Manasvi Upadhyaya

Primary hepatic sarcomas in children—a single-center experience over 19 years

BACKGROUND/PURPOSE Primary hepatic sarcomas are rare and account for about 13% of primary hepatic neoplasms. There are few reported series of pediatric hepatic sarcomas, and the aim was to review our experience. METHODS A retrospective analysis of cases managed from 1988 to 2007 by the pediatric liver unit in Birmingham, UK, was conducted. RESULTS Nineteen children were identified. These presented with sudden abdominal pain (n = 6), obstructive jaundice (n = 3), incidental mass (n = 3), and chronic pain/distension (n = 3). Vascular involvement was identified in 3, and 6 had pulmonary metastases. Three patients had primary resection, and 3 only a biopsy. Thirteen had a biopsy followed by chemotherapy and resection. Surgery included extended hepatectomy (n = 11), hepatectomy (n = 3), and nonanatomical resections (n = 2). There was 1 major intraoperative complication. Median inpatient stay was 7 days. One biliary leak developed 4 weeks postoperatively. Five of the 16 patients who underwent resection of the primary tumor died. Eleven were alive at a median follow-up of 3 years. CONCLUSION This is a challenging group of patients. Local control remains pivotal to successful treatment. Good results can be achieved in a specialist center with multidisciplinary approach.

A UK wide cohort study describing management and outcomes for infants with surgical Necrotising Enterocolitis.

The Royal College of Surgeons have proposed using outcomes from necrotising enterocolitis (NEC) surgery for revalidation of neonatal surgeons. The aim of this study was therefore to calculate the number of infants in the UK/Ireland with surgical NEC and describe outcomes that could be used for national benchmarking and counselling of parents. A prospective nationwide cohort study of every infant requiring surgical intervention for NEC in the UK was conducted between 01/03/13 and 28/02/14. Primary outcome was mortality at 28-days. Secondary outcomes included discharge, post-operative complication, and TPN requirement. 236 infants were included, 43(18%) of whom died, and eight(3%) of whom were discharged prior to 28-days post decision to intervene surgically. Sixty infants who underwent laparotomy (27%) experienced a complication, and 67(35%) of those who were alive at 28 days were parenteral nutrition free. Following multi-variable modelling, presence of a non-cardiac congenital anomaly (aOR 5.17, 95% CI 1.9–14.1), abdominal wall erythema or discolouration at presentation (aOR 2.51, 95% CI 1.23–5.1), diagnosis of single intestinal perforation at laparotomy (aOR 3.1 95% CI 1.05–9.3), and necessity to perform a clip and drop procedure (aOR 30, 95% CI 3.9–237) were associated with increased 28-day mortality. These results can be used for national benchmarking and counselling of parents.

Long-term efficacy of a tongue tie service in improving breast feeding rates: A prospective study

BACKGROUND Breast feeding rates in England at 3months of age are approximately 17% for exclusive breast-feeding and 55% for breast-feeds supplemented with formula. Tongue-tie (TT) in infants is cited as a significant cause of difficulty with maintaining breast-feeding. Early treatment and support can improve breast-feeding and allow infants to benefit from the many long-term benefits of breast-feeding. Our aim was to determine BF rates in infants 3months after attending our tongue-tie clinic (TTC). METHODS Institutional ethical approval and study approval were obtained. Patients attending the TTC from May to July 2016 were included. Telephone contact was made 3months postprocedure to establish current behaviour. Symptom resolution was recorded as complete resolution (CR), moderate resolution (MoR), or minimal resolution (MiR). Feeding pattern was either exclusively BF or combined breast and formula feeds or exclusively formula fed. RESULTS 100 infants were included with complete follow-up data on 87 (87% response rate). Median age at release of TT was 17 (2-88) days without any recurrence. Status at 3months was CR (n=70, 80%); MoR (n=13, 15%), and MiR (n=4, 5%). 43 (49%) were exclusively BF, 36 (41%) were supplementing BF with some formula (2/3 by choice and 1/3 owing to insufficient milk production), and 8 (10%) were using only formula milk (7 by choice and 2 owing to ongoing feeding difficulties). Of the 17 mothers still experiencing symptoms, 5 were exclusively breast-feeding, and 8 were persisting with combined feeds. CONCLUSION Infants attending our tongue-tie clinic achieved both higher exclusive breastfeeding and combined breast and bottle-feeding when compared to national breast-feeding data at 3months of age. This can facilitate the achievement of long-term breastfeeding, exposing infants and mothers to many of the associated benefits. LEVEL OF EVIDENCE 4.

Bronchoscopy as a screening tool for symptomatic tracheomalacia in oesophageal atresia

AIM Oesophagealatresia/tracheo-oesophageal fistula (OA-TOF) is associated with tracheomalacia (TM). In our institution it is routine for OA-TOF patients to undergo dynamic flexible bronchoscopy (DFB) assessing both the site of the fistula and the presence or absence of TM. We aimed to determine the value of this investigation as a screening tool to predict subsequent symptomatic tracheomalacia in these patients. METHODS All patients with OA-TOF who underwent DFB at the time of initial repair between June 2014 and November 2016 were included prospectively. The findings at DFB were recorded. Patients were grouped according to the presence or absence of TM and followed to determine which of them developed symptomatic airway problems. The sensitivity and specificity of TM at initial bronchoscopy as a screening tool for subsequent symptomatic TM were calculated. The study was given ethical approval by our institution. MAIN RESULTS Twenty-three patients were included in the study. Median follow-up was for 7 (1-27) months. Fifteen (65%) were found to have TM at their first DFB; 13 (57%) subsequently developed airway symptoms, and of these 11 had TM at initial DFB. One patient with severe TM (>90% tracheal collapse) at initial DFB was completely asymptomatic following OA-TOF repair. The sensitivity was 85%, and specificity was 60%. The positive and negative predictive values were 73% and 75%, respectively. CONCLUSIONS DFB is a useful tool in many aspects of the management of OA/TOF. However, it is not a good screening tool to predict symptomatic tracheomalacia with moderate sensitivity and a low specificity. LEVEL OF EVIDENCE Level IIb, Retrospective cohort study.

Dangerous deliveries: lessons learned during retroperitoneal specimen retrieval

Laparoscopy is now a standard technique in pediatric surgery and urology. Unique complications have been reported during port/instrument insertion and dissection, often relating to issues of visibility or working space. Complications during specimen retrieval are currently unreported. We describe our experience of 2 serious complications occurring during attempted retrieval of a specimen through a port site at the end of the laparoscopic procedure.

10 Years' Experience in Balanitis Xerotica Obliterans: A Single-Institution Study

Introduction Balanitis xerotica obliterans (BXO) is uncommon in children. Diagnosis of the condition is almost always clinical and supported by histology. Our aim was to evaluate the outcomes of children undergoing circumcision for BXO and explore the correlation between surgical and histological findings. Materials and Methods A 10‐year retrospective review (2007‐2017) of all children, aged 16 and less, undergoing circumcision at a tertiary teaching hospital was conducted. Statistical analysis was performed using Fishers exact test. Results BXO occurred in 91/1025 (8.9%) children. The highest incidence of BXO was in the 5 to 10 age group (13.3%; p < 0.0001). The commonest symptom was foreskin scarring (62.6%). Intraoperatively, involvement of foreskin alone was seen in 26.4%, foreskin and meatus in 47.2%, and foreskin, meatus, and glans in 26.4%. Preoperatively, 24.2% of patients were prescribed steroid cream. Histologically, all patients showed microscopic changes confirming BXO. A total of 87.9% of patients only had a circumcision, and 11% required a meatal procedure along with the circumcision. Postoperatively, 19.8% of patients required a further procedure after an average duration of 5.8 months (range: 2‐12 months); the majority of whom (83.3%) were prescribed postoperative steroid cream. Conclusion Clinical correlation by surgeons has a high degree of accuracy (>90%). Meatal involvement is more common than previously reported. The use of pre‐ or postoperative steroids does not obviate the need for further surgical procedures. Patients can have recurrent symptoms 1 year following surgery, and prolonged follow‐up is necessary.

Management of pneumatosis intestinalis in children over the age of 6 months: a conservative approach

Background Pneumatosis intestinalis (PI) is an uncommon and poorly understood condition. Although it can be an incidental finding in asymptomatic individuals, it can also be secondary to life-threatening bowel ischaemia and sepsis. In premature infants, it is a pathognomonic sign of necrotising enterocolitis. There is no consensus regarding management and long-term outcome of children with PI. Aim Review of our experience of PI in children beyond the early infantile period. Methods Retrospective review of patient’s records and radiological images from 2013 to 2015. Results Eighteen patients (three girls) had radiologically confirmed PI. The median age was 4.5 years (range 8 months–13 years). Background medical conditions (number): short bowel syndrome (one), congenital heart disease (two), sickle cell disease (one), epilepsy (three), cerebral palsy (six), myotonic dystrophy (four) and peroxisomal biogenesis defect (one). Six children (33%) presented with abdominal distension, four (22%) with abdominal pain, three (17%) with bilious vomiting, two (11%) with diarrhoea and one (6%) with rectal bleeding. Two (11%) were asymptomatic. One had air in portal vein and two had pneumoperitoneum. All patients with symptomatic PI were treated conservatively with successful outcome and complete resolution of PI. None required surgical intervention. Conclusion PI in children who are not on chemotherapy or immunosuppressant appears to follow a benign course and is responsive to conservative management. In contrast to adults, portal venous gas and pneumoperitoneum do not predict the need for surgical intervention.

G253(P) The risk of developing necrotising enterocolitis in infants with congenital heart disease undergoing invasive cardiac procedures

Aim Prematurity is the most significant risk factor for developing necrotising enterocolitis (NEC). However, congenital heart disease (CHD) is also a well-recognised risk factor. Our aim was to identify the incidence of NEC in term children undergoing invasive cardiac procedures. Methods This was 5 year (January 2010 to December 2015) retrospective review of all infants admitted with CHD. Data was collected on demographics, NEC (incidence, clinical management and mortality) and invasive cardiac procedure (ICaP) defined as either open-heart surgery or cardiac catheterisation. Incidence of NEC was compared in the two groups (ICaP and non-ICaP). Institutional ethical approval was given. p value<0.05 was significant. Result 5103 infants with CHD were identified. 31.5% (n=1608) patients had an ICaP.Overall 128/5103 (2.5%) of patients developed NEC: 102/1608 in ICaP group; 26/3495 in non ICaP group, p<0.001.Median gestational age was 37 weeks (range 29 to 42). Median age at presentation was 6 weeks (range 0.4 to 104).The most common cardiac diagnoses were, hypoplastic left heart 45 (33.5%), ventricular septal defect (VSD) 16 (12%), isolated PDA 10 (7.4%).60 had multiple cardiac procedures and 42 had a single cardiac procedure, p>0.5. 52% (n=66) of patients had pneumatosis and 7% (n=9) had pneumoperitoneum. 79% (n=101) of patients were managed conservatively. 21% (27/128) required surgery: 18/27 (67%) in ICaP and 9/27 in non-ICaP group, p>0.5. Multiple cardiac procedures significantly increased the risk of needing a laparotomy, p=0.01. Intraoperatively, 44% (n=12) had a bowel perforation and rest had significant NEC. 41% (n=11) had primary resection and anastomosis; 16/27 had a stoma.Overall mortality was 7%: 5 patients died pre-operatively and 4 patients died post-operatively, however an ICaP was not a significant factor for mortality in NEC (p>0.5). Conclusion Invasive cardiac procedure is a significant factor in developing NEC in term infants. A single ICaP did not increase the risk for either needing laparotomy or mortality, but multiple cardiac procedures did further increased the risk for laparotomy in children with NEC. This information could be used for counselling and risk stratifying cardiac patients.

Short article: Buried bumper syndrome in children: incidence and risk factors.

Objective Buried bumper syndrome (BBS) is a serious complication in gastrostomy-dependent children. Many need surgical correction. On account of comorbidities, this becomes a high-risk procedure. Our aim was to review the incidence of BBS in children and to identify the risk factors. Patients and methods Retrospective review of patients’ records over 10 years, 2006–2015, was carried out. Types of tubes, operative interventions, comorbidities and records were noted. Two-tailed Fisher’s exact test was used for statistical analysis. Results A total of 535 patients were reviewed. Overall, 475 had only percutaneous endoscopic gastrostomy (PEG) and 60 had a jejunal extension with percutaneous endoscopic gastrostomy (PEG-J). Twenty-nine patients (PEG-J – 16/26; PEG – 13/26) had a total of 31 BBS episodes. The overall incidence of BBS in our study was 5.4%. The age at presentation ranged from 1 to 18 years (median 8.6 years). All had significant comorbidities (neurodevelopmental 26/29, cardiorespiratory 14/29, genetic 16/29). Overall, 27/29 had two or more comorbidities. The mean time to development of BBS was 1025±634 days. BBS was found in the second or the subsequent tube in four patients with PEGs (P<0.0004) and in 10 PEG-Js (P<0.0001). Twenty-five patients needed laparotomy. There were no postoperative deaths. Conclusion In BBS, the two significant risk factors identified were a having PEG-J and two or more previous gastrostomy insertions. Vigilance in documentation and prolonged follow-up to provide regular education to carers can reduce the incidence of this preventable complication.

Inflammatory duodenal necrosis complicating gastroschisis

Babies with gastroschisis have an increased risk of necrotizing enterocolitis (NEC) that can lead to short bowel syndrome, a long-term parenteral nutrition requirement, and its associated complications. To our knowledge, this is the first case report of recurrent duodenal ischemia and necrosis associated with gastroschisis in the absence of NEC totalis.