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.

Efficacy of Core Needle Biopsy in the Diagnosis of Pediatric Extracranial Solid Malignancies: A 10-Year Study.

Introduction: Core needle biopsies of solid masses in children are a minimally invasive technique. It guides to a definitive diagnosis and facilitates management. Aims and Objectives: To determine the accuracy, sensitivity, and specificity of core needle biopsies in diagnosing pediatric solid masses. Materials and Methods: A retrospective analysis of 430 children, who underwent core needle biopsy for solid masses between January 2007 and December 2016 at CMC Vellore, was done. Results: Retroperitoneal and intra-abdominal masses constituted 66% of cases. Real-time image guidance was used in 44% of cases. An accurate diagnosis was obtained in 93.6% of cases, while results did not correlate with the final diagnosis in 3.4%. Three percent had inadequate or necrotic tissue. None of the children had postprocedure complications. Conclusion: Core needle biopsies serve as good diagnostic modality, with minimal risks, in making a conclusive diagnosis and deciding on the line of management.

Long-Term Outcomes and Prognostic Factors Affecting Survival after Pulmonary Metastasectomy in Solid Tumors of Childhood: A Single Center Experience.

Introduction: This study assessed the long-term survival and the prognostic variables affecting survival following pulmonary metastasectomy (PM) secondary to childhood solid tumors. Materials and Methods: A retrospective analysis was done on 22 children who underwent PM for solid tumors between January 2007 and February 2020. The overall survival (OS) and event-free survival (EFS) at the end of the study period were noted. Tumor histology, completeness of resection, disease-free interval, laterality, location, number, and size of lung nodules were assessed for their significance in contributing to survival. Results: High-grade osteosarcoma (54.5%), followed by Wilms' tumor (18.2%), was the most common histological types. Unilateral nodules (59.1%) situated in a peripheral, sub-pleural location (91%) were the most common presentation. Pleural extension was noted in 12 (54.5%) patients. Synchronous pulmonary metastases were noted in 12 (54.5%) patients. Two developed metastases while undergoing chemotherapy and eight after the completion of therapy. The EFS and OS were both 31.8% at a median follow-up of 15.5 months (range 3-129 months). The median time required for an event to occur was 4 months (95% confidence interval [CI]: 1.4, 6.6 months) and median post-PM survival interval was 17 months (95% CI: 6.6, 27.4 months). Significant association was noted between preoperative tumor response to chemotherapy (P = 0.002) and survival. Conclusion: PM can improve survival in a select group of children with metastatic solid tumors. Favorable tumor response to chemotherapy was found to be a significant prognostic factors influencing survival.

Papillary Thyroid Carcinoma in Children: Clinicopathological Profile and Outcomes of Management.

BACKGROUND: We aim to analyze the clinicopathological profile and outcomes of management for children with papillary thyroid carcinoma (PTC). METHODS: Relevant clinical data of children ≤ 18 years of age managed for PTC between January 2006 and July 2018 as well as details of their follow-up till December 2019 were retrospectively collected and analyzed. RESULTS: There were 82 children with PTC that were managed during the study period. At presentation, 39 (47.6%) had cervical lymphadenopathy, while 9 (11%) had systemic metastasis. Majority of patients 39 (47.6%) underwent total thyroidectomy with a selective neck dissection, while total thyroidectomy alone was performed in 26 (31.7%). Following surgery, hypocalcemia was seen in 39 (47.6%): 28 (34.1%) were temporary, while 11 (13.4%) were permanent. Twenty-eight (34%) developed persistent disease after surgery and 131I therapy. Significant risk factors for persistence and metastatic disease were metastatic cervical lymph node at presentation (p = 0.002) and tumor size (p = 0.014), respectively. The mean duration of follow-up was 60.3 (range 12-150) months with a mean overall disease-free survival of 60 months (95% CI 57.11, 77.95). CONCLUSION: Children with papillary thyroid cancers present with aggressive disease, 47.6% with cervical nodal metastasis and 11% with distant metastasis in this cohort. The rate of post-thyroidectomy hypocalcemia in this study is substantial, and efforts to reduce it are actively being pursued. The presence of metastatic cervical lymph node at presentation (p = 0.002) and tumor size (p = 0.014) were the only significant risk factors for persistent and metastatic disease, respectively, in this study.

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.

Testicular Volume in a Cohort of Prepubertal Indian Children.

CONTEXT: There are orchidometer-based testicular volume nomograms for Indian children; however, accurate and reliable values measured by ultrasound are lacking. AIMS: The aim of this study was to (1) measure the testicular volumes of boys from birth to 8 years and generate reference values and (2) to identify factors if any that may influence variation in testicular volumes. SETTINGS AND DESIGN: This was a prospective observational study conducted on 320 children in the Department of Pediatric Surgery, Christian Medical College, Vellore, India. SUBJECTS AND METHODS: A total of 320 boys without any genital abnormalities were studied. The testes were scanned using a linear transducer, and the length, width, and depth of each testis were recorded. Testicular volume was calculated using Lambert's equation - length × width × depth × 0.71. STATISTICAL ANALYSIS USED: Mean testicular volumes and standard deviation for every year of age were calculated. The centile values for testicular volume were computed using R software. RESULTS: Age-specific nomogram of each testis was created separately. Interobserver variability of the measurement was shown to be up to 0.3 ml. No difference was demonstrated in the testicular volumes between the right and left testis. No correlation was found between body weight and body mass index with testicular volume. From the data on differences in size between the two sides, a volume differential index of 27% corresponds to the 95th centile. CONCLUSIONS: Reference values have been created for testicular volumes in prepubertal Indian children that could be used to assess the effects of disease and surgical interventions in this age group.

Multiloculated Cystic Renal Tumors of Childhood: Has the Final Word Been Spoken.

BACKGROUND: Multicystic renal tumors which include cystic nephroma, cystic partially differentiated nephroblastoma (CPDN) and cystic Wilms tumor has been a diagnostic and therapeutic challenge. Histopathological examination has been the only reliable differentiating method. Management of these tumors is still riddled with controversy as a definitive preoperative differentiation between the three has not been possible. METHODS: A retrospective evaluation was performed of the treatment strategies employed with nine cases of multicystic renal tumors treated from 2005 to 2015. RESULTS: The median age at presentation was 12 months with all except one being boys. All except two children underwent primary surgery. The median follow-up was 50 months with six children having long-term survival. One child succumbed to the disease process, one died due to an unrelated cause and another was lost to follow-up. Although there was no ambiguity with cases of cystic nephroma (CN) and cystic Wilms tumor, three of the four cases of CPDN had problems. CONCLUSION: Primary surgery for multicystic renal tumors is safe and should be seriously considered as it prevents overtreatment in cases of CN and early stage CPDN. Further studies are needed to fully understand the biological behavior of CPDN.

Use of Pretherapy Core Biopsy in the Diagnosis of Pediatric Renal Tumors.

BACKGROUND: Pretreatment core biopsy of pediatric renal tumors has been advocated by United Kingdom Children's Cancer Study Group to circumvent the disadvantage of International Society of Paediatric Oncology protocol, where neoadjuvant chemotherapy initiated without histopathological confirmation can result in over- or under-treatment. AIM: This study aims (a) to assess if pretherapy core biopsy correlates with the nephrectomy biopsy; (b) to assess if neoadjuvant chemotherapy changes Wilms tumor (WT) histology, and (c) to assess the incidence of biopsy site recurrence. MATERIALS AND METHODS: Seventy-six children from 2005 to 2016 with renal tumors who underwent a pretherapy core biopsy were included in the study. The biopsy was performed through the posterior flank post-ultrasound marking of the renal mass, by administering intravenous anesthesia. RESULTS: Of the 62 children with WT, an accurate diagnosis was possible in 61. Accurate prediction of anaplasia was possible only in 25%. Reduction in blastemal elements was seen in 26 patients with 10 of them showing completely necrotic tumor. Eleven of the 14 children with non-WT were accurately diagnosed. Core biopsy corroborated with the nephrectomy biopsy in all but 4 patients. Two specimens were inadequate and two cases of congenital mesoblastic nephroma were inaccurately diagnosed, one as spindle cell neoplasm and the other as WT. Biopsy site recurrence was seen in 1 child. CONCLUSION: Pretreatment posterior flank core biopsy in the diagnosis of pediatric renal tumors is safe, simple, and cost-effective with minimal complications.

Clinical profile and management options of children with congenital esophageal stenosis: A single center experience.

AIM: The aim of the study is to review 7 patients with congenital esophageal stenosis treated in our institution from a diagnostic and therapeutic point of view. MATERIALS AND METHODS: This is a retrospective cohort study of 7 patients treated in Christian Medical College, Vellore from 2008 to 2014. The data were analyzed with regards to age at onset of symptoms, investigative findings, age at definitive treatment, pathology, modalities of treatment, and outcomes. RESULTS: Symptoms started within the 1(st) year of life in all children with a median age of 4 months. The time of delay in diagnosis ranged from 8 months to 81 months with a mean period of 37 months. About 6 patients had a lower esophageal stenosis and 1 patient had a mid-esophageal stenosis. About 4 of the 7 children underwent endoscopic balloon dilatation from elsewhere, with 2 of the above 4 undergoing a myotomy for a wrongly diagnosed achalasia. The number of dilatations ranged from 2 to 7 with a mean of 4 dilatations. Resection of the stenotic segment with end to end anastomosis was employed in 6 of the 7 patients, and a transverse colon interpositioning was done in 1 patient. An antireflux procedure was performed in one patient. Histopathological examination of the resected specimen revealed tracheobronchial remnant in 3 patients, fibromuscular thickening in 3 patients, and membranous web in 1 patient. Postoperatively, 2 of the 7 patients had asymptomatic gastroesophageal reflux and 1 patient had postoperative stricture requiring one session of endoscopic balloon dilatation. The mean follow-up period was 42 months (range 18-72 months). At the time of the last follow-up, all 7 patients were able to eat solid food, and none of the children were found to have symptoms suggestive of obstruction or gastroesophageal reflux. There was a statistically significant increase in the weight for age after the operation. CONCLUSION: Congenital esophageal stenosis is rare and often confused with other causes of esophageal obstruction. Although endoscopic balloon dilatation offers an effective temporary relief, we feel that definitive surgery is curative. Long-term results following definitive surgery have been good, especially with respect to symptoms and weight gain.

A rare case of bilateral cystic nephroma associated with embryonal rhabdomyosarcoma of the penile urethra.

Bilateral cystic nephroma is an extremely rare benign renal neoplasm. Here we present a case of bilateral cystic nephroma in a four month old boy who subsequently developed embryonal rhabdomyosarcoma of the urethra. Both tumors were successfully treated. To our knowledge this is the first reported case of this association which could be related to Dicer-1 mutation.

A rare case of kaposiform hemangioendothelioma presenting as intussusception in a 4-month-old child without Kasabach-Merrit syndrome: A case report.

We report the identification of Kaposiform hemangioendothelioma of the cecum presenting as colocolic intussusception in a 4-month-old boy without Kasabach-Merrit syndrome. To our knowledge this is the first reported case in the literature of such a presentation.

Assessment of Quality of Life and Functional Outcomes of Operated Cases of Hirschsprung Disease in a Developing Country.

PURPOSE: Children treated for Hirschsprung disease (HD) are adversely affected by fecal incontinence and soiling. This can be detrimental to their physical, psychosocial quality of life (QoL) and impacts the normal functioning of their family. QoL studies in HD are predominantly from developed countries. We measured general quality of life, impact on family and functional bowel status using validated questionnaires in HD children in a developing country. METHODS: Patients with HD, treated in a tertiary paediatric institution in India between 2010 and 2017, were identified. Patients and/or their proxy completed the Pediatric Quality of Life and Family Impact Module questionnaires. Functional outcomes were assessed using Rintala's score. RESULTS: A 86 children and their parents participated in the study. Majority had rectosigmoid disease (67.4%) and underwent Soave's endoanal pull through (74.4%). A 21% of patients had low Rintala score indicating poor functional bowel outcomes. Only 11% of children had poor QoL scores. Family functioning outcomes were also severely affected in the same subgroup of patients. There was statistically significant correlation between Rintala score and QoL scores (p-value<0.001). Disease severity, type of surgery, and duration of follow-up did not have a statistically significant impact on the QoL. CONCLUSION: QoL in children with HD was comparable to the general population. Bowel dysfunction affects a notable number of children and was the most significant determinant of poor QoL.

Working classification and the quest for an effective, reconstructive management strategy in Y duplication of male urethra.

INTRODUCTION: Y-duplication is a rare subtype of urethral duplication whose surgical correction has traditionally yielded less than satisfactory results. Herein we report 18 patients, 17 of whom have completed successful urethral reconstruction. MATERIALS AND METHODS: A retrospective analysis was done on 18 children who have undergone repair of Y-duplication urethra in two tertiary care institutes from 2013 to 2020. The various subtypes encountered were classified in order to develop a rationale for reconstruction based on the underlying pathology. The various modalities used for reconstruction and the outcomes of the repair were studied. All but one boy voided mainly via the posterior (ventral) channel opening in the ano-rectum or perineum. These boys had the urethral reconstruction incorporating the healthy proximal ventral urethra at its origin. Further reconstruction up to the glans tip depended on the degree of dorsal (orthotropic) urethral patency, which forms the basis of our classification into Types I (completely stenotic), II (penile urethra patent), III (penile and bulbar urethra patent) and IV (fully patent dorsal urethra). RESULTS: Reconstruction resulting in voiding via a glanular or coronal meatus was completed in 17 boys. One boy is awaiting further surgery while being dependent on Mitrofanoff CIC. While urethral continuity could be achieved using patent segments of native urethra alone into two boys (Types II and III), all other children needed a neo-urethral segment to replace (Type I) or supplement (Type II) the dorsal urethra. Neo urethra was constructed from a tubularized preputial island flap (n = 11), Monti tube constructed from ileum (n = 3) or sigmoid colon (n = 4). A perineal operative exposure alone sufficed in 9, while the rest required an additional trans pubic approach. The only boy with Type IV anomaly underwent excision of the ventral urethral limb. Three boys with initially elevated serum creatinine have normal levels after reconstruction. CONCLUSION: Successful reconstruction of boys with Y- duplication of the urethra entailed elucidation of anatomical variations, adequate operative exposure (trans pubic and/or perineal) and innovative utilisation of local and/or distant tissues (preputial island flap, ileal/sigmoid Monti tubes).

An approach to giant neonatal hydrocolpos with normally sited anus - Diagnosis and management including a novel one stage operation.

INTRODUCTION: Giant non cloacal neonatal hydrocolpos is a rare entity where early recognition of the underlying cause and drainage of the distended vagina is paramount. Conventionally, immediate management has been by abdominal tube vaginostomy or, in urogenital sinus by vaginal clean intermittent catheterisation (CIC). Though effective in providing temporary relief, the abdominal tube vaginostomy is replete with complications, making it imperative to search for alternatives. Here we describe our method of emergent and definitive management of these children, where definitive repair, depending on the aetiology, has been made possible in a single stage. MATERIAL AND METHODS: Seven girls who presented primarily or with history of giant non cloacal neonatal hydrocolpos at two tertiary care centres from January 2013 to March 2021 were studied retrospectively. None had imperforate anus or a bulging hymenal membrane. Four had high vaginal atresia (VA) and three had Urogenital sinus (UGS), one with high confluence. Four girls (2 VA, 2 UGS) presented to us as neonates and were managed by emergency drainage procedures other than tube vaginostomy: CIC in one (low UGS) and a novel abdominoperineal U flap vaginoplasty in three (2 VA, 1 high confluence UGS) which was also the definitive procedure. This procedure involved fashioning an inferiorly based inverted U flap from the posterior wall of distended vagina which is tubularised and flipped down by an abdomino-perineal procedure. The girl with a low UGS later underwent a perineal procedure. Three presented elsewhere as neonates (2 VA, 1 UGS) and had abdominal tube vaginostomy as the emergency drainage procedure. This was complicated by tube dislodgement and pyocolpos till successful reconstruction was performed by us. Successful reconstructions included the abdominoperineal U flap vaginoplasty in one (VA) and a perineal procedure in another (low UGS). One girl with high VA had an inflamed vagina after tube vaginostomy, precluding the U flap vaginoplasty, and underwent abdominal vaginal mobilisation and pull through complicated by partial vaginal necrosis with septic complications. RESULTS: Reconstruction resulted in a patent and a good calibre neo-vagina in all at a mean follow up of 48 months. CONCLUSION: Immediate management of giant non cloacal neonatal hydrocolpos may either be vaginal CIC or a surgical procedure. The abdomino-perineal U flap vaginoplasty is a one stage neonatal procedure that provides effective drainage and forms a definitive neo-vagina. An abdominal tube vaginostomy can be avoided except where neonatal surgical expertise is unavailable.

Urethro vaginal injuries associated with pelvic fracture - A spectrum of clinical presentation and management.

BACKGROUND: Blunt trauma to the paediatric pelvis is associated with urethral rupture, sometimes even without a bony fracture. While such rupture of the male urethra has received considerable mention, female urethral injury is both less common and has received less attention. OBJECTIVE: We describe 4 cases of urethro vaginal injury associated with pelvic fracture, its varied presentations and the modalities employed in its management. PATIENTS AND METHODS: A retrospective study was done to identify 4 patients who presented to our institution between 2007 and 2018. All 4 girls suffered a loss of a urethral segment ranging from the distal urethra (n = 2) to complete urethral loss (n = 2) and rupture of the bladder neck in one. All 4 girls also had associated vaginal injury resulting in varied clinical presentation including total urinary incontinence, urocolpos, vaginal voiding via an acquired hypospadias and urinary retention with late onset haematocolpos. All were managed initially with SPC and three of them underwent urethral substitution with Monti ileal tube in two and vestibular mucosal flap in one. The hypospadiac neo-meatus was continent and was left alone. The ruptured vagina was repaired by rectus muscle interposition, direct suturing, posterior vaginal U flap or colonic patch. An appendicular Mitrofanoff was added for safety in 2 girls. RESULTS: Follow up was done till June 2019 (range 1-12 years). All patients voided satisfactorily and were fully continent. Normal menstrual function was present in the 3 post pubertal children. CONCLUSION: A traumatic force capable of causing urethral injury may often result in associated vaginal injury and should not be overlooked. Thus urinary retention is not the only presentation of urethral injury in girls. Fistulation to the vagina may result in continent or incontinent vaginal voiding if the vagina has not been transected and urocolpos/haematocolpos in those with vaginal transection. Various modalities maybe employed to maintain continuity of the urethra and vagina including use of bowel and local flaps.

Unusual variant of pseudo prune belly syndrome.

Unilateral pseudo prune belly syndrome (PPBS) is a rare variant with only two other cases found in the main literature until. We present a 9-month old boy with left-sided lax abdominal wall, undescended testes and major vesicoureteric reflux involving only the left side. He underwent left orchidopexy and left end ureterostomy followed by left nephrectomy. Unilateral variant supports the theory of mesodermal arrest as a cause for prune belly syndrome. Treatment is individualised and prognosis is relatively better when compared with other variants of PPBS.

Management and outcome in dehisced exstrophy with a simplified bladder re-closure and further reconstruction.

INTRODUCTION: The traditionally accepted method of bladder re closure in dehisced exstrophy involves osteotomy assisted pubic bone approximation (PBA). Continent voluntary voiding is achieved in a small proportion of children sometimes after many operative procedures. We propose a simple yet reliable method of repair of the dehisced bladder using Rectus abdominis muscle flap (RAM) instead of PBA to bridge the inter-pubic gap, followed by concomitant or subsequent bladder augmentation (BA). AIM: The aim of this study is to assess the outcome of dehisced exstrophy, using a RAM flap assisted redo closure (without PBA) with concomitant or subsequent further reconstruction. MATERIALS AND METHODS: This is a retrospective analysis of children who presented with dehisced exstrophy after repair in other institutions and who have undergone redo repair using RAM flap in two tertiary care centers from 2001 to 2019. The outcome of the redo closure and subsequent or concomitant further reconstruction as regards dryness, stability of the upper tracts and resolution of vesico ureteric reflux (VUR) was studied. RESULTS: Fifty five children (34 boys) underwent redo exstrophy repair for dehisced exstrophy using the RAM flap. Epispadias repair was performed concomitantly in 31 boys. In 26 children (group1) of mean age 12 months further surgery was deferred while in 29 children (group 2) of mean age 69 months underwent concomitant BA. Nine group 1 children underwent BA subsequently. Ureteric reimplantation was done at the time of BA in 54 ureters, 40 into the bladder plate and rest into the bowel segment of BA. 22 ureters were not reimplanted. Bladder neck surgery including 18 bladder neck closure and Mitrofanoff port for Clean Intermittent catheterization (CIC) were done along with BA. The RAM assisted bladder closure was event free and none needed redo operation. 35/38 augmented children are dry on Mitrofanoff CIC and one unaugmented boy voids normally. The upper tracts remain stable on ultrasound and VUR has resolved in 67/76 ureters. At current follow up, after a mean period of 53 months eGFR was normal in all except 3 who had initially presented with severe hydro uretero nephrosis. CONCLUSION: We present a simple and reliable method of repair of dehisced exstrophy using RAM flap with the feasibility of concomitant BA. Dryness was achieved with stable upper tracts in 36/39 children, 27 of them with a single reconstructive attempt.

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.

Clinical outcomes of children with Wilms tumor treated on a SIOP WT 2001 protocol in a tertiary care hospital in south India.

INTRODUCTION: Wilms tumor is the most common pediatric renal malignancy. While developed countries have had excellent survival, it remains poorer by comparison in developing countries. The aim was to analyze the clinical outcome of children with Wilms tumor managed in a developing country from 2004 to 2014 by the SIOP WT 2001 protocol. METHODS: Fifty-nine children with Wilms tumor managed by a SIOP WT 2001 regimen from 2004 to 2014 were analyzed. RESULTS: The median age at presentation was 36 months, and 59% were boys. The average size of the tumor at presentation was 523 mL. Inferior vena cava thrombus was present in 11, distant metastases in 18, and bilateral tumors in six. Preoperative chemotherapy was given to all children after a diagnostic core needle biopsy. Preoperative chemotherapy reduced the tumor size to a mean of 208 mL and resolved venacaval thrombus in eight. Fifty-five children underwent definitive surgery while two children died during preoperative chemotherapy and two remained inoperable. All surviving children received adjuvant chemotherapy with 17 receiving radiotherapy as well. The overall survival (OS) was 80% and the event-free survival (EFS) was 73% after a mean follow up of 42 months after completion of therapy. DISCUSSION: The tumor volumes at presentation and the incidence of venous tumor thrombosis in our cohort were much higher than those reported from developed countries. The incidence of metastatic disease at diagnosis (30.5%) was significantly higher than the 10-12% reported in Western data, but similar to that reported from various developing countries (14.1-31%). The OS in our cohort was 80% and the EFS was 73% with there being no events after 28 months. Although the survival rate for localized disease is similar to that in developed countries, the OS for metastatic disease was significantly less (50% vs. 75%). We also found that using an upfront posterior flank core biopsy was safe and beneficial for differentiating Wilms tumor from other pediatric renal tumors that are less chemosensitive. CONCLUSION: In a resource-restricted environment such as ours, the SIOP WT 2001 protocol has been found to show excellent results.