Delayed presentation of duodenal atresia.

Background: The most common type of duodenal atresia (DA) (Type I), also known as duodenal web or membrane can present later in infancy or early childhood if the membrane or web is fenestrated. We describe six patients with delayed presentation of DA. Materials and Methods: Retrospective review of hospital records of six patients with delayed presentation of DA due to fenestrated web managed in Paediatric Surgery Department at a tertiary care institute over a period of 2 years (January 2019 to December 2020) was done. The data of these patients were analysed on the basis of age at presentation, clinical presentation, associated anomalies, radiological findings, intra-operative findings, management and postoperative course. Results: The median age at presentation was 6.5 months (range: 1 month to 10 years). There were four males and two females. The most common presentation was emesis seen in all six patients. Two patients had Down syndrome. Associated congenital anomalies were cardiac in one patient, anterior ectopic anus in one patient and malrotation of midgut in one patient. Upper gastrointestinal contrast suggested incomplete duodenal obstruction in all patients. At laparotomy, fenestrated duodenal membrane was observed in all patients - preampullary in three patients and postampullary in three patients. Lateral duodenotomy, web excision and transverse closure was done in all six patients. The postoperative period was uneventful in all patients and mean duration of hospital stay was 9 days. Conclusion: Fenestrated duodenal webs present a diagnostic challenge to the paediatric surgeons because of delayed and variable clinical presentation. A modification of the present classification of DA has been proposed in this study which would help in better reporting of epidemiology and aid in early diagnosis of this congenital anomaly.

Do more mature preterm babies with surgical necrotizing enterocolitis predominantly develop the colonic disease?

Background: The primary aim was to scrutinize our hypothesis: "Do more mature preterm (MMP) babies with surgical necrotizing enterocolitis (NEC) predominantly develop the colonic disease and are different in their response and behaviour in comparison to exceedingly preterm (EP) babies?" Secondary outcomes were to define time taken in developing NEC, time from diagnosis to laparotomy, requirement of parenteral nutrition (PN), and ventilatory support. Methods: We defined MMP babies as ≥30 weeks of gestation and EP babies as ≤29 weeks+6 days of gestation. Inclusion criteria were all babies <37 weeks with NEC requiring surgery (called surgical NEC group). Data were collected retrospectively and analyzed using QuickCalcs. Results: Of the total, 41% (97/234) of babies underwent laparotomy between 2010 and 2019. Totally, 81% were EP and 19% were MMP babies. Pure colonic involvement was seen in 9% of EP babies in comparison to 56% in the MMP babies (p=0.0001). Involvement of only the small bowel was seen in two-thirds of EP babies in comparison to only one-third in MMP babies (p=0.01). EP cohort required PN for 82 days (median) in comparison to 17 days (median) in the MMP cohort (p=0.001). Ventilation requirement in the EP group versus the MMP group was 24 vs 9 days (median), respectively (p=0.0006). Conclusions: MMP babies predominantly developed colonic disease, whereas EP babies predominantly developed small bowel disease. EP babies required a longer duration of PN and ventilation support. This study opens a new area of research to differentiate pathogenesis and maturation patterns of the small and large bowels in babies with NEC.

Readmission within 30 days of discharge (ReAd): a quality-of-care indicator in paediatric surgery.

PURPOSE: Following a previously published 1 year audit of readmissions, this is a reaudit of our readmission rate (ReAd) in paediatric surgery, asking: is ReAd reproducible, can it be an indicator of quality of care in paediatric surgery, and can it be improved? METHOD: Prospectively collected Hospital Episode statistics were used to identify readmissions over 1 year. Patients were subdivided into emergency vs elective regarding the first admission and outcomes compared including with our previously published ReAd data. RESULTS: 2616 children (67% male) were admitted during 2016: 1398 (53%) elective and 1218 (47%) emergency admissions. The overall ReAd was 0.9%, comparable to and lower than our previously published rate of 2%. The commonest cause for readmission was appendicitis-related (22%). The emergency cohort ReAd was 1.5% (18/1218) compared to 0.4% (5/1398) in the elective cohort, 4× higher (p = 0.002). In the emergency cohort, the commonest causes for readmission were abdominal pain and perforated appendicitis. 80% of elective group readmissions were related to urological procedures. More of these required surgical intervention to treat (80% vs 22%) (p = 0.03). CONCLUSION: (1) ReAd is a reproducible and reducible quality-of-care indicator in paediatric surgery. (2) Emergency admission is a risk factor for readmission. (3) Appendicectomy was associated with the highest ReAd.

Paediatric duodenal injury complicated by common bile duct rupture due to blunt trauma: a multispecialist approach.

We report a case of late presenting duodenal perforation with common bile duct rupture secondary to blunt handlebar trauma in an 11-year-old boy. The patient presented with upper abdominal wall ecchymosis, pain and vomiting. He was discharged after 24 hours with resolving symptoms. However, the boy presented 2 days later febrile with signs of peritonitis. CT indicated duodenal perforation, which was confirmed during laparotomy where common bile duct rupture was also demonstrated. Primary repair of the duodenum was undertaken. Here, decompression was achieved with a nasogastric tube proximal to the injury and T-tube duodenostomy distally. Common bile duct repair was achieved over a biliary stent. This case represents a rare subset of duodenal injury for which there is a paucity of evidence for optimal surgical management, particularly in the paediatric setting. This operative plan will guide surgeons and junior doctors in managing complicated cases like this in future.

Systematic review and meta-analysis of appendiceal carcinoid tumors in children.

OBJECTIVE: The study's aim was to review the literature regarding past and current practices in managing incidental appendiceal carcinoid tumors and need for more procedures. METHOD: A search of MEDLINE, Embase, CINAHL, and Cochrane databases of systematic reviews was undertaken of the English language literature. The mesh terms used were "carcinoid" or "neuroendocrine," "tumour" or "tumor," "appendix," "appendicectomy," or "appendectomy," and "child," "pediatric," or "paediatric." Of the 369 articles found, 37 met the inclusion criteria. Our hospital records and pathology database identified 11 patients with confirmed histological diagnosis of appendicular carcinoids from January 1996 to December 2016. Those cases were also included in this study. RESULTS: A total of 958 cases were identified from the literature and our own experience. There were 566 females and 343 males giving us a ratio of 1.65:1. The frequency was 0.3% of appendicectomies. There was a 28-fold increase in the risk of having a positive lymph node if the tumor size was >2 cm compared with the risk of having a positive lymph node if the tumor size was ≤2 cm. There was no recurrence or mortality for those with criteria for secondary surgery, who were observed after appendicectomy compared to those that had secondary surgery. Mean follow-up was 58.6 months (4.8 years) with a range of 0-396 months (33 years). CONCLUSION: Appendicectomy alone is an adequate treatment for an appendicular carcinoid in children irrespective of size, position, lymph node, or mesenteric involvement. Post-appendicectomy investigations were found to be not helpful in this study.

Pneumoperitoneum in the Setting of Pneumatosis Intestinalis in Children: Is Surgery Always Indicated?

Aim Pneumatosis intestinalis (PI) is a condition in which multiple gas-filled cysts form within the wall of the gastrointestinal tract in either the subserosa or submucosa. The presence of pneumoperitoneum in the presence of PI can present a therapeutic dilemma. The aim of our study was to review our experience and management of this condition. Methods A single-center retrospective study of consecutive children (> 1 year) presenting with a pneumoperitoneum and evidence of PI (2009-2015). Demographics, case notes, microbiology, and imaging were reviewed. Results Seven patients were identified (four males; age range 5-14 years). Four children had global developmental delay and were percutaneous endoscopic gastrostomy or jejunostomy fed, one was immunocompromised (acute lymphoblastic leukemia). The others had encephalitis and eosinophilic gastroenteritis. One patient proceeded to an exploratory laparotomy; no perforation was identified, pneumatosis of the colon was observed, and a loop ileostomy was formed. The remaining six were managed conservatively and made nil by mouth with intravenous antibiotics commenced. Five of the six had a computed tomography (CT) scan which revealed PI and free air with no other worrying signs. One died from nongastrointestinal causes, while the remaining five had feeds reintroduced uneventfully. Conclusion Free air in the setting of PI may represent rupture of the gas cysts and not always transmural perforation. Surgery may not always be indicated and conservative management may suffice. A CT scan can be useful for excluding other intra-abdominal pathological findings and continued clinical assessment is essential.

Littre's hernia with an impalpable testis in a boy: a diagnostic dilemma.

Littre's hernia is a rare clinical entity. The presence of a Littre's hernia in a 2-year-old boy with an impalpable testis is even rarer. We describe this interesting case with a review of the literature.

Penile resurfacing for denuded penis following circumcision.

Major loss of penile shaft skin following circumcision has been rarely reported in the paediatric literature and when it occurs is usually due to the injudicious use of monopolar diathermy, infection and poor surgical technique. We report the reconstruction of a penis following complete loss of penile skin due to circumcision. We employed a split-skin graft for the glans and full-thickness graft for the shaft to achieve a more natural cosmetic appearance. This approach has not been described previously in the paediatric literature and should be considered in the unfortunate event of significant penile skin loss.

An 18 years' review of exomphalos highlighting the association with malrotation.

INTRODUCTION: This was a retrospective 18 years' review of infants with exomphalos with particular attention to its association with malrotation. MATERIALS AND METHODS: We reviewed all exomphalos cases presenting to our neonatal surgery unit, from October 1991 to September 2009. Data were presented as median and range values. Categorical data were analyzed using Fisher's exact tests and P value of ≤0.05 was considered as significant. RESULTS: Forty-two infants with exomphalos (E. major 23, E. minor 19) were treated during this period. The incidence of surgically confirmed malrotation was 45% in E. major and 31% in E. minor. CONCLUSION: The association of malrotation in exomphalos major and exomphalos minor infants is alarmingly high and supports the need to actively exclude this abnormality, either operatively or radiologically.

Neonatal Meckel's diverticulum: spectrum of presentation.

Symptomatic neonatal Meckel's diverticulum is rare. We describe 2 interesting presentations of neonatal Meckel's diverticulum with review of the literature. Our first patient presented with massive bleeding per rectum in the first week of life, which is the only case reported so far. The second case presented with milk curd obstruction in the Meckel's diverticulum. We could find only one similar case in the literature.

Twenty years' experience of steroids in infantile hemangioma--a developing country's perspective.

BACKGROUND: Hemangioma is a common vascular tumor. Though it involutes spontaneously, results are unpredictable. Steroid therapy is an effective mode of its regression. We present our experience of largest series and possible recommendations for treatment. MATERIALS AND METHODS: A total of 2398 patients were treated during the study period of 20 years. They were given oral prednisolone, intralesional triamcinolone, or combination of both as per the protocol and followed for the response. Response to the treatment was graded as excellent, good, poor, or no response. RESULTS: The male-to-female ratio was 1:2.3. In 81% of patients, hemangioma was noticed within first month of life. The commonest site of involvement was head and neck (57%). The commonest clinical presentation was discoloration and swelling. Mean age and size were 8.43 +/- 7.04 months and 23.64 +/- 20.13 cm(2). Response rate was highest for superficial type using any modality of treatment. Patients younger than 1 year showed better response (90.3%) in comparison with children older than 1 year (80.8%). The specific complications occurring were infections in 249 (12.4%), cushingoid facies and growth delay in 62 (3.1%), and hypertension in 51 (2.5%) patients. CONCLUSION: Steroid therapy either oral or intralesional as per the requirement is an easy and safe modality. Results are good to satisfactory in most patients. The complications are minimal. If treatment is needed, it should be used as a first-line therapy, especially when cost is an important concern.

Congenital diaphragmatic hernia: prognostic indices in the fetal endoluminal tracheal occlusion era.

INTRODUCTION: Management of congenital diaphragmatic hernia (CDH) in the UK now includes the possibility of fetal endoluminal tracheal occlusion (FETO) for poor prognosis fetuses. The objective of this study was to investigate the value of variables previously thought prognostic in the FETO era. METHODS: A retrospective single-centre study was performed of all infants with CDH born between January 1994 and December 2007. Fetal endoluminal tracheal occlusion was available and had been used with parental consent for fetuses with lung-to-head ratio (LHR) of 1.0 or less and a liver-up position from 2002. Univariate analysis was used to predict survival (to leave hospital) using both prenatal (eg, polyhydramnios) and perinatal variables [eg, best oxygenation index on day 1, or BOI (d1)] and their dependence tested in a logistic regression model. Data were quoted as medians (range). P < .05 was regarded as significant. RESULTS: Eighty-six infants with CDH (1994-2002, n = 35 and 2002-2007, n = 51 "FETO era") were studied. Successful FETO intervention was performed in 31 infants. Univariate analysis showed liver position, birth weight, LHR, and BOI (d1) were significant prognostic predictors (all P < .05); however, only BOI (d1) retained significance using logistic regression analysis (odds ratio, 21; 95% confidence interval, 6-74; P < .001). Best oxygenation index on day 1 was then used as a surrogate marker for outcome to test the relationship with LHR (available since 2002) and showed a significant inverse correlation (r(s) = -0.5; P < .001). There was no difference in median BOI (d1) between the FETO group and all those treated expectantly (40 [34-1046] vs 59 (23-581); P = .3]. CONCLUSION: Best oxygenation index on day 1 is the best early postnatal predictor of survival. The more recently evaluated prenatal index, LHR, has an observable relationship with BOI (d1) when it is used as a surrogate marker of outcome.

Feasibility of complex minimally invasive surgery in neonates.

The aim of this study was to assess the feasibility of complex minimally invasive surgery in neonates (and infants <5 kg). A systematic search of databases was performed. Inguinal herniotomy and pyloromyotomy were specifically excluded. Various operations were assessed for operative times, conversion rates, complications and centres performing the procedures. From 479 abstracts, 50 papers were identified describing 1,215 patients. Common operations performed were fundoplication (n = 473), operations for ovarian pathology (n = 151) and oesophageal atresia repair (n = 123). Other not-so-common operations included Kasai procedure (n = 61), Ladd's procedure (n = 55), bowel atresia surgery (n = 45) and congenital diaphragmatic hernia repair (n = 41). Mean operation times varied from 43 min for fundoplication to 209 min for Kasai procedure, with other operation times falling in between the two extremes. Likewise, conversion rates varied from 0.2% for fundoplication to 15% for congenital diaphragmatic hernia, with other conversion rates falling in between. Complications ranged from 0 to 67%. About 70-94% of the cases were performed in six pioneering centres. Fundoplication and operations for ovarian pathology have low complication and conversion rates and appear to be well suited to the average-trained paediatric laparoscopic surgeon. More complex procedures are feasible and safe, but technically demanding and need further expertise.

Dismembered pyeloplasty using double 'J' stent in infants and children.

PURPOSE: The purpose of the present study was to comprehensively assess the results of dismembered pyeloplasty in pelvi-ureteric junction obstruction (PUJO) using double 'J' ureteric stent. MATERIALS AND METHODS: Records of 61 dismembered pyeloplasties in 60 consecutive patients with PUJO who underwent pyeloplasty in a 5-year period were reviewed retrospectively. In 58 cases, a double 'J' stent was passed in an antegrade fashion during the operation. No other mode of drainage was used. Stent was not placed on three occasions. Records were reviewed for age at diagnosis, age at surgery, post-operative complication, post-operative drainage and length of hospital stay. RESULTS: There were no episodes of urinary leak or re-obstruction in the stented group. Median post-operative stay in stented patients was 2 days. The use of this stent was associated with no serious complications. There were no episodes of post-operative urinary tract infection or stent displacement. CONCLUSIONS: We recommend double 'J' stent as the safest mode of drainage in pyeloplasty in infants and children. Keeping a double-J stent across the anastomosis reduces the complications and the hospital stay in these patients.

Antegrade continence enema (ACE): current practice.

The purpose of this study was to assess current status of antegrade continence enema (ACE) procedure taking into account the recent improvement in the technique and outcome. Reviewing our record of 48 patients with ACE procedure performed between January 2002 and May 2007, we found that the underlying diagnoses were idiopathic constipation in 56%, anorectal malformation in 31%, spina bifida in 8% and Hirschsprung's disease in 4%. Mean age of operation was 10.7 years. Appendix was used as stoma in 73% of cases. Stomal stenosis requiring revision was seen in 6% of cases and continence was achieved in 92% of cases. A systematic search of database was performed for the same period. Twenty-four studies describing 676 patients were found. The mean age was 10 years and various sites used for ACE were, right side of abdomen in 71%, umbilicus in 15% and left side of abdomen in 14%. The incidence of open and laparoscopic procedures were 87 and 13%, respectively. Appendix was used for stoma in 76% procedures. Other operative modalities were retubularised colon, retubularised ileum, caecal button and caecostomy tube, etc. The mean volume of enema fluid used was 516 ml. The mean evacuation time was 42 min. Stomal stenosis requiring revision was seen in 13% of cases. Continence was achieved in 93% of cases. There has been significant improvement in the outcome during last 5 years in comparison to the outcome published in late 1990s. Advancements in techniques, better-trained stoma care nurses and better stoma appliances could have played major role in this success.

Short-segment Hirschsprung's disease, cat eye syndrome, and anorectal malformation: a unique association.

The association of Hirschsprung's disease (HD) and anorectal malformations has been reported in 2.3% to 3.4% cases. Only 2 cases have previously been published where cat eye syndrome was associated with long (but not short) segment HD. Here, we report a case where there appears to be an association among short segment HD, cat eye syndrome, and anorectal malformation, which has not previously been identified. An abnormality in chromosome 22 may be involved in the development of this association.

Haematogenous metastasis to ascending colon in a patient with hepatocellular carcinoma and autoimmune hepatitis.

The association of hepatocellular carcinoma with chronic active autoimmune hepatitis and haematogenous metastasis to ascending colon has not been reported previously in the literature. The patient was asymptomatic for colonic disease and the finding of colonic involvement was incidental on scans subsequently confirmed by colonoscopy. Usually hepatocellular tumour mass would involve the colon by direct contiguity. Owing to haematogenous metastasis, which is extremely rare, the colonic mass was discontiguous from hepatic tumour lesions. The unique presentation of this case and the differential diagnosis of hepatic tumours coexisting with colonic tumours are highlighted in this case report.

Splenoptosis complicated by a large splenic cyst: case report and discussion of combined management.

Splenoptosis is a rare condition in children. Its association with a cyst is rare. A case of splenoptosis complicated by a large cyst is reported. The child presented with features of intermittent abdominal pain, constipation and clinical examination revealed a mass apparently rising out of the pelvis. Ultrasound confirmed the mass as spleen with a 15 cm cyst in the lower pole. At surgery the cyst was marsupialised and splenopexy undertaken placing the cyst in a retro-peritoneal pouch. This is only the second report of such a case in the English literature.