Management of intussusception in children: A comparative study of hydrostatic reduction with saline under ultrasound guidance versus laparoscopic assistance.

Introduction: Intussusception is a common cause of intestinal obstruction in infants and children. Ultrasound-guided hydrostatic reduction (USGHR) with saline is considered the gold standard with a success rate of more than 90%. Hydrostatic reduction with laparoscopic assistance has its own advantage of direct visualisation, assessment of bowel vascularity and controlled distension. The choice of procedure depends on available resources and surgeon's preference. This study aims to compare the outcomes of the two methods, i.e., laparoscopic-assisted hydrostatic reduction (LAHR) and USGHR under general anaesthesia (GA). Materials and Methods: This was a prospective study carried out at two different centres over a 3-year period. All patients of intussusception were managed by either hydrostatic reduction with saline under ultrasound guidance or hydrostatic reduction with laparoscopic assistance. Both the procedures were done in operation theatre under GA. The operating time and amount of fluid used for reduction were noted. Results: There were 27 patients in Group 1 (USGHR) and 20 patients in Group 2 (LAHR). The two groups were similar in terms of demographic parameters. The various outcomes such as number of attempts for reduction, fluid required for reduction, time to start oral feeds, complication and length of stay were similar in both the groups. The mean operating time for Group 1 was 19.4 ± 4.5 min and for Group 2 was 34.9 ± 4.8 min (P < 0.001). Conclusion: Both the procedures fare equally in terms of outcome except mean operating time, therefore, LAHR is a good alternative to USGHR in resource-poor nations where logistics of intraoperative ultrasound may not be present.

Assessing the Feasibility of Predicting Intermediate Outcomes of Esophageal Atresia Using Okamoto Classification: A Prospective Study.

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.

Minimal access surgery of corrosive and thermal strictures of the foregut.

Background and Aim: : Conventional surgery for caustic/thermal strictures (CS/TS) entails considerable trauma, which may be mitigated by minimal access surgery (MAS). Experience with its use in CS/TS is both heterogeneous and limited, hence, warrants a comprehensive review. Methods: : Medical literature/indexing databases were systematically searched for pertinent articles published in English, from 1990 to 2021, and analysed. Results: : Fifty relevant articles, pertaining to over 200 patients, were found. They showed that MAS is feasible in CS/TS management. It reduces the access damage in chest and abdomen whilst facilitating resection or bypass of the affected gut segment through different combination of operations, sequence of steps, conduits and routes. The procedures range from completely minimal access to hybrid ones, with reduced complications and faster recovery. Hybrid procedures prove as expeditious as open ones. Conclusions: : MAS proves efficacious in restoring alimentary continuity in corrosive/thermal strictures of the foregut.

Mesenteric Injury in Blunt Abdominal Trauma in Children: Is Early Surgical Intervention Need of the Hour?

Objective: There is an increasing trend of observational, nonsurgical management of abdominal injuries in children. We analyzed the feasibility and outcome of our management protocol in managing cases of the mesenteric injuries in blunt trauma abdomen in pediatric age group. Methods: Single-center retrospective analysis of pediatric trauma case records from July 2018 to March 2020 was performed. Results: Thirty-four cases of blunt abdominal trauma of whom 13 had mesenteric injuries were reviewed. The male-to-female ratio was 2.2:1, and the mean age was 9.11 ± 4.90 years. Mesentery of the small bowel was the most commonly injured segment and treatment consisted of repair of mesenteric tear, resection, and anastomosis with or without stoma formation. Conclusions: Isolated mesenteric injury of all grades should be managed either with diagnostic laparoscopy or laparotomy irrespective of hemodynamic status.

When Fryn met Edward: Two rare syndromes in a single patient.

A neonate born at our centre was diagnosed as Fryns Syndrome ie congenital diaphragmatic hernia with facial dysmorphism and distal limb anomalies, which is a rare disorder with only a few hundred cases reported till date.With high clinical index of suspicion and further evaluation, the diagnosis was confirmed. The baby was initially stabilized and later underwent repair of the diaphragmatic hernia. Despite best measures, the baby could not be salvaged. When severe, this can be lethal and diagnosis can only be made after autopsy. However, with early suspicion, better modalities of investigations available and improved NICU care, these babies can be salvaged. We report a case of Fryns Syndrome who was incidentally found to have Edward Syndrome as well. Such an extremely rare combination is yet to be reported in medical literature.Also with updated genetic studies, better diagnostics and treatment options coming up in future, there are chances to improve the survivability of these babies. It is prudent to document all such cases to aid in better understanding of the disease process.

Common cloaca: An uncommon entity posing diagnostic and management challenges: Our experience with nine cases.

BACKGROUND: Cloacal anomalies are the severest and most complex of all anorectal malformations (ARMs). They require careful evaluation and meticulous surgery tailored to suit each variant. We present our experience in a series of nine cases. METHODS: This includes a retrospective review of 9 cases of cloaca managed at a tertiary care centre between 2015 and 2019. RESULTS: Associated anomalies were seen in 44% cases. The definitive surgery was performed at a mean age of 15.2 months (10 months-19 months), the definitive surgery being rectal separation with total urogenital mobilisation. The common channel as measured during panendoscopy was up to 3 cm in 7 patients (78%), and only 2 patients had a common channel of more than 3 cm (22%). Of the 34 procedures that these nine patients underwent, there were four complications (12%). The median follow-up period after stoma closure was 18 months (5-32 months), and the mean age at last follow-up was 38 months (22-48 months). Five children (63%) had spontaneous voiding and remained dry in the intervening period. Three patients (37%) had poor urinary stream with dribbling and high postvoid residue requiring clean intermittent catheterisation. Six patients had faecal soiling (66%); four had daily soiling; and two had occasional soiling. Four patients had constipation (44%). Seven patients (77%) required daily enemas for bowel evacuation and to remain dry. CONCLUSION: Cloacal anomalies are rare and complex ARMs. Satisfactory urinary and bowel continence rates can be achieved even in these complex anomalies.

Klippel Feil syndrome with crossed fused renal ectopia with pelviureteric junction obstruction: A rare association.

An 11-year-old female patient presented with congenital spinal deformity with a history of occasional pain in the right flank for a duration of six months. On evaluation, she was diagnosed to be a case of type III Klippel Feil syndrome (KFS) with crossed fused renal ectopia (left to right) and pelviureteric junction obstruction (PUJO) of the right moiety. The patient underwent successful pyeloplasty of the right moeity. To the best of our knowledge, this is the first reported case in the literature with a combination of KFS, crossed fused renal ectopia, and PUJO.

Comparison of outcomes between 2 week versus 4 week stenting in pediatric pyeloplasty-A single centre observational study.

OBJECTIVE: To compare the surgical outcomes, improvement in renal function and complications between early stent removal (2 weeks) and late stent removal (4 weeks) after pediatric open pyeloplasty. METHODS: A total of 72 open pyeloplasty were included in the study. Forty-three underwent late stent removal (Group 1) and 29 underwent early stent removal (Group 2). Pre-operative and post-operative follow-up data were compared to see the effect of early stent removal on the postoperative drainage pattern at 6 months after surgery and improvement in split function of affected kidney. The complications between the two groups were also compared. RESULTS: Both the groups were matched with respect to age, sex, side and antero-posterior diameter of pelvis. Pre-operative mean split function in Group 1 was 42% (26%-54%) while it was 39% (19%-42%) in Group 2 (p=0.37). Postoperative improvement in drainage pattern was seen in 69 out of 72 (96%) patients, 41 out of 43 (95%) in Group 1 and 28 out of 29 (97%) in Group 2. Improvement in split function occurred in 35 of 38 (97%) in Group 1 and 23 of 26 (88%) patients in Group 2 (p=0.51). Complications were seen in nine out of 72 (12.5%) patients. Incidence of complication in Group 1 was 16% (7/43) and Group 2 was 7% (2/29), and relative risk was 2.36. CONCLUSION: A shorter duration of double J stenting is as effective as a longer stenting period in terms of surgical success outcomes and improvement in split renal function along with a decreased risk of stent related complications.

First-Year follow-up of Newborns Operated for Esophageal Atresia in a Developing Country: Just Operating is not Enough!

PURPOSE: To identify complications, their incidence and risk factors for their occurrence in patients of esophageal atresia (EA) in the 1st year after discharge following surgery. MATERIALS AND METHODS: Cases of EA discharged after surgical intervention in the period of July 2011-July 2013 were considered a cohort. All data regarding demographics, investigations, surgical procedure, outcome, and follow-up were recorded. RESULTS: Seventy-six such patients were discharged in the study period, six of whom were lost to follow-up, and hence, seventy patients were included in the study. Of these 70, 48 (69%) had esophageal continuity restored (46 EA + tracheoesophageal fistula [TEF]; 2 pure EA), while 22 (31%) had been diverted (3 pure EA; 8 EA + TEF following major leak; 11 long gap EA + TEF). Risk of developing any complication (except death) was 48/70 (68%; 95% confidence interval [CI] = 57.4-79.7). Twenty-six of 48 patients with esophageal continuity restored, demonstrated narrowing on contrast study (54%; 95% CI = 39.5-68.7) but only 18 of these 48 (37.5%) had dysphagia. Thirty-one of seventy had an episode of lower respiratory tract infection (LRTI) (44.2%; 95% CI = 32.3%-56.2%). Poor weight gain was observed in 27/70 (37%), and this was significantly common in diverted patients (63% vs. 25%; P = 0.009). Twenty-one of total 70 (30%) patients died within the 1st year following discharge. CONCLUSIONS: Sixty-eight percent of cases developed some complication, while 30% succumbed within the 1st year of life following discharge. The common complications were stricture, LRTI, and poor weight gain. All of these were common in diverted patients.

Influence of Interstitial Cells of Cajal in Congenital Ureteropelvic Junction Obstruction.

BACKGROUND: The etiopathogenesis of congenital ureteropelvic junction obstruction (UPJO) has been inconclusive in spite of the numerous studies carried out to find the possible causative factor. The results of different studies have been conflicting and contradictory. It has been postulated that the interstitial cells of Cajal (ICC) are the pacemaker cells located in the ureteropelvic junction (UPJ) and regulate the peristalsis in this region. Paucity of these cells may be one of the causative factors for congenital UPJO although there is no clear consensus on this issue. Therefore, the present study has been carried out to ascertain the role of ICC as one of the possible etiological factors for congenital UPJO. The aim of this study is to first identify the presence of ICC at UPJ, second to compare the average number of ICC in congenital UPJO with a control population without UPJO, and third to ascertain whether any correlation exists between the number of ICC and postoperative improvement in function of the affected kidney. MATERIALS AND METHODS: A total number of 30 patients who underwent dismembered Anderson-Hynes pyeloplasty for congenital UPJO between June 2016 and November 2017, were compared with seven controls who underwent nephroureterectomy for various other reasons. The specimen was subjected to immunohistochemistry (IHC), and a quantitative comparison was made for the ICC between cases and controls. The preoperative and postoperative function was evaluated by renal diuretic scintigraphy. RESULTS: The disease was more common among males in the ratio of 6.5:1, and there was a predominance of the left-sided involvement. In the studied cases, the average number of ICC seen for every high-power field (hpf) was 4.86 ± 0.76/hpf, whereas in control it was 11.74 ± 0.86/hpf (P = 0.04). The postoperative outcome, as measured by the improvement in split renal function, did not have any correlation with the number of ICC. CONCLUSION: The ICC are present at the UPJ and can be detected by immunohistochemistry due to their CD117 positivity. These cells are significantly low at this site in cases of congenital UPJO when compared to controls without any obstruction. The number of ICC bears no correlation to the postoperative improvement in function.

Persistent Mullerian Duct Syndrome: A rare clinical entity.

Persistent Mullerian duct syndrome(PMDS) is a rare clinical entity in the wide spectrum of disorder of sexual differentiation (DSD), often termed as intersex disorder.

Mucopolysaccharidosis type I disguised as rickets.

A 16-year-old boy with widening of the large joints of the extremities and bilateral genu valgum had been extensively treated with oral vitamin D, with little clinical benefit. A diagnosis of vitamin D-resistant rickets was considered initially but a thorough clinical examination and skeletal survey was suggestive of mucopolysaccharidosis. The diagnosis was confirmed biochemically and subtype classification pointed toward the type I variety of the storage disorder. Absence of mental retardation is very unusual in mucopolysaccharidosis type I, which itself is an uncommon clinical entity. This particular disease can be misdiagnosed as vitamin D-resistant rickets in the absence of thorough systemic examination and an attentive look at the skeletal surveys. Spondyloepiphyseal dysplasia is another close differential of mucopolysaccharidosis and it should be ruled out in all cases of suspected spondyloepiphyseal dysplasia.

Malignant systemic hypertension, encephalopathy and bradycardia following splenectomy for hereditary spherocytosis.

An 8-year-old girl suffering from hereditary spherocytosis underwent splenectomy for chronic severe anaemia. Surgery was uneventful and the patient had a good early postoperative recovery. On the third postoperative day, however, she developed severe headache with associated abnormal movements of upper limbs and nystagmus. She had a heart rate of 50 bpm and a blood pressure of 180/110 mm Hg. She was managed with triple antihypertensives, antiepileptics and sedatives. She recovered slowly over 2 weeks and is fine at 5 months follow-up.

Haemorrhagic pericardial effusion in type I Gaucher's disease.

Gaucher's disease is a rare lysosomal storage disorder. Excess accumulation of glucosylceramide predominantly occurs in abdominal viscera. Cardiac involvement is rare, though they generally represent as restrictive cardiomyopathy and pericarditis. Our case, a 19-year old boy initially presented with hepatosplenomegaly but later diagnosed with haemorrhagic pericardial effusion. Enzyme supplementation gives successful outcome but our case was managed symptomatically with pericardiocentesis.