Rectourethral Fistula after Posterior Urethral Valves Ablation in an Infant: A Rare Complication.

Acquired rectourethral fistula (RUF) is a rare but major complication of posterior urethral valve ablation. We present a case of a 1-year-old boy managed in a staged manner by completion of posterior urethral valves ablation, colostomy, RUF division through anterior sagittal transanorectal approach, and stoma closure. The child is continent for urine and feces.

To Compare Short-term Surgical Outcome among Patients given Continuous Postoperative Antibiotic Prophylaxis and those given no Postoperative Antibiotics after Urethroplasty for Hypospadias: A Pilot Study.

Introduction: There is no well-accepted guideline or uniform practice for the usage of prophylactic antibiotics along with urethroplasty for hypospadias. As antibiotic resistance is growing, it is imperative to rationalize the usage of antibiotics when a patient is operated for hypospadias. Aims and Objectives: The study is aimed at finding if there is any difference in outcome if prophylactic antibiotics are given after urethroplasty for hypospadias. Study Design: Prospective randomized controlled study. Material and Methods: Forty patients between 6 months and 12 years of age were included in the pilot study. All patients received a single preoperative antibiotic and surgery as per the discretion of the operating surgeon. The participants were randomly assigned to Group A or B, Group A not receiving any prophylactic antibiotic after surgery, and Group B receiving prophylactic antibiotics till indwelling urethral catheter was in situ as per the present antibiotic policy of the institute. The patients were followed up clinically at catheter removal, 1 week after surgery and 1 month after surgery. Urine was analyzed at the start of surgery and after catheter removal. Data were tabulated and analyzed using nonparametric Fischer's exact test with help of Epi Info™ v5.5.8. Results: Twenty-four patients were included in Group A and 16 in Group B. The clinical profile is presented in the detailed manuscript. Although pus cells could be demonstrated on urine examination in 82.5% of the study participants, only 10% grew organisms on culture media. No difference could be demonstrated among the two groups statistically. On following up with the patients for 1 month, the groups were comparable with respect to surgical site infections, and surgical complications such as urethrocutaneous fistula/dehiscence and thin stream. [Table: see text]. Discussion: There was a wide variability among practicing pediatric urologists in prescribing antibiotic prophylaxis for patients undergoing urethroplasty for hypospadias. In the Urologic Surgery Antimicrobial Prophylaxis Policy by the American Urology Association, no recommendation has been made with respect to urethroplasty. Our results are in concurrence with the available English literature which has not shown any benefit of prophylactic antibiotics after hypospadias repair. Conclusions: Antibiotics may not have a definite role in the prevention of surgical complications and it may be imperative to avoid unnecessary antibiotics to reduce antibiotic resistance.

Female with two perineal openings: Do not forget rectovaginal fistula.

Anorectal malformations (ARM) in females are identified by abnormal location of the anal opening. Management is guided by clinical examination to find the number of perineal openings. Two openings in the perineum of a female may be seen in cases of imperforate anus without fistula, vaginal agenesis with vestibular fistula or imperforate anus with recto-vaginal fistula (RVF). We present a case series of ARM with RVF and discuss their diagnosis and management.

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.

Comparison of clinical outcome and anal manometry following laparoscopic-assisted anorectoplasty and posterior sagittal anorectoplasty in patients with high and intermediate anorectal malformation: A randomised controlled trial.

Introduction: High and intermediate types of anorectal malformations (ARMs) may be managed by either open posterior sagittal anorectoplasty (PSARP) or by laparoscopic-assisted anorectoplasty (LAARP). Most of the literature favours one approach over the other based on retrospective analysis. We performed this study with the aim to compare the short-term outcomes of both procedures. Materials and Methods: All paediatric patients with high and intermediate ARM were enrolled and randomised into two groups: open PSARP group and LAARP group. Outcome parameters such as faecal continence using Kelly's scoring system, anal manometric parameters and post-operative complications were compared between the groups. Results: A total of 16 patients were included with equal distribution in the open PSARP and LAARP group. Patient's variables were comparable in both the groups. Five patients developed immediate post-operative complications, three in the LAARP and two in the open group. The mean Kelly's score was 3.63 ± 1.6 versus 2.57 ± 1.9 (P = 0.132) for LAARP and PSARP group, respectively. The mean resting pressure was 34.71 ± 6.26 cm of H2O and 35 ± 6.16 cm of H2O (P = 0.384) in LAARP and open group, respectively. Rectoanal inhibitory reflex was demonstrated in 6/7 patients in LAARP group and 5/7 patients in open group. Conclusion: Faecal continence in patients undergoing either of the procedure is comparable. However, wound-related complications are lesser in LAARP procedure.

Pediatric Colorectal Carcinoma: A Series of Seven Cases.

Introduction: Colorectal carcinoma (CRC) accounts for <1% of all the neoplasms of children. Unfavorable histology and delayed diagnosis often result in poor outcome. This study aimed to investigate the clinical characteristic and prognosis of pediatric patients with CRC. Materials and Methods: A retrospective review of medical records of all patients diagnosed of CRC between 2015 and 2020 was performed. Data regarding gender, age, location, and histopathology were collected. Results: Seven children (5 males and 2 females) were included in the study. Age ranged between 8.6 and 11.8 years. Abdominal pain was the most common symptom. The duration between onset of symptoms to the diagnosis ranged from 5 days to 8 months. Right-sided tumors were more common than the left side. Histopathological examination showed mucinous adenocarcinoma in four/seven patients and signet cell variant was seen in 2/7. Immunohistochemistry was positive for cytokeratin and beta-catenin, however, all microsatellite instability markers were found negative. Despite surgical treatment and chemotherapy, four of our cases faced mortality and one patient was lost to follow-up. Conclusion: CRC can present in first decade of life and often misdiagnosed. Pediatric CRC has distinct tumor biology and shares a poor prognosis. Paucity of literature from the Indian subcontinent should urge further clinicopathological trials for establishing etiology and for refining treatment recommendations in these children.

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.

Atypical scrotal masses: Surgical surprise.

We present three unusual cases of atypical scrotal lesions in children. The first was a firm left scrotal mass with the testis indistinguishable. The second with apparently a large hydrocoele, which was a cyst, and the third with multiple nodular lesions, pushing the left testis into the right hemiscrotum. These turned out to be a pigmented neuroectodermal tumour, a lymphatic malformation and neurofibromas respectively. Paediatric surgeons should be aware of such surgical surprises.

Delayed referral for orchidopexy: Scrutinising the causes.

Orchidopexy for undescended testis is recommended at a younger age than heretofore; our study aimed to assess delays, and their causes, by retrospective analysis of data from a single tertiary care centre over one year (2015-2016). Almost 80% of children were brought after 1 year of age, mostly because of delayed referral by primary physicians (60%), or missed diagnosis by parents or primary physicians (20%). Misconception about the risk of surgery below 1 year was significant (15%). A timely referral is encouraged.

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.

Evaluation of Risk Factors Affecting Outcome in Outborn Surgical Neonates.

BACKGROUND: Mortality in surgical neonates contributes to neonatal mortality rates. The study was conceptualized to study clinical and nonclinical factors affecting mortality in surgical neonates so that timely intervention could result in improved survival of the neonates. MATERIALS AND METHODS: The study was initiated after approval from the institutional ethics committee and included 120 surgical neonates over a period of 18 months after obtaining consent from the parents/caregivers. Predesigned pro forma was used to record the details of antenatal care received, place of birth, travel history, maternal education and gestational age, and clinical condition at the time of admission. Values of biochemical tests such as serum electrolytes, serum creatinine, and arterial blood gasses were recorded. The need of inotrope support, blood or blood product transfusion, and postoperative ventilator support and intensive care unit (ICU) care was recorded. The results of the two groups, i.e., survivals and mortality, were compared. Outcome was recorded as mortality at 30 days or earlier. RESULTS: Irrespective of the surgical condition, the survival rate was significantly better in those babies who weighed more than 2.5 kg at the time of admission, had capillary refill time of <3 s, had serum ionized calcium levels more than 1 mmol/L, and did not require inotropes, blood or blood product transfusion, and postoperative ICU care and ventilator support. The place of birth, educational status of the mother, gestational age, and distance traveled for care had no statistically significant effect on survival. CONCLUSION: There is a statistically significant correlation between the survival of the babies who weighed more than 2.5 kg and are more physiologically preserved at the time of admission. Mortality rates can be decreased by timely interventions to reduce the need of inotropes, blood or blood products, and ICU care and ventilator support during their postoperative recovery.

Lipofibromatosis: A Rare Diagnosis on Fine Needle Aspiration Cytology.

Lipofibromatosis is a recently recognized slow growing rare pediatric tumor. Paucity of its cytological description in the literature leads to its pre operative misdiagnosis and further incomplete management. A twelve-month-old female presented with a rapidly progressive mass in the right thigh and buttock region. On examination, the mass was huge and involved the medial, posterior and lateral aspects of the thigh. The cytological smears showed mature adipocytes with few spindled out cells. FNA was reported as a lipoma, corroborating with the radiological presumptive diagnosis. However, histopathological and immunohistochemical features favoured a diagnosis of Lipofibromatosis. The cytological smears were reviewed and a cyto-histo correlation was established. The diagnosis of Lipofibromatosis rests upon classical cytological features in a clinically and radiologically suggestive picture. An early and accurate diagnosis if established can help the surgeon plan excision with wider margins as incomplete excision is associated with a high rate of recurrence.

Non-malignant fibroblastic/myofibroblastic tumors in pediatric age group: Clues and pitfalls to the cytological diagnosis.

INTRODUCTION: Fibroblastic/myofibroblastic tumors constitute 12% of all pediatric soft tissue tumors with the majority of them belonging to the benign and intermediate prognostic categories. They are often misdiagnosed owing to their variable clinical presentation and unusual microscopic features. The diagnosis, specially cytological diagnosis of benign and intermediate categories is difficult due to paucity of cellular component and increased amount of extracellular matrix as compared to malignant ones. We hereby discuss the Fine needle aspiration cytology (FNAC) findings of non-malignant fibroblastic/myofibroblastic lesion in the pediatric age group encountered at our institute. METHODS: All the benign and intermediate fibroblastic/myofibroblastic/fibroadipocytic lesions (age 0-12 years) diagnosed on FNAC over a period of 3½ years (Jan 2016- July 2019), with availability of corresponding histopathology were included in the study. RESULTS: A total of seven pediatric benign and intermediate fibroblastic/myofibroblastic lesions with histopathological confirmation were identified which included Infantile digital fibromatosis (IDF) (n = 2), Lipofibromatosis (n = 1), Fibrous hamartoma of infancy (FHI) (n = 1), Fibromatosis colli (FC) (n = 2) and myofibroma/myofibromatosis (n = 1). FNAC smears were mainly paucicellular with presence of benign spindle shaped cells in a collagenous stroma common to almost all the cases. A few additional findings such as degenerated skeletal muscle fibres, muscle giant cells and mature adipose tissue were also present in some cases. CONCLUSION: Fibroblastic/myofibroblastic tumors although uncommon, form an important category that must be considered in the differential diagnosis of pediatric soft tissue tumors. FNAC cytology features when assessed in a proper clinical setting (specially the age and site of presentation) are helpful in suggesting probable preoperative diagnosis in these lesions.

Intraoral Foregut Cystic Developmental Malformations: Three cases with a brief review of literature.

Foregut cystic developmental malformations (FCDM) are a type of rare cystic lesion. The occurrence of FCDM is exceedingly uncommon in the intraoral location. We report three cases of FCDM with intraoral location who presented at Chacha Nehru Bal Chikitsalaya, New Delhi, India, in 2016, 2017 and 2018 with symptoms of respiratory distress and feeding difficulties. Two patients were male and one was female with an age range of 29 days to eight years. The clinical differential diagnosis included mucocele, ranula, dermoid, lymphangioma, teratoma, thyroglossal duct cyst, etc. All patients were treated with simple surgical excision and diagnosed, based on histopathology, with FCDM. These should be considered as differential diagnosis of head and neck midline cystic mass lesions. This case report aimed to discuss differential diagnosis and appropriate terminology for these cystic masses as there is varied and ambiguous nomenclature.

Childhood Phimosis Secondary to Lichen Sclerosus: Is There a Spatial Pattern of Histopathological Changes?

INTRODUCTION: The accurate histopathological diagnosis of the phimotic prepuce is indispensable because early diagnosis, treatment, and close follow-up are crucial in genital dermatosis such as lichen sclerosus (LS). This study analyzes the histopathological spectrum of childhood phimosis with special emphasis on LS. We also highlight a peculiar pattern of histopathological evolution in LS, prepuce. MATERIAL AND METHODS: The histopathology slides of all the pediatric preputial circumcision specimens performed for the indication of pathological phimosis (n = 43) during the study period (2012-2017) were analyzed. Eight histopathological features viz. hyperkeratosis, hypergranulosis, epidermal atrophy, acanthosis, dermoepidermal cleft, upper dermal edema and homogenization, mid dermal lymphocytic band, and interface dermatitis were studied in each case, separately in inner preputial surface, tip, and outer preputial surface. On the basis of evolution of the disease and histopathological features, the lesions of LS were classified into early, established, and advanced. RESULT: LS was found in 32 cases, whereas 11 cases showed nonspecific inflammation and fibrosis. The upper dermal homogenization (n = 29), dermoepidermal cleft (n = 28), and mid dermal band (n = 27) were the commonest histopathological changes. The established and advanced changes were confined to the inner preputial surface (n = 31), and the outer preputial skin surface was unaffected in all the cases. A peculiar histopathological evolution pattern was seen with established or advanced lesions, early lesion, and normal histology on the inner preputial surface, mucocutaneous junction, and outer preputial skin, respectively. CONCLUSIONS: LS is a common cause of childhood phimosis. It shows a peculiar histopathological evolution that mandates the thorough analysis of inner mucosal surface.

Spigelian hernia in children: low versus classical.

PURPOSE: Pediatric spigelian hernias are very rare. They are often missed or misdiagnosed. A series of cases with spigelian hernia, presented to a tertiary care center are presented here with emphasis on different anatomy of spigelian hernias with cryptorchidism and those without associated cryptorchidism. MATERIALS AND METHODS: Over a period of seven years, nine cases of spigelian hernia presented to our tertiary care center. Male:female ratio was 3:1.There was a preponderance of right sided hernias. Three patients had associated cryptorchidism. One patient had associated lumbar hernia. All three patients with cryptorchidism had low spigelian hernia while others had classical spigelian hernia. CONCLUSION: There is a likelihood of anatomical variation in SH associated with UDT and those without UDT. Understanding this anatomy may help in correct scrotal placement of testis. TYPE OF STUDY: Prospective Observational. LEVEL OF EVIDENCE: 4.