Disorders of sex development (DSD) are a sensitive and stressful condition for the family as well as the treating physician to deal with. The main issue in managing such cases is sex assignment. The decision is influenced by the cultural background, the sex of rearing, clinical features, the biochemical parameters including hormonal studies, the imaging reports, parental preference, fertility potential, and the assessment of mental make-up of the child when possible. In third world countries, there is diagnostic dilemma as most children with DSD present late and a detailed-lengthy work-up often delay their definitive treatment. In this article, the authors try to identify the important clinical features in children presenting with various types of DSD, which may aid in making a quick provisional clinical diagnosis and expediting the diagnostic work-up. The data have been gathered from 38 years of experience of the senior author while managing about 1200 cases of DSD in the pediatric intersex clinic at the tertiary care level institute.

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Objective: Empyema thoracis (ET) in children is a disease of significant morbidity and mortality. In the event of failure to resolute following intercostal chest tube drainage (ICD), thoracotomy decortication (TDC) remains the treatment of choice. We have reviewed the outcome of management of 96 cases of ET with the intent to establish the scope of ICD as primary form of the management. Materials and Methods: This is a retrospective study of 96 patients of ET who were managed in pediatric surgery department over a period of 5 years (April 2013 – March 2018). Ninety-six patients at a single center met inclusion criteria for having ET and underwent ICD. We excluded the cases where video-assisted thoracoscopic surgery was provided as primary treatment. The patients were categorized into complicated and uncomplicated groups. Those with pyopneumothorax, encysted empyema, multiloculated empyema, and bilateral ET were assigned as complicated group. There were two treatment groups: (I) those responded with ICD alone (II) those with ICD followed by TDC. Results: All 96 cases received ICD as primary management. There were 54 uncomplicated cases and 42 complicated cases. Out of 42 complicated cases, 26 patients recovered with ICD alone and 16 patients needed TDC. A total of 80 (83.33%) patients (54 uncomplicated ± 26 complicated) recovered with ICD alone. Significant complications were encountered in follow-up of patients who underwent delayed thoracotomy in the form of overriding of the ribs (n = 3) and postoperative air leak (n = 4). There was no mortality in our series. Conclusion: Early initiation of management of ET with intercostal tube drainage is simple, safe, effective even in complicated cases, and has less complications. Thoracotomy with decortication should be reserved for ICD failure cases.

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Aims and Objectives: Biliary atresia (BA) is a cholangiodestructive disease of the biliary tree. The first line of treatment is a Kasai portoenterostomy (PE) following which patients may develop cholangitis. We studied the effect of early cholangitis on the outcome of PE, namely jaundice clearance and early native liver survival (NLS). Methods: We reviewed the data of all children who developed cholangitis after PE from our prospectively maintained database of children with BA. The standardized treatment of all children in the database is described. The frequency and nature of these episodes were characterized, and the outcome of PE and NLS 1 year after PE was calculated. Results: Of 62 children who underwent PE in our institutions, 27 developed cholangitis. All episodes of cholangitis occurred within 14 months of PE. Of 25 children who cleared jaundice in the overall series, 19 had cholangitis. The incidence of cholangitis was significantly higher in children who cleared jaundice. Nine children who had cholangitis are alive with native livers for more than 1 year after PE. Twelve children had intractable cholangitis. Three of these children are alive with native liver 1 year after PE. Conclusion: In our series, cholangitis occurred in most children who cleared jaundice. Furthermore, the 1-year NLS of children who developed cholangitis was 33%.

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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 95^th 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.

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Aim: The aim is to study the demographic characteristics of neonates with congenital diaphragmatic hernia (CDH) and to analyze the prognostic factors determining mortality. Settings and Design: A retrospective cohort of CDH admitted at a tertiary level hospital during January 2005–December 2017. Materials and Methods: All cases of CDH admitted to our tertiary care neonatal intensive care unit before undergoing surgery were included in the study. Babies admitted after surgery and those with eventration of the diaphragm were excluded from the study. Results: Thirty cases (66.66% males, 53.33% inborn, and 63.3% >37 weeks) formed the study cohort. Mean birth weight was 2762 ± 579.67 g and mean gestational age was 37.12 ± 1.76 weeks. About 56.66% of the cases were detected antenatally. The survival rate was 60%. Predictors of poor survival included herniation of the liver or stomach (P < 0.05), low Apgar score at 5 min (<5), presence of moderate-to-severe persistent pulmonary hypertension of the newborn (PPHN) (P < 0.001), presence of shock (P < 0.003), low partial pressure of oxygen, high alveolar–arterial oxygen gradient, and high oxygenation index during first 24 h. Conclusions: Majority of the neonates with CDH at our center were >37 weeks and survival was 60%. The predictors of adverse outcome were low Apgar score, presence of moderate-to-severe PPHN, need for higher ventilatory settings, and shock. Antenatal detection of diaphragmatic hernia did not impact survival rates.

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Context: An aesthetically acceptable umbilicus is an important component of the body and absent or dysmorphia may lead to psychological discomfort. Therefore reconstruction of neoumbilicus attains importance in abdominal surgical planning. This innovative surgical creation of umbilicus was planned during the initial surgery of umbilical defects to achieve these goals with minimal scarring. Materials and Methods: Our technique was applied to all 26 cases: primary omphalocele repair (n = 5), abdominal wall reconstructions after conservative management of large omphaloceles (n = 17), large umbilical hernias (n = 3), and one case of patent vitelointestinal duct with redundant skin. All patients were followed up yearly for the aesthetic appearance and clinical photographs were recorded. Results: Technique applied to all 26 patients had excellent cosmesis and long term follow up was very encouraging. Conclusions: All patients had successful abdominal wall closure. Most of the patients had minimal scar of the large hernia repair and the umbilicus was normal looking and well accepted cosmetically.

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Aim: The primary treatment for the subset of infantile hemangiomas (IHs) which develops complication is pharmacological intervention, and propranolol has become a popular choice. Here, we evaluated the efficacy and safety of propranolol in a clinical cohort of IHs and analyzed clinical characteristics associated with a good outcome. Materials and Methods: We retrospectively reviewed a total of 52 IHs patients, between ages 1 and 48 months (median age: 7.5 months), who were treated with oral propranolol, with dose ranging from 2 to 3 mg/kg/day. Efficacy was evaluated using mean percentage reduction, visual analog scale (VAS), and parental satisfaction levels at week 2 and months 1, 2, 6, and 12. The adverse effects were noted and responses after 6 months were graded. Statistical analyses of the outcome were also performed for the responses with regard to age at propranolol initiation, site of lesion, and mean duration of treatment. Results: A therapeutic response with at least 50% mean percentage reduction in size was noted in 84.6% at the end of 6 months. VAS score and parental satisfaction levels correlated well with mean percentage reduction (63.7 ± 15.6) at 6 months. Patients aged <6 months and those with cephalic lesions exhibited a greater therapeutic response rate with shorter overall mean duration of the treatment. Conclusions: Oral propranolol at 2–3 mg/kg/day dosing has shown to be effective and safe for IHs in pediatric age group. Intervention in the early proliferative phase, with especially, the cephalic lesions result in better resolution rates with shorter duration of overall treatment.

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Despite being the second most common malignant bone tumor, Ewing's sarcoma remains uncommon in younger children and seldom seen in neonates and infants. Extraskeletal locations are even rarer, hardly ever suspected, and often misdiagnosed, causing delays in management. The histologic similarities of Ewing's sarcoma to more common pediatric small-blue-round-cell tumors such as lymphoma and neuroblastoma necessitate immunohistochemistry and molecular genetics for clinching the diagnosis. We report a soft-tissue Ewing's sarcoma in a 4-month-old female infant masquerading as a benign neck mass clinically, radiologically, cytologically, and intraoperatively. We also reviewed literature for any existing guidelines on when to biopsy neck masses in the pediatric population.

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A 12-year-old male child, during evaluation of chronic constipation, was incidentally diagnosed to have a fronded bladder growth. Transurethral en bloc excision of the tumor was achieved using holmium laser. Histopathology confirmed it to be an inflammatory pseudotumor. This case is reported for its unusual presentation and management by holmium laser.

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Teratoma of the kidney is uncommon. We report a case of a young boy with a large, right-sided retroperitoneal cyst suspected as lymphangioma causing hydronephrosis. His renal pelvis was dilated, containing purulent fluid, and a nephrectomy was performed. Histopathology revealed a mature cystic teratoma of the renal pelvis.

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Thymic cyst is regarded as a rare anomaly, accounting for about 3% of all anterior mediastinal masses. It can be either congenital or acquired. Congenital thymic cysts are usually asymptomatic, with about 50% found incidentally in childhood or adolescence. We report a case of 1-month 23-day-old male baby who was antenatally diagnosed at 32 weeks' gestation. Spontaneous resolution did not take place in the antenatal or neonatal period; instead, there was an increase in size on follow-up imaging. The baby was successfully managed by surgical excision.

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Introduction: Laparoscopic splenectomy (LS) became the standard choice for splenectomy in children with benign hematological disease. There are few reports about pancreatic injury during LS. The purpose of this study is to spot on factors increasing the risk of pancreatic injury during LS in children. Patients and Methods: A total of 140 children had LS for benign causes. Children were categorized into A and B groups. LigaSure™ was used to control pedicle in Group A, while endoscopic staplers were used in Group B. Preoperative levels of amylase, lipase, and lactate dehydrogenase (LDH) were obtained. The mean of pancreatic enzymes and LDH values was calculated on the 3 postoperative successive days. Results: A total of 71 boys and 69 girls had LS. The mean splenic size was 13.50 cm in Group A and 12.51 cm in Group B. The mean operative time in Group A was 41.91 min and in Group B was 56.36 min. The mean level of amylase was 42.99 IU/ml in Group A and 75.70 IU/ml in Group B (P = 0.001). The mean level of lipase was 37 IU/ml in Group A and 76.66 IU/ml in Group B (P = 0.001). Conclusion: Pancreatic injury during LS is a rare complication usually presented on biochemical level. We believe that it is a hemostatic-dependent complication rather than splenic size or nature of disease.

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We present a case of a perianal and perianal spindle cell variant of embryonal rhabdomyosarcoma (RMS). A 3-month-old male child presented with a firm mass in the perianal region. The mass measured 5 cm × 3 cm × 2 cm was surgically removed. Biopsy was performed; it showed embryonal RMS. Immunohistochemical (IHC) stains were performed using vimentin, myogenin, spinal muscular atrophy, and muscle-specific actin, which all showed positive results. The histological examination and IHC stains were consistent with a spindle cell variant of embryonal RMS.

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We describe a posterior wall intratracheal embryonal rhabdomyosarcoma (RMS) arising in a 6-year-old tracheostomized child masquerading as reactive granulation tissue and review all reported cases of pediatric intratracheal RMS. The child underwent laser debulking of the tumor and postoperative radiation and chemotherapy with no evidence of recurrence at 2-year follow-up. A literature review revealed four previous cases of pediatric primary tracheal or intratracheal RMS, and remission was achieved in all but one case with surgery, chemotherapy, and radiation. Pathologic evaluation of tracheal mucosal granulation tissue may merit consideration, particularly in patients with increased risk factors.

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The treatment plan and the decision for surgery in a significant proportion of patients with pelvi-ureteric junction (PUJ) obstruction type of hydronephrosis are dependent on the findings of renal scintigraphy. We report a case of a 3.5-year-old girl with right-sided PUJ obstruction, wherein the tracer excretion into the cecum and ascending colon complicated the clinical picture thereby misleading the final diagnosis or treatment plan and blurring the distinction between hydronephrosis and hydroureteronephrosis. Additional investigations may be required in such cases to reach a conclusion. The authors considered reporting this case in view of the deep-rooted clinical implications toward making a correct diagnosis. Besides, the possible mechanisms to explain the presence of the tracer inside the bowel have been discussed.

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