Anaplastic Rhabdomyosarcoma of Upper Lip in a 3-Year-Old Girl.

Rhabdomyosarcoma is an aggressive malignant striated muscle neoplasm commonly seen in children involving orbit, paranasal sinuses, cheek, tongue, and rarely upper lip. The anaplastic subtype is further rare and associated with poor prognosis. Herein, we report a 3-year-old female with this uncommon variant at an uncommon site.

Newer Insights into Prostatic Utricle in Proximal Hypospadias.

Introduction: Prostatic utricles (PUs) are the remnants of Mullerian ducts found in the male posterior urethra. Enlarged symptomatic utricles are known to be associated with hypospadias. There is a dearth of literature defining an enlarged utricle and also its clinical significance. Aims: The aim of this study was to describe anatomical difference of PUs of cases with severe hypospadias and also to find their clinical significance if any. Materials and Methods: A prospective study was carried out, and all patients with proximal hypospadias were enrolled. Cystoscopy was performed in all cases. Symptomatology, size, and location of PU were recorded. PU of >0.5 cm was considered enlarged. Results: In the present series, a total of 70 cases were included over a period of 2 years. Enlarged utricle was found in 47/70 (67.14%) on cystoscopy. They were wide-mouthed and negotiated 9 Fr/11 Fr cystoscope with ease. Recurrent epididymo-orchitis, recurrent urinary tract infection, and obstructive features were the most common complications requiring intervention. Conclusions: PUs in boys with hypospadias are enlarged as well as wide-mouthed. Most of these remain asymptomatic, but few of them carry the potential of complications. Cystoscopy helps in direct visualization of utricular anatomy, so it should be a preferred investigating modality for investigating a PU.

Gastrointestinal mucormycosis in the pediatric age group: an evolving disease.

BACKGROUND: Mucormycosis is a devastating opportunistic fungal infection resulting in significant mortality, especially in pediatric patients with predisposing risk factors. MATERIALS & METHODS: Biopsies and surgical specimens reported and proven as Mucormycosis in children under 12 years of age were retrieved from the records for three years (January 2018 to January 2021). Complete data, predisposing factors, treatment, and clinical outcome were recorded. RESULTS: 15 cases were identified, ranging from 9 days to 5 years. The male-female ratio was 3:1; three children were preterm. Fourteen children were diagnosed with gastrointestinal Mucormycosis (14/15), and one had palatal and sinusoidal involvement. Abdominal pain with distention was the most typical complaint. On microscopy, biopsies and surgical specimens showed extensive liquefactive necrosis with broad aseptate fungal hyphae. An intraoperative diagnosis was rendered in two cases. All neonates underwent exploratory laparotomy with surgical debridement and were administered Liposomal Amphotericin B. However, only two neonates survived out of the fifteen cases, one with disease limited to the appendix and pouch colon. The others succumbed to the disease despite antifungal therapy and surgical debridement. Thus, the overall mortality in the current study was calculated to be 86%, with neonatal mortality of 75%. CONCLUSION: Gastrointestinal involvement is more common in neonates and infants with a male preponderance. The diagnosis relies on direct microscopy, histopathology, and fungal culture. Intraoperative tissue may be sent in all suspected cases for direct microscopic examination for rapid diagnosis and treatment.

Correlation of the Position of the Anal Dimple with the Caudal Termination of the Striated Muscle Complex in Patients with Anorectal Malformations.

Background: In children with anorectal malformations (ARM), the vertical fibres of the striated muscle complex (SMC) are believed to be located within the limits of the anal dimple (AD). Methods: Forty five cases of ARM underwent posterior sagittal anorectoplasty (PSARP), median age 8.5 months. During PSARP the anterior, posterior limits and midpoint of the AD and SMC were marked. The location of AD was correlated with SMC. Results: A 'well developed' AD and SMC was seen in 80 % and 86.7 % patients respectively. The mean width of the AD and SMC was more in females than in males (20.96 vs. 18.98 mm and 14.24mm vs. 13.45mm respectively). In 36 cases (80 %), across the spectrum of ARM, the SMC was posterior in relation to the AD. In 7 cases (15.5 %) it correlated in position with the AD and in 2 cases (4.44 %), it was anterior to the AD. Conclusions: In the majority of cases AD and SMC were 'well developed' and the location of the SMC does not correlate with that of the AD. This has significant practical value in the important step of optimizing the placement of the rectum through the center of the SMC during repair.

Catheter test: A reliable alternative to assess adequacy of bladder management in patients of PUV in developing countries.

INTRODUCTION: Following posterior urethral valve ablation, persisting bladder dysfunction can lead to lack of improvement or worsening of hydronephrosis and as well, to lack of improvement or even worsening renal function. On the other hand, managing bladder dysfunction offers the possibility of lessening both the degree of hydronephrosis and the level of serum creatinine. As we have no available urodynamics we feel impelled to find other means of diagnosing and managing abnormal bladder dynamics in these boys. We wanted to determine if a short course of continuous bladder drainage would be an effective means of lowering abnormal serum creatinine levels. Based on an assumption that most bladders following valve ablation, especially in those boys with a persistently elevated serum creatinine post valve ablation, have some form of dysfunction, we wanted to determine if a short course of continuous catheter drainage could improve these abnormal serum creatinine levels. METHODS: The study was conducted over a 3year period and included all children up to 3 years of age who had higher age-appropriate nadir serum creatinine, 3 months post valve ablation. Patients were followed up at 1 week, 2 month and 3-month post valve ablation. On third follow-up visit patients with an elevated serum creatinine level were subjected to continuous bladder drainage (catheter test) for 5 days and serum creatinine level was recorded at the end of 5th day. Enforced bladder management which included dose titration of anti-muscaranic drugs and clean intermittent catheterization (CIC) with or without nocturnal catheter drainage (NOC) was instituted for next 3 months. Serum creatinine was determined at the end of the 3 month period and compared with prior values. RESULTS: A total of 21 patients were included in the study. The mean age of the children at the time of valve ablation was 6.97 ± 4.38 months. There was a significant fall in serum creatinine from 0.64 ± 0.12 mg/dl pre 5 days of drainage to 0.48 ± 0.10 at the end of the 5 day period (p = 0.024) along with a significant rise in GFR (p = 0.001). Following 3 months of enforced bladder management, there was a very mild rise in creatinine to 0.51, a normal age adjusted value. DISCUSSION: The study demonstrates the importance of early diagnosis of bladder dysfunction in boys with an elevated serum creatinine levels post valve ablation. Elevated nadir serum creatinine 3 months post ablation seems to be a useful indicator of possible bladder dysfunction; lowering of serum creatinine following a 5 day trial of continuous bladder drainage confirms this diagnosis. However, the lack of availability of urodynamic testing leaves the specific type of bladder dysfunction undiagnosed. CONCLUSION: Lowering of serum creatinine following a 5-day trial of continuous bladder drainage can be used as a rough indicator of existing bladder dysfunction in boys with a history of PUV and should be used in countries without urodynamic equipment as an indicator for at least a 3 month trial of enforced bladder management.

Laparoscopic Surgery in Pediatric Upper Tract Urolithiasis: An Alternate Modality.

INTRODUCTION: Incidence of pediatric urolithiasis has increased over the last few decades. Procedures such as extracorporeal short wave lithotripsy, percutaneous nephrolithotripsy, and ureterorenoscopic lithotripsy are not widely available for pediatric age group in many developing countries. It is desirable that advantages of minimally invasive surgery be offered to selected cases with urolithiasis. MATERIALS AND METHODS: All patients with pediatric upper tract urolithiasis managed laparoscopically from January 2015 to April 2020 were retrospectively reviewed. RESULTS: A total of 38 patients were included. The mean age of the patients was 8 ± 2.85 years. Thirty-four patients (renal and upper ureteric) were managed through retroperitoneal approach, while those with lower ureteric calculi (n = 4) were approached transperitoneally. A total of eight patients required conversion to open technique. The stone clearance rate was 79% by laparoscopic approach alone. There were no procedure-related complications. CONCLUSION: Our study suggests that laparoscopic management for pediatric upper tract urolithiasis is a radiation-free, single-time curative treatment and is feasible in centers where facilities for other endoscopic procedures are unavailable.

Kaposiform haemangioendothelioma of duodenum in a neonate.

Kaposiform haemangioendothelioma, an endothelial borderline tumour, is typically seen in childhood involving extremities. It has been closely associated with a consumptive coagulopathy state, Kasabach-Merritt phenomenon (KMP). Extracutaneous involvement is uncommon. Intestinal involvement is quite uncommon and can masquerade as an acute abdomen. A 24-day-old neonate presented with bilious vomiting and fever for 5 days. Sections from the resected gangrenous duodenum contained a submucosal tumour composed of infiltrating nodules of slit-like or crescentic CD34-positive spindled-to-flattened endothelial-lined vascular spaces along with zones of fibrosis. No nuclear pleomorphism or necrosis identified. The findings were classic example of kaposiform haemangioendothelioma with an absence of any deranged coagulation profile. The index case raises interest given its congenital incidental presentation at an uncommon site, like duodenum, and absence of coexistent KMP.

Gastrointestinal Mucormycosis in a two-year-old child: A clinical and radiological enigma.

Mucormycosis is a rare, fatal angioinvasive infection occurring in immunocompromised individuals. Gastrointestinal mucormycosis is rare variety with high mortality rate. We present a case of GI mucormycosis in a 2-year-old male child who presented with abdominal mass with no underlying risk factors. The aim of this case report is to emphasize on its etiopathogenesis and keeping mucormycosis high in the differential diagnosis in a child presenting with abdominal mass.

Prediction of Gap Length by Plain Radiograph of Chest with Nasogastric Tube in the Upper Esophagus in Patients with Esophageal Atresia and Distal Tracheoesophageal Fistula.

AIM: This study was aimed at prediction of the gap length between the two esophageal ends in cases of esophageal atresia and distal tracheoesophageal fistula (EA-TEF) by preoperative radiography with a nasogastric tube in the upper esophagus and its correlation with intraoperatively measured gap length. MATERIALS AND METHODS: All consecutive cases of EA-TEF were prospectively included in this study. Plain radiographs were taken with an 8 Fr nasogastric tube inserted in the upper esophageal pouch till its arrest. The patients were grouped into T1-T2; T2-T3; T3-T4; and T4 groups depending on the thoracic vertebral level of arrest of the NG tube on the radiograph. Intraoperative gap between the two esophageal ends was measured with Vernier caliper, and the patients were grouped into A, B, and C groups based on gap length (gap length >2.1 cm; >1-≤2 cm; and ≤1 cm). The operative gap groups were compared with the radiography groups. RESULTS: A total number of 118 cases were included over a period of 3 years. The arrest of nasogastric tube at T1-T2 and T2-T3 vertebral level corresponded to gap length Group A in 39/41 (95.12%) * patients. In gap length Group B, the arrest of tube at T2-T3 and T3-T4 vertebral level was seen in 44/44 (100%)* patients, in gap length Group C, the arrest of tube was noted at T3-T4 and T4 vertebral level in 31/33 (93.93%)* patients (*P < 0.001). CONCLUSION: Prediction of gap length by vertebral level of arrest of the nasogastric tube in the upper pouch in a preoperative chest X-ray correlated well with intra operatively measured gap length in cases of EA-TEF.

Radiologic Evaluation of Uterovaginal Abnormalities in Girls with Congenital Pouch Colon.

BACKGROUND/PURPOSE: The radiologic investigations of 25 girls with congenital pouch colon (CPC), managed over 17 years, were retrospectively reviewed. In 13 girls who form the study group, the investigations provided information about the anomalous uterovaginal (UV) anatomy and these findings were studied. MATERIALS AND METHODS: Age at presentation was 2 months to 10 years. The subtypes of CPC were Type I (n = 2), Type II (n = 9), and not recorded (n = 2). All patients had a double vagina and a unicornuate uterus on each side in the pelvis. The radiologic studies, performed at varying periods after surgery, included an intravenous urogram (IVU) (n = 4), micturating cystourethrogram (MCU) (n = 3), distal ileostogram/colostogram (n = 6), and magnetic resonance imaging (MRI) (n = 7). RESULTS: IVU and MCU showed retrograde filling of the vaginas with contrast during micturition with a small-capacity urinary bladder and a relatively open bladder neck. The two vaginas were quite apart, but symmetrical in appearance and position. A distal dye study showed filling of the colonic pouch, its terminal fistula, and the two vaginas in six patients. Opacification of the bladder was seen in 3/6 girls, including one girl with left-sided Grade IV vesicoureteral reflux. MRI (n = 7) showed a monocornuate uterus on each side in the pelvis. The upper vaginas on each side were apart, being widely apart (n = 5) and somewhat closer (n = 2). The lower vaginas were closer with an intervaginal septum. Other findings were a widely open bladder neck and urethra in two girls with urinary incontinence and visualization of the terminal fistula of the colonic pouch (n = 2). The lumbosacral spine was normal in all patients. CONCLUSIONS: In girls with CPC, retrograde reflux of contrast into the vaginas during a distal dye study or an IVU/MCU may provide useful details of the anatomy of the vaginas. An MRI scan is recommended as essential for comprehensive evaluation of the anomalous UV anatomy.

Congenital Pouch Colon.

Congenital pouch colon (CPC) is an unusual abnormality in which a pouch-like dilatation of a shortened colon is associated with an anorectal malformation. It is categorized into four subtypes (Types I-IV) based on the length of normal colon proximal to the colonic pouch. In males, the pouch usually terminates in a colovesical fistula just proximal to the bladder neck. In girls, the terminal fistula opens either into the urethra or in the vestibule, close to the urethral opening. Girls usually have a double vagina with a wide inter-vaginal bridge, a monocornuate uterus on each side, and urinary incontinence due to a widely open bladder neck. Associated major malformations are uncommon with CPC but sometimes, especially in reports from outside India, major abnormalities are present suggesting an early, severe error in embryogenesis. The more severe Types I/II CPC can usually be diagnosed by a large gas shadow or air-fluid level on X-Ray abdomen. For all subtypes of CPC, it is preferable to preserve a segment of the pouch by fashioning a narrow colonic tube for pull-through, the technique known as coloplasty or tubular colorraphy. Girls need additional management of the genitourinary abnormalities. Postoperatively, fecal continence levels are usually poor, especially with Types I/II CPC.

Anorectal Agenesis with Rectovaginal Fistula: A Rare/Regional Variant.

AIMS AND OBJECTIVES: The anatomical types of female anorectal malformation (ARM) are complex and include several rare and regional variants. The aim of this report is to highlight the diagnosis and management of cases of anorectal agenesis with rectovaginal fistula (RVF). MATERIALS AND METHODS: This is a retrospective chart review of cases of RVF who underwent definitive surgery between November 2000 and September 2016 in a single institution. RESULTS: Of the 244 female patients with anorectal anomalies treated in our institution over a 16-year period, there were 15 cases of RVF with anorectal agenesis giving it an incidence of 6%. All cases of RVF with anorectal agenesis presented with absence of anal opening, varying degree of abdominal distension, and history of passage of fecal matter per vaginum. The mean age of presentation was 3 months (newborn to 1 year). Sigmoid colostomy was done in 13 cases. Distal colostogram demonstrated high RVF which was further confirmed by examination under anesthesia (EUA) and endoscopy before definitive surgery. With the posterior sagittal approach, division of the RVF and anorectoplasty was possible in 14 cases; however, one patient required an abdominal mobilization of the colon for pull through due to a very short distal bowel segment. During a mean postoperative follow-up of 5 years, cosmetic and functional results were satisfactory. CONCLUSION: RVF with anorectal agenesis is a rare/regional variant of female ARMs. Clinical examination along with distal colostogram, EUA, and endoscopy clinches the diagnosis. Anorectal reconstruction by posterior sagittal anorectoplasty results in a satisfactory outcome.

Role of nasogastric tube in children undergoing elective distal bowel surgery.

BACKGROUND: Nasogastric tubes are being routinely used in children and adults undergoing elective abdominal surgery without much scientific evidence supporting their true usefulness. The aim of our study was to assess the role of nasogastric tube in children undergoing elective distal bowel surgery. MATERIALS AND METHODS: All pediatric patients undergoing elective distal bowel surgery were enrolled and randomized into two groups: those with nasogastric tube (NG group) or without nasogastric tube (NNG group). Outcome parameters such as resumption of bowel function, enteral feed tolerance, postoperative complications, hospital stay and patient with their parent satisfaction were compared between the groups. RESULTS: A total of 60 patients were included with equal distribution in the NG and NNG groups. Patient variables were comparable in both the groups. Patients in NNG group progressed to full oral feeds significantly earlier (57 ± 18 vs. 106.07 ± 18.35 h, p < 0.001) and had shorter duration of hospital stay (91.93 ± 26.03 vs. 114.67 ± 18.83 h, p < 0.001) as compared to the NG group. Significant number of patients with nasogastric tube reported sore throat (9 vs. 1 p = 0.03) and nausea (5 vs. 0 p = 0.010). There was no significant difference in return of bowel function (39.43 h ± 15.92 vs. 43.60 h ± 17.77, p = 0.171), hiccups, sleep disturbance, complications and nasogastric tube reinsertion rate between the two groups. CONCLUSION: Routine use of nasogastric tube after elective distal bowel surgery in children is not necessary.

Protocol-based management of 154 cases of pediatric liver abscess.

AIM: Although liver abscess (LA) is prevalent worldwide, especially in developing countries, there is a paucity of data regarding the protocol for management of LA in children. The aim of this study was to analyze the outcome of a protocol-based management of pediatric LA from a single institution. MATERIALS AND METHODS: This prospective observational study was conducted in a tertiary-care children's hospital and included all patients with LA managed over a 5-year period. Detailed clinical, laboratory, microbiological and imaging parameters were recorded, and the patients were treated according to a standardized management protocol. Small abscesses (<5 cm) and those with solid appearance on initial ultra-sonogram (US) were managed with antimicrobial therapy alone. Liquefied abscesses on imaging were additionally subjected to US guided percutaneous needle aspirations (PNA). Large abscesses pointing to the surface were treated by percutaneous drainage (PCD). Ruptured LA, abscess inaccessible to image-guided drainage and those not responding to other modes of treatment were subjected to open surgical drainage (OSD). Pleural collections were treated by aspirations or intercostal tube drainage. Outcome was analyzed in terms of duration of recovery and complications including mortality. RESULTS: Over the 5-year period, 154 pediatric LA patients underwent protocol-based management. The mean age of the patients was 6.76 years with the male:female being 1.26:1. Medical management alone, PNA, PCD and OSD were successful in 38 (24.6%), 76 (49.3%), 11 (7.14%) and 29 (18.8%) cases, respectively. Pleural collections developed in 43 (27.9%) patients out of which aspiration/drainage was needed in 24 (55.81%) cases. The mean duration of hospital stay was 20.67 ± 9.52 days. Ongoing sepsis and multi-organ failure lead to mortality in 6 (3.8%) cases; rest of the patients were doing well on follow-up. CONCLUSION: Ultra-sonography was useful for the initial diagnosis, monitoring the progress and management of LA in children. The outcome of a protocol-based management of LA in children was favorable.

Ruptured Liver Abscess in Neonates: Report of Two Cases.

Neonatal hepatic abscess is a rare disease seen mainly in preterm following umbilical catheterisation. Liver abscess in term neonates without any predisposing factor is still rarer and only few cases have been reported in the literature. Here we report two cases of liver abscess in term neonates presenting with abdominal mass due to rupture.

Pediatric esophageal substitution by gastric pull-up and gastric tube.

AIM: The aim of this study was to report the results of pediatric esophageal substitution by gastric pull-up (GPU) and gastric tube (GT) from a tertiary care pediatric center. MATERIALS AND METHODS: Retrospective analysis of the surgical techniques, results, complications, and final outcome of all pediatric patients who underwent esophageal substitution in a single institution was performed. RESULTS: Twenty-four esophageal substitutions were performed over 15-year period. The indications were pure esophageal atresia (EA)-19, EA with distal trachea-esophageal fistula-2, EA with proximal pouch fistula-1, and esophageal stricture in two patients. Mean age and weight at operation were 17 months and 9.5 kg, respectively. GPU was the most common procedure (19) followed by reverse GT (4) and gastric fundal tube (1). Posterior mediastinal and retrosternal routes were used in 17 and 7 cases, respectively. Major complications included three deaths in GPU cases resulting from postoperative tachyarrhythmias leading to cardiac arrest, cervical anastomotic leak-17, and anastomotic stricture in six cases. Perioperative tachyarrhythmias (10/19) and transient hypertension (2/19) were observed in GPU patients, and they were managed with beta blocker drugs. Postoperative ventilation in Intensive Care Unit was performed for all GPU, but none of the GT patients. Follow-up ranged from 6 months to 15 years that showed short-term feeding difficulties and no major growth-related problems. CONCLUSIONS: Perioperative tachyarrhythmias are common following GPU which mandates close intensive care monitoring with ventilation and judicious use of beta blocking drugs. Retrosternal GT with a staged neck anastomosis can be performed without postoperative ventilation.

Congenital pouch colon in girls: Genitourinary abnormalities and their management.

AIMS: To discuss the assessment and management of genitourinary (GU) tract abnormalities in 21 girls with Types I-III congenital pouch colon (CPC), studied over a period of 10 years. MATERIALS AND METHODS: Assessment included clinical and radiological assessment, examination under anesthesia (EUA), endoscopy of the lower GU tract, and evaluation of the surgical findings, operative procedures for the GU anomalies, and the results of management. RESULTS: Initial examination of the external genitalia showed a "clover-leaf" appearance (n = 6) and a single perineal opening (n = 6). In 9 patients, the openings of the urethra and double vagina were seen, of which a vestibular fistula was seen in 5 and an anterior perineal fistula in 1. Seventeen patients (81%) had urinary incontinence (UI) - partial in 10, and complete in 7. Renal function tests, X-ray sacrum, and abdominal US were normal in all patients. Micturating cystourethrogram (n = 9) showed a wide, bladder neck incompetence (BNI) with reduced bladder capacity in seven patients. EUA and endoscopy revealed a septate vagina in all patients and the urethral opening at a "high" position (n = 14) or at a relatively normal or "low" position (n = 7). In 8 patients, the intervaginal septum was thick and fleshy. Endoscopy showed a short, wide urethra, an open incompetent bladder neck, poorly developed trigone, and reduced bladder capacity in the patients with UI. The fistula from the colonic pouch opened in the proximal urethra (n = 4), high in the vestibule (n = 3), low in the vestibule (n = 8), perineum just posterior to the vestibule (n = 1), and undetermined (n = 5). Vaginoscopy (n = 8) showed normal cervices in all and cervical mucus in 4 patients. The subtypes of CPC were Type I CPC (n = 4), Type II CPC (n = 16), and Type III CPC (n = 1). All 21 patients had uterus didelphys. In four patients with UI, during tubular colorraphy, a segment of the colonic pouch was preserved for later bladder augmentation if required. A Young-Dees bladder-neck repair (BNR) was performed in four older girls for treatment of UI, with marked improvement in urinary continence in two girls, some improvement in one girl with complete urinary incontinence, and minimal improvement in one child. Division of the intervaginal septum was performed in three girls. CONCLUSIONS: GU abnormalities in girls with CPC need to be assessed and managed by a tailored protocol. UI is frequent, and its correction may require BNR. A segment of the colonic pouch can be preserved for possible future bladder augmentation. All girls have a septate vagina, often widely separated, and uterus didelphys. Gynecologic assessment and monitoring is required throughout adult life. Considering the wide opening of the vestibule, surgical management of the urogenital component by division of the intervaginal septum and if required, the vagino-fistula septum on each side results in a normal or a hypospadiac urethral opening and an adequate vaginal inlet.