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Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
Official journal of the Indian Association of Pediatric Surgeons         
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   Table of Contents - Current issue
July-August 2021
Volume 26 | Issue 4
Page Nos. 213-277

Online since Monday, July 12, 2021

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Pandemic and pediatric surgery: A wholistic impact assessment p. 213
Ravi Prakash Kanojia
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Growth and development assessment of children (1–5 years) operated for tracheoesophageal fistula/esophageal atresia: A case control study p. 216
Monika Maan, Sukhwinder Kaur, Geetanjli Kalyan, Ram Samujh, Nitin James Peters, Bhavneet Bharti, Prahbhjot Malhi
Introduction: Among children, esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is one of the major and common congenital anomalies. It is a life-threatening emergency and at birth may be associated with three C's coughing, choking, and cyanosis. It requires surgical interventions in the early neonatal period. The postsurgical period is associated with poor growth which can be developmental outcomes particularly in the first 5 years of life and attributed to postsurgical complications. The aim of the study is to assess and compare the growth and development of the children (1–5 years) operated for TEF/EA attending Pediatric Surgery OPD/admitted inwards at APC, PGIMER, Chandigarh versus healthy controls. Materials and Methods: A case–control study was conducted on age-matched 40 children aged between 1 and 5 years operated for TEF/EA and healthy controls. The sampling technique for cases was total enumeration and for controls was purposive sampling. Tools used were socio-demographic sheets of children, clinical profile of children, Trivandrum Development Screening chart, and Vineland Social Maturity Scale for Indian adaptation. Results: Majority 33 (82.5%) of children had distal TEF and more than two-third 28 (70%) have undergone primary repair. More than one-third 14 (35%) had a respiratory infection, 12 (30%) anastomosis leakage and 6 (15%) had Gastroesophageal reflux (GER) as one of the early and late postoperative complications. More than one-fourth 11 (27.5%) of TEF/EA operated children had less weight, 11 (30%) had less height and 16 (40%) had less weight for height for their reference age. A significant difference was found for height for age, weight for height, and social maturity among children who had TEF repair as compared to their healthy counterparts. Conclusion: Growth monitoring reflected (more than one-fourth of children were underweight and stunted while more than one-third were wasted) and showed development delay in TEF/EA operated children as compared to healthy controls.
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Results of injection sclerotherapy with bleomycin in pediatric lymphatic malformations p. 223
Vipan Kumar, Subhasis Roy Choudhury, Partap Singh Yadav, Vikram Khanna, Amit Gupta, Rajiv Chadha, Rama Anand
Aim: The aim of the study was to evaluate the results of injection sclerotherapy with bleomycin in pediatric patients with lymphatic malformations. Materials and Methods: In this prospective cohort study, all consenting pediatric patients with macrocystic lymphatic malformations were managed with injection bleomycin sclerotherapy (0.5 mg/kg, not exceeding 5 mg at a time) under ultrasound (US) guidance. After aspirating the cyst fluid bleomycin was instilled intralesionally in a ratio of 5:1 (aspirated cyst fluid volume: diluted bleomycin solution volume). Patients were reassessed at three weekly intervals. The response to therapy was assessed clinically as well as by size and volume on ultrasound Doppler study. The response was classified as excellent response, i.e., complete regression, good response >50% regression, and poor response <50% regression. Results: Sixty patients with lymphatic malformations were enrolled in the study, the mean age was 3.22 years, and the male-to-female was 2.5:1. The most common site of lesion was in the neck (43.3%), followed by the axilla (15%) and flank (8.3%). The responses were excellent, good, and poor in 43 (71.6%), 12 (20%), and five (8.3%) patients, respectively. Two patients underwent surgical excision of the residual lesion. Complications noted were fever in six, local pain in five, and residual lesion in three patients. Conclusion: Sclerotherapy with bleomycin is simple, safe, and effective in the first line of management for macrocystic lymphatic malformations in children.
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Asymptomatic cholelithiasis in children: Management dilemma p. 228
Kuntal Bhaumik
Aims and Objectives: The incidence of cholelithiasis is now increasing in children. Besides hemolytic diseases, there are also nonhemolytic conditions and idiopathic group. A large number of children belong to asymptomatic group who do not present with gallstone-related symptoms. There is no consensus in the management of these children. The aim of this study is to evaluate the role of elective cholecystectomy in asymptomatic cases to prevent complications which leads to more morbidity. Materials and Methods: One hundred and seventy-eight children were treated over a period of 12 years and they were divided into two groups according to their age at presentation. Sixty-four children below 5 years belonged to Group A and 114 children between 5 and 12 years belonged to Group B. About 71.8% of children of Group A and 49.1% of children of Group B were asymptomatic. Cholecystectomy was advised in all cases of Group B and all symptomatic cases of Group A. In Group A asymptomatic cases, ursodeoxycholic acid (UDCA) was given for 6–12 months and followed up. Results: Laparoscopic cholecystectomy was performed in all cases except in five cases where conversion to open surgery was done as there was gross adhesions due to previous laparotomy. Of 46 asymptomatic cases of Group A, the stone disappeared in seven cases, 12 children developed symptoms, and there was no change in 27 patients. Stone reappeared again in three of seven children who were managed by cholecystectomy. Cholecystectomy was also performed in 12 cases that developed symptoms. In the rest of the 27 children, cholecystectomy was advised after 1 year trial of UDCA. Cholecystectomy was advised in both symptomatic and asymptomatic cases of Group B. Sixteen of 56 asymptomatic cases did not agree for cholecystectomy and 12 of them returned with complications. Endoscopic retrograde cholangio-pancreaticography (ERCP) and stone extraction was performed in four cases. In all the acute cases, cholecystectomy was performed after a period of conservative management. Conclusion: UDCA can be tried in the smaller age group below 5 years, but there is a chance of recurrent stone formation. Elective laparoscopic cholecystectomy should be the choice in all asymptomatic cases to prevent complications.
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Angiotensin-converting Enzyme Inhibitors: Can it be a Potential Treatment of Infantile Hemangioma p. 234
Archika Gupta, Shiv Narain Kureel, Anand Pandey, Gurmeet Singh, Akhilesh Kumar, Gaurav Shandilya, Rahul Kumar Rai, Survesh Kumar Gupta
Aims: The aim of the sudy was to evaluate potential role of oral captopril, an angiotensin-converting enzyme (ACE) inhibitor, and in treatment of infantile hemagioma (IH) and report our preliminary results. Methods: This prospective study included 18 children with IH admitted in the department of pediatric surgery with no history of prior treatment of any type. Baseline blood pressure (BP), electrocardiogram, two-dimensional echocardiography, serum electrolytes, and renal function test (RFT) were noted. Oral captopril was started as first-line drug at a dose of 0.1 mg/kg orally 12 h with gradually increase of dosage up to 2.0 mg/kg 12 h over the period of 10 days with monitoring of BP, serum electrolytes, RFT, and occurrence of any side effect. If no side effects were noted and patients were stable, they were discharged and followed up until 6 months after stopping treatment. During follow-up, response to treatment was documented clinically and photographically. Development of any side effect was also noted. Results: Excellent response to captopril was noticed in nine patients over 16–18 months. Four patients showed good response. Oral propranolol had to be administered alternatively in one patient showing fair response during the initial 4 months but no response afterward and in four patients showing no response at all. One patient developed an allergic reaction to propranolol and was started oral corticosteroid. These five patients had near complete resolution of lesion for the next 8–10 months. Conclusions: ACE inhibitors might have a role, though slow, in the involution of IHs. Therefore, these may have the potential to emerge as an alternative treatment for IH in future after confirmation with randomized studies with propranolol.
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Plasma fibrinogen: An independent predictor of pediatric appendicitis p. 240
MS Vinod Kumar, Mannu Kumar Tiwari, Jasdeep Singh, Anil Malik
Aims: Appendicitis, in spite of all the diagnostic advances, achieving an accurate and timely diagnosis of this common condition in children remains a challenge. Plasma fibrinogen (FB) is an acute inflammatory mediator and has been proposed and evaluated as an adjunct laboratory marker for improving diagnostic accuracy. The study evaluates the plasma values of Se FB along with other serum markers in pediatric appendicitis patients, to determine their diagnostic accuracy. Methods: Prospective observational study on 120 patients between the age group of 5 and 12 years. All eligible enrolled cases underwent total leukocyte count (TLC), plasma FB, C reactive protein (CRP), neutrophil-lymphocyte ratio (NLR), absolute neutrophil count (ANC), and erythrocyte sedimentation rate on admission along with pediatric appendicitis score. Final confirmation of diagnosis and allotment of cohort was made by intra operative findings and histopathological confirmation. Two groups were defined: (1) Histopathologically confirmed acute appendicitis-Cases (2) Nonspecific abdominal pain-Controls. Laboratory results were statistically analyzed between the case and the control groups for diagnostic accuracy. Results: Study reflected strong statistical significance in terms of leukocyte count, ANC, NLR, CRP, and FB levels. However, plasma FB (value above 4.02 g/L) had the highest diagnostic accuracy rate of 82.50% compared to other laboratory values (TLC-70.83%, CRP-70.00%). Conclusion: Plasma FB has emerged as an accurate diagnostic tool and its diagnostic accuracy is superior to all other laboratory parameter studied (TLC, CRP, NLR, and ANC). Plasma FB values above 4.02 g/L is an independent predictor of appendicitis and can help in reducing negative laparotomy in pediatric age group.
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Diaphragmatic hernia after pediatric living donor liver transplantation: An Indian experience with review of literature p. 246
Bramha Pattnaik, Piyush Kumar Sinha, Nilesh Patil Sadashiv, Nihar Mohapatra, Viniyendra Pamecha
Aim: Although intestinal obstruction following living donor liver transplantation (LDLT) is relatively common, diaphragmatic hernia (DH) as the attributing cause is rarely reported. The study aimed to find out the incidence, clinical presentation, and management of DH after liver transplant in pediatric patients. Materials and Methods: A retrospective review of all cases of pediatric liver transplants was performed. Case sheets, operation records, and investigations including radiology were studied. Results: Out of 79 cases of pediatric liver transplants, two cases of posttransplant DH were identified. The first case is a 2-year-old male child who was transplanted for progressive familial intrahepatic cholestasis, and the second one is a 4-year-old boy who underwent LDLT for hepatitis A-induced acute liver failure. The first child presented abdominal symptoms and the second one with pulmonary symptoms. The DHs were diagnosed at 132 and 70 days, respectively, posttransplant by chest radiographs and computed tomography scan. The defects were located at the posteromedial aspect of the diaphragm in both the cases and were closed by primary closure with mesh reinforcement. Both the children are doing well with no recurrence. Conclusion: High index of clinical suspicion is mandated in pediatric patients after liver transplant and once the diagnosis is confirmed, urgent surgical exploration is mandatory.
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Percutaneous cystolithotomy in augmented bladders p. 250
Uday Sankar Chatterjee, Indranil Chatterjee
Aims: Incidence and recurrence of bladder stone in augmented exstrophy bladder rate is high. So, recurrent open cystolithotomy is not a preferred procedure; particularly through scarred tissues, consequence of previous surgeries. Percutaneous cystolithotomy (PCCL) is an old but standard procedure for retrieval of bladder stones in adults. We extrapolated PCCL for bladder stone in augmented bladders in children. Patients and Methods: In three patients, we made suprapubic (SP) needle track with initial puncture (IP) needle under cystoscopic guidance. Following that laparoscopic cannula was placed through dilated SP track that was crafted with Alken's dilators and bladder stones were removed with grasper. Results: On cystoscopy, we also observed the patches of skin tissues in native bladders. Continence and bladder capacity were not affected following PCCL. Conclusion: PCCL in augmented bladder showed good outcome. High recurrence of bladder stone is possibly due to presence of keratin in dermal tissue; invaded mucosa in open bladder plate. It seems shaving or fulguration of those dermal elements during bladder reconstruction might decrease incidence of stone formation. However, we haven't attempted fulguration during PCCL.
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Transanal small bowel evisceration in a 3-year-old victim of motor vehicle accident p. 253
Minakshi Bhosale, Ameya Sangle, Ganesh Bhat, Praveen Dambal
Although motor vehicle accidents (MVAs) in children are common, pediatric rectal perforations secondary to MVAs leading to transanal evisceration of the small bowel are very rare. Herniation of bowel through breach in the rectal wall seen eviscerating through the anus is a surgical emergency requiring laparotomy and necessary surgical procedure. We report case of a 3-year-old boy, victim of run-over MVA accident, presenting with bilateral fracture shaft femur, fracture of the left humerus, and transanal small bowel evisceration. About 30–40 cm long, gangrenous, small bowel loop was hanging outside the anal canal. Two consecutive surgeries were performed to manage this unusual and complex case with an excellent outcome. This report is presented for an extremely rare presentation of MVA injury in a child.
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Congenital pyloric atresia and epidermolysis bullosa: Report of a rare association p. 256
Pradyumna Pan
Pyloric atresia (PA) is an unusual congenital disorder that accounts for about 1% of all intestinal atresia, with an incidence around 1 in 100,000 live births. PA may occur as an isolated disorder or may be associated with epidermolysis bullosa (EB). EB comprises a heterogeneous group of cutaneous genetic diseases of inherited blistering and skin fragility disorders. Wound management in EB is complex and influenced by several comorbidities and the fragility of the skin. The prognosis of children with EB depends on the type of mutation inherited. Some types are mild and even improve with age, while others are so severe it is not likely that a child to survive into adulthood. We present three cases of PA in this article and one in conjunction with junctional EB.
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Pentalogy of cantrell: Reconstructive challenges of two complete cases in a resource-constrained setting p. 259
Jideofor Okechukwu Ugwu, Kenneth Chuka Etukokwu, Victor Ifeanyichukwu Modekwe, Hyginus Okechukwu Ekwunife, Samuel Chukwudi Orjiakor, Chinedu Nnaemeka Ilokanuno, Nwanneka Ogechukwu Ugwu
Pentalogy of Cantrell (POC) is a very rare congenital anomaly and treatment has remained a challenge worldwide owing to extensive reconstructive surgeries, more so in resource-constrained settings. We, therefore, report two cases of complete POC. Both successfully underwent planned reconstructions by a multidisciplinary team. The techniques and challenges are hereby highlighted.
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Hemoperitoneum due to ruptured botryoid sarcoma of the uterus in young girl p. 262
Rajendran Ramaswamy, Elsayed Ali, Saif Saeed Ghalib, Ghazi Mukattash
Five-year-old girl presented with lower abdominal pain, pelvi-abdominal mass, and generalized abdominal tenderness. Ultrasound and computed tomography scans diagnosed heterogeneous pelvi-abdominal soft-tissue mass and a large amount of free peritoneal fluid. Laparotomy revealed hemoperitoneum and ruptured tumor at the posterior uterine wall. Histopathology report was botryoid rhabdomyosarcoma (BRMS). This case is unique due to ruptured BRMS of the uterus in early childhood, with no vaginal bleeding.
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Melanotic neuroectodermal tumor of infancy: Does enucleation alone suffice? p. 265
MM Zameer, Sanjay Rao, Vinay Chandrashekar, Ashley D’Cruz
Melanotic neuroectodermal tumor of infancy (MNTI) is a rare melanin-containing mesenchymal tumor of neural crest origin. We present a case of MNTI in a 1-year-old girl. It was managed successfully with conservative excision (enucleation).
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Cystic nephroma and its varied management scenarios: A report of two cases p. 268
Prashant Jain, Ashish Prasad, Sarika Jain, Kanta M Rahul
Cystic nephroma is a rare benign cystic neoplasm of the kidney. The preoperative diagnosis with its malignant counterparts cystic partially differentiated nephroblastoma or cystic Wilms' tumor is not easy but is important when one is considering for nephron-sparing surgery.
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Omphalomesenteric duct remnant: A rare and unusual cause of intestinal atresia p. 271
Gavin Kane, Hind Zaidan, Brice Antao
Intestinal atresia is generally considered to result from mesenteric vascular disruptions during fetal life. This report describes an unusual case of ileal atresia, resulting from an omphalic ring closure anomaly and an omphalomesenteric duct (OMD) remnant. A 2-day-old male neonate presented with bilious vomiting and abdominal distension. At laparotomy, the distal portion of the atretic ileum was entrapped within the umbilical ring, causing volvulus of the proximal dilated atretic bowel around the fixed distal bowel at the omphalic ring. This case report supports OMD remnants as a rare, potential cause of jejunoileal atresia.
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Spontaneous necrosis in a type I sacrococcygeal teratoma p. 274
Charu Tiwari, Nilesh Nagdeve, Rajendra Saoji
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Novel method of managing residual rectal spur, post modified duhamel's operation p. 276
Aniruddha D Bhagwat, Pranav Jadhav, Dhananjay Vaze
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