Context: Despite advances in neonatal intensive care, surgical methods, and anesthesia, congenital diaphragmatic hernia (CDH) is still associated with significant mortality. Predicting which babies will have poorer outcomes is essential to identify the high-risk babies and to give targeted care and accurate prognosis to the parents, especially in a resource crunch set-up. Aims: The aim of this study is to evaluate the antenatal and postnatal prognostic factors in neonatal CDH that can be used to predict the outcome. Settings and Design: This was a prospective observational study in a tertiary care center. Subjects and Methods: Neonates presented with CDH within 28 days of life were included in the study. Bilateral disease, recurrent diseases, and babies operated outside were excluded from the study. The data were collected prospectively, and babies were followed until discharge or death. Statistical Analysis Used: Data were expressed in mean with standard deviation or median with range based on normality. All the data were analyzed using the SPSS software version 25. Results: Thirty babies with neonatal CDH were studied. There were three right-sided cases. The male-to-female ratio was 2.3:1, and 93% of babies were antenatally diagnosed. Seventeen out of the 30 babies underwent surgery. Nine (52.9%) underwent laparotomy, and 8 (47%) underwent thoracoscopic repair. Overall mortality was 53.3%, and operative mortality was 17.6%. Demographic characteristics were comparable between expired versus survived babies. The significant predictors of outcome identified were – Persistent pulmonary hypertension (PPHN), mesh repair, high-frequency oscillatory ventilation (HFOV), use of inotropes, 5-min APGAR, ventilator index (VI), and HCO3 levels. Conclusions: We conclude that the prognostic indicators associated with poor prognosis are low 5-min APGAR, high VI, low HCO3 levels in venous blood gas analysis, mesh repair, HFOV, inotropes usage, and PPHN. None of the antenatal factors studied showed any statistical significance. Further prospective studies with a larger sample size are recommended to confirm the findings.

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Introduction: Testis is essential for male sexual hormone production, fertility, and psychological well-being of a male. In the unfortunate event of testicular loss, placement of a testicular prosthesis perhaps will offer a sense of well-being, improved body image, and overall confidence in the growing child. Aims: The aim is to evaluate the feasibility and assessment of outcomes following concurrent placement of testicular prosthesis in children following orchiectomy. Materials and Methods: This is a cross-sectional study conducted by reviewing the reports of patients who underwent simultaneous insertion testicular prosthesis following orchiectomy for various indications between January 2014 and December 2020 at tertiary hospitals in Bengaluru. Children <18 years were included in this study. A transscrotal approach was preferred in cases where a transscrotal orchiectomy was done. Transinguinal approach was preferred in children undergoing prosthesis insertion as an isolated procedure. The size of the prosthesis was chosen based on the age of the child and the size of the scrotum. Outcomes were assessed on follow-up. Results: A total of 29 children underwent prosthesis insertion (25 unilateral and four bilateral). The mean standard deviation age was 5.58 (3.92) years. The indications for prosthesis insertion were cryptorchidism with atrophic testis (22), torsion (3), Leydig cell tumor (2), and severely virilized Congenital adrenal hyperplasia (CAH) (2). Of these, three children (9%) had complications (wound gaping in two and wound infection in one) that needed removal of the implant. The mean duration of follow-up was 49.23 months. All the parents reported a good outcome, and none of the children who underwent prosthesis placement needed a change during this follow-up. Conclusion: Concurrent placement of a testicular prosthesis is technically easy and a safe procedure, achieves satisfactory cosmetic appearance with minimal morbidity.

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Context: Gastrointestinal (GI) duplications are rare congenital malformations with diverse presentations. They usually present in the pediatric age, especially in the first 2 years of life. Aims: To present our experience with GI duplication (cysts) at a pediatric surgery tertiary care teaching institute. Settings and Design: It is a retrospective observational study undertaken in the department of pediatric surgery at our center between 2012 and 2022 for GI duplications. Materials and Methods: All children were analyzed for their age, sex, presentation, radiological evaluation, operative management, and outcomes. Results: Thirty-two patients were diagnosed with GI duplication. Slight male predominance was present in the series (M: F ≈ 4:3). Fifteen (46.88%) patients presented in the neonatal age group; 26 (81.25%) patients were under 2 years. In the majority of cases (n = 23, 71.88%), the presentation was acute onset. Double duplication cysts on opposite sides of the diaphragm were present in one case. The most common location was ileum (n = 17), followed by gallbladder (n = 6), appendix (n = 3), gastric (n = 1), jejunum (n = 1), esophagus (n = 1), ileocecal junction (n = 1), duodenum (n = 1), sigmoid (n = 1), and anal canal (n = 1). Multiple associations (malformations/surgical pathologies) were present. Intussusception (n = 6) was the most common, followed by intestinal atresia (n = 5), anorectal malformation (n = 3), abdominal wall defect (n = 3), hemorrhagic cyst (n = 1), Meckel's diverticulum (n = 1), and sacrococcygeal teratoma (n = 1). Four cases were associated with intestinal volvulus, three cases with intestinal adhesions, and two with intestinal perforation. Favorable outcomes were present in 75% of cases. Conclusion: GI duplications have varied presentations depending on site, size, type, local mass effect, mucosal pattern, and associated complications. The importance of clinical suspicion and radiology cannot be underrated. Early diagnosis is required to prevent postoperative complications. Management is individualized as per the type of duplication anomaly and its relation with the involved GI tract.

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46, XX testicular differences of sex development (DSD) is a rare cause of DSD presenting as a phenotypical male with chromosomal sex of 46, XX. Sex-determining region of the Y chromosome (SRY)-positive 46, XX DSDs have a well-characterized pathogenetic mechanism, whereas in SRY-negative 46, XX DSDs, the pathogenesis is not clearly delineated. Herein, we present a case of a 3½-year-old child who presented with ambiguous genitalia and bilateral palpable gonads. On the basis of a karyotype and fluorescent in situ hybridization, we arrived at a diagnosis of SRY-negative 46, XX testicular DSD. Basal serum estradiol and human menopausal gonadotrophin stimulated estradiol levels and inhibin A blood levels were against the presence of any ovarian tissue. Imaging of the gonads showed bilateral normal-looking testis. A clinical exome sequencing revealed a heterozygous missense variant NR5A1:c275G>A (p. Arg92gln) located at exon 4 in the affected child. Protein structure analysis was further performed, and the variant was found to be highly conserved. Sanger's sequencing showed that the mother was heterozygous for the variant detected in the child. This case highlights the rarity of SRY-negative 46, XX testicular DSD with a unique variant. Largely under characterized, this group of DSDs needs to be reported and analyzed to add to the spectrum of presentation and genetic characteristics. Our case is expected to add to the database, knowledge, and approach to cases of 46, XX testicular DSD.

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Long tubular duplication of the small intestine is one of the most difficult surgical challenges. The presence of heterotopic gastric mucosa necessitates resection of the duplicated bowel, but shared vascularity with the normal adjoining bowel makes the task daunting. We present a case of long tubular small intestinal duplication with certain special surgical and perioperative challenges which were successfully managed.

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A 27-day-old male neonate, presented with feculent discharge from the scrotum. Operative findings revealed incarcerated right inguinal hernia with perforated Meckel's diverticulum as its content, leading to enteroscrotal fistula. Resection of the Meckel's diverticulum and end-to-end ileoileal anastomosis was performed along with repair of inguinal hernia from within the abdominal cavity. The outcome was favorable. Enteroscrotal fistula due to incarceration of inguinal hernia is a rare presentation. We are adding to the literature, an extremely rare case of incarcerated Littre's hernia in the right inguinal region presenting as enteroscrotal fistula in a neonate.

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Aims and Objectives: This study aims to study the variation in the expression of CD117-positive interstitial cells of Cajal-like cells (ICC-LC) across the upper urinary tract region in children presenting with pelvic-ureteric junction obstruction (PUJO) and its association with renal functional and sonological parameters of patients. Materials and Methods: A prospective observational study was done on 20 children with congenital PUJO who underwent dismembered pyeloplasty. All children underwent renal sonography (anteroposterior pelvic diameter [APPD], pelvicalyceal ratio [P/C ratio], Mid polar renal parenchymal diameter [MPPD]) and functional imaging scan (LLEC scan or DTPA scan). Three specimens were taken intraoperatively from above PUJ, at the level of PUJ, and below PUJ. Those were examined immunohistochemically using CD117 to count ICC-LC using standard criteria. Variation in the expression of CD117-positive ICC-LC was correlated with the abovestated parameters. Results: The number of CD117-positive ICC-LC showed a continuous decreasing trend above downward. P/C ratio and APPD showed a parallel trend with ICC-LC distribution, whereas split renal function (SRF) showed an inverse relationship with the expression of ICC-LC. Children with lesser severity of obstruction (APPD <30 mm and SRF >40%) showed a uniform decreasing trend in the number of CD117-positive ICC-LC across PUJ. Children with more severe obstruction (APPD >30 mm and SRF <40%) showed a decrease in the expression of ICC-LC up to the level of PUJO followed by a sudden relatively increased expression of ICC-LC below the obstruction. Conclusion: The expression of ICC-LC shows a uniformly decreasing trend across obstruction when the severity of obstruction is less. Resurgence in the number of ICC-LC below PUJ in subjects with severe obstruction hints at the emergence of a new pacemaker area below severely blocked PUJ akin to that seen in complete heart block patients and deserves early attention.

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Background: Pancreatico-biliary maljunction (PBM) is often found to be associated with choledochal cysts (CCs). According to a European multicenter study, the prevalence of PBM in cases of CC is found to be 72.2%, however, there is no Indian study depicting the prevalence of PBM in Indian children with CCs, which is one of the main postulated factors in the etiopathogenesis of CC. In this study, we have attempted to observe prospectively the prevalence of PBM in children with CC and correlate this to its morphological and biochemical parameters. The association between the presence of PBM and histopathological findings such as epithelial changes of the mucosa of the CC, inflammation, metaplasia or dysplasia, and histopathology of the liver has also been evaluated. Materials and Methods: We carried out a single center, prospective observational study with a single arm study group. We prospectively selected all patients of CC admitted for surgery from November 2018 to October 2020. Data on biochemical, radiological, and histopathological parameters were collected and analyzed. Results: We included a total of 20 patients in our study. The mean age of the participants was 6.22 ± 4.32 years. Among them, 11 (55.0%) were male and 9 (45%) were female. Abdominal pain was the most common presenting complaint among our patients (75.0%) and had a significant association with the presence of a PBM (P = 0.001). In symptomatic children, the mean duration of symptoms was 4.50 ± 2.26 months for jaundice, 4.50 ± 1.98 months for abdominal distension, and 5.07 ± 2.02 months for abdominal pain. Among the 3 children with cholangitis, the mean number of episodes was 3.33 ± 2.08, with a median of four episodes. Fourteen (70.0%) of the children had type I a CC, 1 (5.0%) participant each for types I b, I c, II and IV a and 2 (10.0%) of them had type IV b cyst. The mean size of the cyst (cm) was 7.41 ± 3.03 with a median of 6.85 cm. Among the children, 9 (45%) showed the presence of PBM on magnetic resonance cholangiopancreatography (MRCP), with 7 (77.8%) showing Komi's C-P type and 2 (22.2%) showing Komi's PC type. The mean common channel length (mm) on MRCP was 8.11 ± 2.47 with a median length of 8.00 mm. The biochemical analysis of a bile fluid amylase and lipase is the functional indicator of the presence of a PBM. Histopathological examination showed the presence of ulceration in the walls of the CC in 10 (50.0%) of the specimens. There was a significant association of the presence of PBM and ulceration in the mucosa of the CC (P ≤ 0.001), with the median levels being the highest in the PBM present group. Conclusion: Abdominal pain is the most common complaint in a child with CC, and when present, it is significantly associated with the presence of a PBM. MRCP is the gold standard tool to detect CCs and find out the morphology of PBM. The prevalence of PBM in children with CC of 45% with a mean common channel length of 8.11 mm. The biochemical analysis of a bile amylase and lipase is the functional indicator of the presence of a PBM and there is a significant association of their higher levels and presence of PBM. The presence of chronic inflammation and microscopic ulcers is significant histologic parameters depicting the presence of a PBM.

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Introduction: Surgical complication following esophageal atresia repair is one of the several factors known to influence the final outcomes. Early identification of such complications may help in timely institution of therapeutic measures and translate into improved prognosis. Objective: The objective of this study was to evaluate the role of procalcitonin in early prediction of the adverse events after surgery in patients of esophageal atresia and the temporal relationship with clinical manifestations and other inflammatory biomarkers such as C-reactive protein (CRP). Materials and Methods: This was a prospective study on consecutive patients of esophageal atresia (n = 23). Serum procalcitonin and CRP levels were assessed at baseline (prior to surgery) and on postoperative days (POD) 1, 3, 5, 7, and 14. The trends in the biomarker values and temporal relationships of deviation in trend with the clinical and conventional laboratory parameters and patient outcomes were analyzed. Results: Baseline serum procalcitonin was elevated (n = 23; 1.7 ng/ml: min: 0.07 ng/ml–max: 24.36 ng/ml) in 18/23 (78.3%) patients. Procalcitonin nearly doubled on POD-1 (n = 22; 3.28 ng/ml: min: 0.64 ng/ml–max: 16.51 ng/ml) followed by a gradual decline. CRP was also elevated on POD-1 (three times the baseline) and depicted a delayed peak at POD-3. POD-1 procalcitonin and CRP levels correlated with survival. POD-1 procalcitonin cutoff at 3.28 ng/ml predicted mortality with a sensitivity and specificity of 100% and 57.9% (P = 0.05). Serum procalcitonin and CRP were higher for patients who sustained complications, so was the time required for hemodynamic stabilization. Procalcitonin (baseline and POD-5) and CRP (POD-3 and POD-5) values correlated with the clinical course after surgery. Baseline procalcitonin cutoff at 2.91 ng/ml predicted the possibility of a major complication with a sensitivity of 71.4% and a specificity of 93.3%. POD-5 procalcitonin cutoff at 1.38 ng/ml predicted the possibility of a major complication with a sensitivity of 83.3% and a specificity of 93.3%. Patients who sustained major complications depicted a change in serum procalcitonin trend 24–48 h ahead of clinical manifestation of an adverse event. Conclusions: Procalcitonin is a good indicator to identify the adverse events in neonates after surgery for esophageal atresia. The procalcitonin levels in patients who sustained a major complication depicted a reversal in trend 24–48 h of clinical manifestation. POD-1 procalcitonin correlated with survival while the baseline and POD-5 serum procalcitonin predicted the clinical course.

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Background: Management of short bowel syndrome in children has been surrounded by much debate with timing of the lengthening procedure still controversial. Early bowel lengthening procedure (EBLP) has been defined as any bowel lengthening procedure performed before 6 months of age. The purpose of this paper is to report the institutional experience in EBLP and to review the literature on this subject to identify common indications. Methods: An institutional retrospective analysis of all the intestinal lengthening procedures was performed. Furthermore, an Ovid/Embase search regarding children who underwent bowel lengthening in the past 38 years was conducted. Primary diagnosis, age at procedure, type of procedure, indication, and outcome were analyzed. Results: Ten EBLP were performed in Manchester from 2006 to 2017. Median age at surgery was 121 days (102–140), preoperative small bowel (SB) length was 30 cm (20–49) while postoperative SB length was 54 cm (40–70), with a median increased bowel length of 80%. Ninety-seven papers were reviewed, with more than 399 lengthening procedures performed. Twenty-nine papers matched criteria with more than 60 EBLP were observed of which 10 were performed in a single center from 2006 to 2017. EBLP was performed due to SB atresia, to excessive bowel dilatation or failure to enteral feeds, at a median age of 60 days (1–90). Serial transverse enteroplasty was the most frequent procedure used lengthening the bowel from 40 cm (29–62.5) to 63 cm (49–85), with a median increased bowel length of 57%. Conclusions: This study confirms that no clear consensus on indication or timing to perform early SB lengthening is reported. According to the gathered data, EBLP should be considered, only in cases of actual necessity after review of qualified intestinal failure center.

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Historically it was recommended for emergency thoracotomy in thoracic trauma as the last resort when there was cardiopulmonary arrest. Nowadays, the only indications are lung transplantation and huge mediastinal masses. We report the use of a clamshell thoracotomy in a 7-month-old boy with a large anterior mediastinal mass extending into the bilateral thoracic cavities.

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In a female neonate with anorectal malformation (ARM), the diagnosis is usually simple. But when there are two openings in the introitus and absent anal opening at the normal site, the scenario poses a diagnostic challenge. Careful and detailed evaluation of anomaly is therefore necessary before planning definitive correction. Imperforate hymen, although is not commonly associated with ARM should always be kept in mind as a differential diagnosis and other vaginal anomalies like Mayer–Rokitansky–Küster–Hauser syndrome should be ruled out before definitive surgical correction.

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Purpose: Several risk classifications based on various preoperative factors have been proposed to prognosticate the immediate survival of children operated for esophageal atresia. A major drawback of these classifications is that they only focus on immediate survival while ignoring the long-term morbidity and mortality in these children. Our study aims to bridge this gap in knowledge by studying the impact of one such classification (Okamoto's classification) on mortality and morbidity during a period of 1 year after discharge from the hospital in operated cases of esophageal atresia. Materials and Methods: After institutes ethical clearance, 106 children operated for esophageal atresia-tracheoesophageal fistula between 2012 and 2015 were studied prospectively for a period of 1 year after their discharge. The children were graded as per Okamoto classification. The primary objective was to determine the efficacy of this classification in predicting the survival rates in infancy and the secondary objective was to compare the complication rates in these children based on the classification. Results: Sixty-nine children met the inclusion criteria. There were 40, 15, 10, and 4 children in Okamoto Classes I, II, III, and IV, respectively. Twenty-one patients (30%) died during the follow-up period with the maximum number of deaths occurring in Okamoto Class IV (75%) and the minimum in Okamoto Class I (17.5%) (P = 0.003). There was a significant correlation between the Okamoto classes with the incidence of poor weight gain (P = 0.001), lower respiratory tract infection (P = 0.007), and failure to thrive (P = 0.01) higher in Okamoto IV and III as compared to I and II. Conclusion: Okamoto prognostic classification during the initial hospitalization is relevant even at 1 year follow-up with increased mortality and morbidity in Okamoto Class IV as compared to Class I.

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Context: Gaucher's disease (GD) is a rare inherited metabolic disorder caused by the defective activity of glucocerebrosidase. Enzyme replacement therapy (ERT) and substrate reduction therapy are the treatment of choice. Total splenectomy has a role when the child develops complications of massive splenomegaly. There are only a few case series of partial splenectomy in GD in the pediatric age group. Aims: To study the role, technical feasibility, and challenges of partial splenectomy in children with GD with hypersplenism. Materials and Methods: Retrospective review of children of GD who had partial splenectomy between February 2016 and April 2018. Demographics, clinical findings, laboratory, operative details, transfusion requirements, and perioperative, immediate, and late complications were retrieved. Clinical courses after discharge were obtained from follow-up data. Results: Eight children with GD underwent partial splenectomy between 2016 and 2018. The median age at surgery was 3 years and 6 months (range −2 years to 8 years). Five children underwent partial splenectomy successfully, of which one child required postoperative ventilatory support for 48 h owing to lung atelectasis. Three children underwent completion splenectomy due to bleed from the cut surface of the splenic remnant. One of the children who underwent completion splenectomy expired on the postoperative day 5 due to refractory shock with multi-organ dysfunction. Conclusion: Partial splenectomy has a definite role in selected children who present with massive splenomegaly with mechanical effects and/or hypersplenism while awaiting ERT.

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Endobronchial tuberculosis is reported in 18% of adults and 30%–60% of children with primary pulmonary tuberculosis. We are reporting two infants who presented with nonspecific respiratory symptoms due to an obstructive tubercular polypoid mass which was detected on computed tomography. Bronchoscopy showed a pale friable polypoid lesion in the bronchus causing a luminal obstruction. The biopsy of the lesion was suggestive of tuberculosis. On treatment with antitubercular medications, both the babies improved and remained asymptomatic on long-term follow-up.

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Primary synovial sarcoma of the thyroid gland is extremely rare, aggressive, and has a dismal prognosis. We report the case of a 15-year-old male who presented with a progressively increasing neck mass, which was excised and the histopathological and immunohistochemical study suggested biphasic synovial sarcoma of the thyroid gland which was confirmed by synovial sarcoma translocation. There are 14 cases of primary synovial sarcoma of the thyroid reported in the literature so far. This study aimed to document the occurrence of synovial sarcoma histology at an unusual anatomical location with a review of the literature on this rare entity.

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