of medical and surgical methods of conserving the prepuce was effective. A high rate of success and the non-mutilating cosmetic result of prepuce preservation were acceptable to these parents.

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.

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.

Encouraging Results of Bowel and Bladder Management in Spina Bifida Aperta in South India with Quality of Life Scores in a Tertiary Care Institution in South India.

CONTEXT: It is often a challenge to counsel parents with children operated for spina bifida aperta in developing countries. Data regarding the efficacy of simple measures and preventive are scarce. AIMS: The aim of this study is to study such children for the incidence, prevalence of bowel bladder dysfunction, and the quality of life (QOL) in children who are involved with a multidisciplinary team in India. MATERIALS AND METHODS: All children with spina bifida occulta were followed - QOL questionnaires (PIN Q, modified Barthels activities of daily living , and the visual analog score [VAS]) were used. Interventions, such as clean intermittent catheterization (CIC), bowel enemas, and surgical procedures, were studied. RESULTS: A total of 68 children were assessed. Twenty-nine of these children over five were evaluated with QOL scores. The prevalence of incontinence of bowel and bladder was studied. The primary outcomes included the QOL scores, and the various surgical options help bowel and bladder management. Hydronephrosis in 17.95% of children <5 years and 65.5% of children over 5 years was noted. Nineteen children were socially independent for their bowel management. The Barthel index and PIN-Q showed a poor QOL in 27.6% and the VAS in 34.5% had the same. This translated to an acceptable QOL for over two-thirds of the children. CONCLUSIONS: Simple procedures and training for bowel management translate to a significant number of children being able to independently manage bowel care. About 30% of children develop hydronephrosis by 5 years; the decision to teach CIC must be made by then. We believe that positive counseling is given to the parents of children with spina bifida aperta as the children are capable of a reasonable QOL.

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.

Intestinal obstruction with a twist: a rare case of congenital portal vein aneurysm causing intestinal obstruction.

Bilious vomiting is often a presenting feature of upper intestinal obstruction in newborn. We present a case of intestinal obstruction in a newborn baby caused by abnormal vascular band arising from portal vein aneurysm in association with a midgut volvulus. Congenital anomalies of portovenous system are very rare, and it usually presents with portal hypertension in late infancy or childhood. In this particular child, the portal vein aneurysm contributed to intestinal obstruction due to both a failure of intestinal rotation and a mechanical band over the transverse colon.

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.