Peutz-Jeghers syndrome: management for recurrent intussusceptions.

BACKGROUND: Peutz-Jeghers syndrome (PJS) is an autosomal dominant disorder characterized by hamartomatous gastrointestinal polyps along with the characteristic mucocutaneous freckling. Multiple surgeries for recurrent intussusception in these children may lead to short bowel syndrome. Here we present our experience of management in such patients. METHODS: From January 2015 to December 2023, we reviewed children of PJS, presented with recurrent intussusceptions. Data were collected regarding presentation, management, and follow-up with attention on management dilemma. Diagnosis of PJS was based on criteria laid by World Health Organization (WHO). RESULTS: A total of nine patients were presented with age ranging from 4 to 17 years (median 9 years). A total of eighteen laparotomies were performed (7 outside, 11 at our centre). Among 11 laparotomies done at our centre, resection and anastomosis of bowel was done 3 times while 8 times enterotomy and polypectomy was done after reduction of intussusception. Upper and lower gastrointestinal endoscopy (UGIE & LGIE) was done in all cases while intraoperative enteroscopy (IOE) performed when required. Follow-up ranged from 2 months to 7 years. CONCLUSION: Children with PJS have a high risk of multiple laparotomies due to polyps' complications. Considering the diffuse involvement of the gut, early decision of surgery and extensive bowel resection should not be done. Conservative treatment must be tried under close observation whenever there is surgical dilemma. The treatment should be directed in the form of limited resection or polypectomy after reduction of intussusception.

Unicentric Portocaval Castleman Disease in an Adolescent: Challenges in Diagnosis and Management.

Castleman disease is an unusual, benign disorder of unknown etiology, characterized by the proliferation of the lymphoid tissue. It can have a unicentric or multicentric presentation, depending on the number of lymph nodes involved. On clinical examination and imaging, it can imitate a malignancy and the diagnosis can only be confirmed on histopathological examination. Retroperitoneal location and presentation in the pediatric age group are extremely rare. We report a case of an adolescent girl with a unicentric lymph nodal mass in the portocaval space which was completely excised.

Physiological and Anesthetic Considerations of Safe and Optimal Pneumoperitoneal Pressures for Laparoscopic Surgeries in Children.

Context: In the era of minimally invasive surgeries, pediatric laparoscopic surgeries are now becoming the standard of care. Aim: In this study, we aim to determine the safe and optimal pneumoperitoneal pressures (PPs) for laparoscopic surgery in children aged 1-5 years, along with the technical ease for the surgeon. Settings and Design: Prospective, randomized, single-blinded study was conducted at SGPGI Lucknow. Materials and Methods: Children aged 1-5 years were randomized into Group I (n = 24): PP = 6-8 mmHg and Group II: (PP) = 9-10 mmHg. Hemodynamic, ventilatory, and blood gas changes were measured before CO2 insufflation (T0), 20 min after insufflation (T1), before desufflation (T2), and 10 min after desufflation (T3). Surgeon's technical ease of surgery, postoperative pain, the requirement of rescue analgesia, time to resume feeding, and complications were recorded and analyzed. Statistical Analysis Used: Paired t-test, Mann-Whitney test, and Wilcoxon signed-rank test were used for nonparametric/parametric data. Chi-square/Fisher's test was used for nominal data. Results: Partial pressure of CO2 (PaCO2) was significantly higher in Group II at T1, T2, and T3, requiring frequent changes in ventilatory settings. Postoperative pain scores were higher in Group II at 1, 6, and 12 h, requiring rescue analgesia. Surgeon's scores and hemodynamics were similar in both groups. Conclusions: Higher PP in Group II caused significant changes in PaCO2, end-tidal CO2, and postoperative pain requiring rescue analgesia, but blood gas changes were clinically insignificant and there were no significant changes in hemodynamic parameters. Since the surgeon's ease of performing surgery was similar in both groups, we recommend that laparoscopy in children aged 1-5 years can be started with lower PPs of 6-8 mmHg, which can be increased if needed based on the surgeon's comfort and the patient's body habitus.

Safety and Efficacy of Enhanced Recovery after Surgery Protocol in Pediatric Patients Undergoing Hepato-Pancreatico-Biliary Surgery: A Prospective Randomized Control Study.

Introduction: Enhanced recovery after surgery (ERAS) is a multidisciplinary approach to optimize patient care. The goal of this approach is to reduce the body's reaction to surgical stress by optimizing the perioperative nutritional status, promoting analgesia without opioids, and early postoperative feeding. In pediatric patients, very limited literature is available for the application of ERAS protocol. This study was done to evaluate the application of ERAS protocol in pediatric hepatobiliary and pancreatic patients. Materials and Methods: This is a randomized prospective study conducted over a period of 2 years at a tertiary center in North India. A total of 40 hepatobiliary and pancreatic patients who were willing to participate in the study were included in the study. Patients were randomized by computer-generated method and data were collected regarding demography, clinical diagnosis, preoperative and postoperative workup, and peri-operative care including analgesia, pain scores, postoperative recovery, hospital stay, and complications. These patients were followed for 6 months postoperatively and the results were evaluated using SPSS software. Results: The study included 20 patients each in both the conventional and ERAS group with median ages of 11.5 years and 7.1 years, respectively. The data analysis showed that the ERAS group of patients had better outcomes in terms of hospital stay and drain removal time with significant statistical differences. Pain scores and complications are almost the same in both groups. Conclusion: Principles of ERAS can be safely applied in pediatric patients undergoing major surgery in the present era of emerging infections and also increasing patient burden without morbidity.

How to suspect the presence of high-risk esophageal varices and when to start endoscopic surveillance in children with biliary atresia?

BACKGROUND AND AIM: Portal hypertension determines the outcome of children with biliary atresia (BA) and is common even after a successful Kasai portoenterostomy (KPE). However, there are no clear-cut guidelines on the age of starting surveillance and the modality (endoscopy vs non-invasive tests [NITs]). In this cohort study, we analyzed our database to find out the utility of NITs in detecting high-risk esophageal varices in BA. METHODS: From June 2010 to May 2022, consecutive children of BA who underwent upper gastrointestinal (UGI) endoscopy were included. Esophageal varices were classified as high-risk (grade II with red-color signs or grade III or IV irrespective of red-color signs. NITs such as splenomegaly (clinical and USG), platelet count, aspartate transaminase to platelet ratio index (APRI), and platelet-to-spleen diameter ratio were compared between cases with high-risk and low-risk varices. RESULTS: A total of 110 children, 75 boys (66 successful KPE and 44 failed/KPE not performed) were enrolled. The median age at KPE was 85 days (IQR 63-98). Thirteen (11.8%) children presented with UGI bleeding. The first endoscopy revealed gastroesophageal varices in 75.4% of cases, and 32% of them had high-risk varices. Multivariate analysis revealed failed KPE, history of UGI bleeding, bigger spleen size (> 3.5 cm), lower platelet count (< 150 000), and higher APRI (> 2) are independent predictors of the presence of high-risk esophageal varices. CONCLUSION: Endoscopy is the best in predicting the presence of high-risk varices that might bleed; hence, early surveillance endoscopy should be started in children with splenomegaly, thrombocytopenia, and high APRI score to prevent variceal bleeding.

Evolution of Antenatally Diagnosed Bronchogenic Cyst in an Infant.

Bronchogenic cyst is one of the rare developmental lung conditions. Depending on the location, it can cause significant compression of the mediastinal structures, especially airways leading to atelectasis, emphysema, wheezing, and stridor. Computerized tomography helps in the confirmation of diagnosis. Surgery is definitive management. We present a case of bronchogenic cyst which presented as emphysema leading to respiratory emergency in an infant.

Fetal Klippel-Trenaunay-Weber Syndrome: Antenatal Diagnosis and Postnatal Management.

Context: Klippel-Trenaunay-Weber syndrome (KTWS) is a rare disease characterized by a triad of venous malformations, vascular skin nevus and asymmetric hypertrophy of bone and soft tissue. The spectrum of disease in utero varies from asymptomatic nevus flammeus to life threatening complications like Kasabach-Merritt phenomena. Aim: The aim of this study was to review our experience of antenatal diagnosis of KTWS and it's postnatal management. Settings and Design: This was a retrospective observational study of all pregnant women who were antenatally diagnosed with KTWS and postnatally confirmed at a tertiary care center in north India between 2012 and 2021. Subjects and Methods: The electronic medical records were reviewed and data were collected regarding demographic information, obstetric history, clinical presentation, sonographic findings, mode of delivery, fetal outcome, and follow-up. Results: During the study period, four fetuses were diagnosed with KTWS on sonography. Three women were multigravida whereas one was a primigravida. Two women opted for medical termination of pregnancy and one each had liveborn child and an intra-uterine fetal death. KTWS was confirmed in all cases. The liveborn child underwent treatment for the vascular malformation and is alive at 4 years of age. Conclusions: This study attempts to add onto the available literature regarding the spectrum of prenatal presentations of KTWS. It emphasizes the importance of prenatal diagnosis and follow-up of the fetus/neonate.

Giant Feeding Artery from Abdominal Aorta in Intralobar Pulmonary Sequestration.

Pulmonary sequestration (PS) is a rare congenital lung malformation. We present a case of newborn with antenatally diagnosed case of PS. The baby was delivered by cesarean section to primigravida mother at 38 + 3 weeks of gestation with birth weight of 2700 g. At 20 weeks of gestation, the antenatal ultrasound showed a triangular echogenic area in left lung supplied by feeding artery from descending aorta. The baby had respiratory distress soon after birth. Computerized tomographic pulmonary angiogram revealed abnormal blood supply of left lower lobe arising from descending aorta. The feeding vessel was abnormally large and was almost half of the diameter of the aorta. The baby underwent left lower lobectomy with double ligation of feeding artery at day 7 of life and was discharged on day 14 of life. Antenatal diagnosis of congenital lung malformation helps in careful planning of perinatal care and facilitates the early diagnosis and management.

Posterior Cloaca: Multicenter Experience of a Very Rare and Unusual Anorectal and Genitourinary Malformation.

Aim: Posterior cloacal malformations (PCMs) are distinguished from classical cloacal malformations by the posterior location of the common opening in the perineum. We aim to describe our experience of management of these rare and complex malformations. Methods: This study was a retrospective chart review of all patients with PCM who underwent treatment at Kalawati Saran Children's Hospital (KSCH), New Delhi, and Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow, between 2013 and 2021. Individual anatomical variations and their impact on the clinical presentation, management, and final outcome (cosmesis and function) were recorded and analyzed. Results: During the study period, four girls with a median age of 2 (range: 0-5) years presented to us with PCM. Three patients were referred to KSCH as ambiguous genitalia whereas one patient was born at SGPGIMS with an antenatal diagnosis of "urorectal septal defect." On examination, three patients had the urogenital sinus (UGS) opening immediately anterior to the normally located anal opening (2 orifices), whereas one had a single orifice at the normal location of the anus with the UGS opening in the anterior rectal wall. Associated anomalies included: (a) hydrocolpos (n = 3), which was managed by a tube vaginostomy; (b) urethral duplication with dorsal atretic urethra (n = 3); (c) uterine didelphys (n = 1); (d) bilateral grade 5 vesicoureteric reflux (n = 1); and (e) vaginal calculus (n = 1). Total urogenital mobilization (anterior sagittal approach) with feminizing genitoplasty was performed for patients with UGS and normal anus (n = 3). In the patient with a single opening at the normal location of the anus, posterior sagittal ano-recto-urethro-vaginoplasty was performed. At a median follow-up of 24 months, three patients are continent for urine and stool, whereas one patient is yet to be toilet trained. Conclusions: PCMs are unusual complex malformations that necessitate meticulous clinical examination, detailed diagnostic workup, and multistaged surgical management. Management should be tailored as per each individual patient's anatomy and clinical presentation for an optimal outcome.

Bronchial Carcinoid Tumor in an Adolescent Female: Diagnosis and Management by a Multi-Disciplinary Team.

Bronchial carcinoid is the most common primary malignant lung tumor in children; however, it remains a very rare diagnosis due to the overall low incidence of childhood lung malignancies. We report a case of a 17-year-old girl with respiratory symptoms who was initially misdiagnosed as a case of COVID pneumonia. She was later detected to have a right mainstem bronchial carcinoid which was managed successfully by a multi-disciplinary team.

Optimising working space for laparoscopic pyeloplasty in infants: Preliminary observations with the SGPGI Protocol.

AIMS: This study aimed to test the efficacy of SGPGI protocol to minimise bowel distension and optimise working space for laparoscopic pyeloplasty in infants. METHODOLOGY: All infants who underwent laparoscopic pyeloplasty for unilateral pelvi-ureteric junction obstruction (PUJO) between January 2017 and March 2020 were included in the study. The patient cohort was divided into two groups: Group A and B. Group A included patients who underwent routine pre-operative preparation. Group B included patients wherein the SGPGI protocol was used. The key features of the protocol were fasting for 8 h, enemas, inserting a nasogastric tube in the pre-operative period and decompressing the colon on the operation table. Demographic features, pre-operative, intraoperative and post-operative parameters were compared between the two groups. RESULTS: A total of 26 infants with unilateral PUJO underwent laparoscopic pyeloplasty during the study period. Group A included 12 patients and Group B included 14 patients. Both the groups were similar in age, weight and sex distribution. The median surgeon's rating score for suturing conditions was 2 for Group A and 5 for Group B patients (P > 0.05). The operating time was significantly longer in Group A (196 ± 21 min) as compared to Group B (114 ± 18 min) (P < 0.05). In Group A, intra-abdominal pressure (IAP) varied between 9 and 14 mmHg (median 12 mmHg), while in Group B, IAP varied between 6 and 9 mmHg (median 8 mmHg) (P < 0.05). In Group A, in 2/12 cases (16.7%), conversion to an open procedure was necessary because of inadequate working space owing to gross intestinal distension. Two patients in Group A also had intraoperative injuries to adjacent structures due to poor working space. CONCLUSIONS: Optimal working space is critical to the performance of advanced laparoscopic surgery like pyeloplasty in infants. SGPGI protocol significantly improves working space, which permits a faster and safer surgery with a lower intra-abdominal working pressure. This protocol is simple, safe and easy to replicate at most centres in our country.

Clinical profile, outcome and challenges in the management of pediatric Burkitt lymphoma: a single center experience.

This study was conducted with the aim to assess the clinico-pathological profile, treatment outcomes and the challenges faced in Low Middle Income Countries (LMIC) during management of pediatric Burkitt lymphoma cases on intensive chemotherapy protocol. This was a single center retrospective analysis of pediatric Burkitt lymphoma cases (age <18 years) managed uniformly with Lymphomes Malins B (LMB) 96 chemotherapy protocol between January 2015 and September 2019. 40 cases were analyzed with a median age 11.5 years (range 4-18 years) and male: female ratio =4.7:1. Patients belonging to different LMB risk groups were: A-3 (7.5%), B-31 (77.5%), and C-06 (15%). 25 (62.5%) patients had abdominal disease at presentation. The survival analysis of different treatment risk groups showed statistically significant difference in mean Overall Survival (OS) between group A-100%, group B- 87%±6.1% and group C-44.4%±16.2%; (p value = .016). On multivariate analysis of prognostic factors affecting survival, CNS involvement (p value = .03) and median time from diagnosis to treatment initiation more than 30 days (p value = .04) were significantly associated with poor outcome. Incidence of culture positive febrile neutropenia episodes was 28.2% of which 69.2% infections were caused due to carbapenem resistant gram-negative organisms. In our study, although the outcomes in risk group A and B patients were comparable to LMB 96 treatment results, the outcome in risk group C was considerably poor primarily due to advanced disease at presentation and delayed diagnosis. The critical challenges that we faced in our cohort were delayed diagnosis, treatment cost affordability, poor nutritional status, and high infection related mortality.

Head-and-Neck Solid Tumors in Children: A Retrospective Review from a Tertiary Care Institute in North India.

OBJECTIVES: The aim of this study was to review the clinical profile, management, and outcome of solid tumors in the head-and-neck region in children at our institute. METHODS: We retrospectively reviewed children with head-and-neck solid tumors who were treated jointly under the departments of Pediatric Surgery and Otorhinolaryngology at our institute between 2016 and 2019. RESULTS: In the study period, 10 children (6 males, 4 females) with a median age of 9.5 years (range 5 days-16 years) were treated by our unit. The patients had four tumors arising from the parotid, 2 from nose/naso-pharynx, 1 each from the tongue, submandibular gland, para-pharyngeal space, and infratemporal fossa. A majority (90%) of the patients underwent complete surgical excision of the tumor, preserving the loco-regional neurovascular structures to minimize postoperative morbidity. Malignant lesions were seen in 4 patients (2 rhabdomyosarcoma, 1 primitive neuro-ectodermal tumor, 1 mucoepidermoid carcinoma) and 6 patients had benign pathology (3 pleomorphic adenoma, 2 mature teratoma, 1 schwanomma). Additional therapy with chemotherapy and local radiotherapy was required in 3 out of 4 patients with malignant pathology. The median follow-up duration is 15 months. At last follow-up, all patients are alive and 9 out of 10 patients (90%) are disease-free. CONCLUSION: Our experience highlights rare and difficult tumors in the head-and-neck region in children. These tumors are not commonly managed by the pediatric surgeon in routine practice. The paper outlines the multi-modality management of these tumors, which is essential for an optimal outcome.

Non-variceal gastrointestinal bleed in children: surgical experience with emphasis on management challenges.

PURPOSE: This exclusively surgical series on pediatric non-variceal gastrointestinal bleed (NVGIB) defines three levels of bleed site and describes etiology, bleed severity, diagnostic algorithm, and surgical management for each bleed site. Management challenges are detailed. METHODS: Patients aged ≤ 18 years treated surgically for NVGIB were analysed. RESULTS: Bleed site (n = 87) was classified as: upper gastrointestinal bleed (UGIB; n = 11); small bowel bleed (SBB: n = 52); and lower GIB (n = 24). Four etiology-based groups were identified: lesions with ectopic gastric mucosa (EGM; n = 33), tumours (n = 23), ulcers (n = 21), and vascular pathology (n = 8). Bleed severity spectrum was: acute severe bleed (n = 12); subacute overt bleed (n = 59); and occult GIB (n = 16). Preoperative diagnosis was obtained in all UGIB and LGIB lesions. Eighty-two percent of surgical SB lesions were diagnosed preoperatively on Tc99m pertechnetate scan, computed tomography enterography-angiography, and capsule endoscopy; remaining 18% were diagnosed at laparotomy with intra-operative enteroscopy (IOE). Surgical management was tailored to bleed site, severity, and etiology. Indications of IOE and approach to management challenges are detailed. CONCLUSIONS: The commonest site-specific bleed etiologies were duodenal ulcers for UGIB, EGM lesions for SBB, and tumours for LGIB. SBB presented diagnostic challenge. Diagnostic algorithm was tailored to bleed site, age-specific etiology, bleed severity, and associated abdominal/systemic symptoms. Management challenges were acute severe bleed, occult GIB, SBB, obscure GIB, and rare etiologies. IOE has a useful role in SBB management.

Yolk Sac Tumor of Stomach: Case Report and Review of Literature.

Gastric yolk sac tumors (YSTs) are very rare. Only seven cases of pure YST of the stomach, with a median age of 65 years, and five cases of YST combined with adenocarcinoma have been reported. We report a 3-year-old boy who presented to us with recurrent episodes of melena and anemia of 2 months' duration. Upper gastrointestinal endoscopy revealed a deeply excavated ulcer on the lesser curvature, 4 cm from the gastroesophageal junction. Computed tomography (CT) abdomen revealed large heterogeneously enhancing soft-tissue mass lesion in the lesser sac with indistinct fat planes with the lesser curvature of the stomach with multiple peripherally enhancing lesions in both lobes of liver and omentum, suggestive of metastasis. Serum alpha-fetoprotein (AFP) was 21,000 ng/ml. Tru-cut biopsy from the mass was suggestive of YST. Following three courses of chemotherapy based on Cisplatin, Etoposide and Bleomycin (PEB), sleeve gastrectomy with excision of the mass with omentectomy with biopsy of the liver nodules with a sampling of perigastric lymph nodes was done. Histopathology revealed only necrotic tissue with no evidence of residual malignancy. Three-month follow-up CT revealed no residual mass with AFP of 3 ng/ml.

Laparoscopic Transperitoneal Adrenalectomy for Adrenal Tumors in Children: Technical Considerations and Surgical Experience.

Aims: This study aimed to review our surgical experience of laparoscopic adrenalectomy (LA) for adrenal masses in the pediatric age group. Materials and Methods: The electronic medical records of all patients younger than 18 years of age who underwent LA between 2016 and 2023 were retrospectively reviewed. Children with adrenal tumors localized to the site of origin without evidence of encasement of major vasculature or involvement of adjacent organs were considered for LA. Patients with diagnosis of malignant adrenal tumor on preoperative work-up underwent open adrenalectomy. Data were collected regarding demography, clinical presentation, hormonal workup, imaging, duration of surgery, transfusion requirement, conversion rate, postoperative recovery, duration of intravenous (i.v.) analgesia and hospital stay, pathology, complications, and status at follow-up. Results: Between 2016 and 2023, LA adrenalectomy was performed in 11 patients (6 boys and 5 girls) with a mean age of 46.3 (8-120) months. A functional tumor was detected in 6 (54.5%) children, manifesting clinically with Cushing's syndrome (3), virilization (1), feminization (1), or Conn's syndrome (1). Seven (63.6%) tumors originated from the right adrenal, and 4 (36.4%) from the left adrenal gland. The mean tumor weight and size was 49.1 (10-80) g and 5.6 (3-8) cm, respectively. Histopathology included adrenal adenoma (5), ganglioneuroma (3), ganglioneuroblastoma (1), myelolipoma (1), and intermediate adrenocortical tumor (1). The mean surgery duration was 186.6 (120-265) minutes. Intraoperative blood transfusion was required in 2 (18.2%) patients and none required conversion. Full feeds were resumed at 42.7 (24-60) hours, i.v. analgesia requirement was for 54.5 (36-72) hours and mean hospital stay was 5.1 (3-8) days. All patients were symptom-free with no recurrence at mean follow-up period of 50.6 (3-83) months. Conclusions: In children with well-circumscribed, localized, and noninvasive adrenal tumors, LA is feasible, effective, and safe with all advantages of minimally invasive surgery.

Discrimination of pediatric cryptogenic multifocal ulcerous stenosing enteritis from small bowel Crohn's disease and gastrointestinal tuberculosis: A retrospective study (with videos).

INTRODUCTION: Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) is a rare entity that mimics various inflammatory strictures of the small intestine. Pediatric literature is scarce. We analyzed the clinical, radiological, endoscopic and histopathological features of children with CMUSE that differentiate it from small bowel Crohn's disease (SBCD) and gastrointestinal tuberculosis (GITB). METHODS: CMUSE was diagnosed by the following criteria: (1) unexplained small bowel strictures with superficial ulcers, (2) chronic/relapsing ulcers of small bowel after resection, (3) no signs of systemic inflammation, (4) absence of other known etiologies of small bowel ulcers. SBCD and GITB were diagnosed based on standard criteria. The clinical features, laboratory parameters, radioimaging, endoscopy (including video capsule endoscopy [VCE], intra-operative endoscopy), histopathological features and treatment outcome were noted. RESULTS: Out of 48, CMUSE was diagnosed in 13 (27%) isolated small bowel and ileocecal strictures, while GITB and SBCD accounted for 41% and 21% cases, respectively. Common presentations were sub-acute obstruction (46%), obscure gastrointestinal bleeding (38%) and protein-losing enteropathy (38%). CMUSE patients had significantly longer disease duration compared to SBCD and GITB (p < 0.001). SBCD (90.0%) and GITB (85%) cases had elevated C-reactive protein (CRP), none with CMUSE had elevated CRP (p < 0.001). The disease was localized in jejunum (100%) and proximal ileum (56%) in CMUSE, ileocecal region (85%) in GITB, but evenly distributed in small intestine in SBCD. Endoscopy showed evenly placed, superficial, circumferential ulcers with strictures in CMUSE, deep linear ulcers in SBCD and circumferential ulcers in GITB. Upfront immunosuppression was given in four; three (75%) of them relapsed. Only surgery was done in three with one (25%) having relapse. Upfront surgery followed by immunosuppression was used in six, but all relapsed and two required repeat surgery. CONCLUSION: CMUSE is important but underdiagnosed in children. Lack of constitutional symptoms, normal inflammatory parameters and characteristic ulcers with strictures helped in differentiating CMUSE from GITB and SBCD.