Are we informing our patients correctly? Evaluation of perioperative counselling for paediatric intestinal stoma formation: a national study.

PURPOSE: Adequate preoperative information is known to improve patient outcomes. We aimed to evaluate perioperative education for paediatric patients and families undergoing intestinal stoma formation. METHODS: UK paediatric surgery centres were invited to recruit patients aged 6-16 years with a stoma in situ or reversed within the last 2 years. Patient and parent questionnaires were posted for potential participants. RESULTS: Eighty-three patient/parent dyads returned questionnaires. Median age was 11.5 years (range 4.1-17.8), with 48% (n = 40) formed electively. Parents rated how well-informed they felt perioperatively out of 10 (0 poorly, 10 highly informed). Parents were better informed about surgical issues and stoma care than psychological and social impacts (7.45 vs 6.11; p < 0.0001). 54% reported surgical complications but significantly fewer patients listed these amongst the worst things about having a stoma (24.4%) when compared with psychosocial issues: distress from bag leaks (90.8%; p < 0.0001), self-consciousness (54.1%; p = 0.0001), and restricted activity (40.2%; p = 0.03). CONCLUSION: Parents felt well-informed for medical and practical aspects but less well-informed of psychological and lifestyle impacts of having a stoma. Surgical complications were less important to patients than the impact on daily life. Increased psychosocial information would enable families to be better prepared for life with a stoma.

A large single-center experience in management of pediatric intussusception.

BACKGROUND: The objective of this study was to present the results of a comprehensive single-center study of the management and outcome of intussusception over a 10-year period and to review the recent literature. METHODS: A retrospective analysis was carried out of all children less than 16 years old, admitted with intussusception to our tertiary center between January 2007 and December 2016. Air enema was attempted routinely, with primary surgery reserved for selected cases. If air enema failed, open surgery was performed. The data collected included age, enema reduction rate, need for laparotomy, detail of bowel resection, hospital stay, and complications noted. RESULTS: One hundred and ninety-one children presented with intussusception, totaling 200 admissions. One hundred and seventy-four patients (87%) underwent air enema. There was a complete reduction in 66% of these cases and a perforation rate of 1.1%. Twenty-six patients (13%) underwent primary surgery. Of the 59 patients with incomplete enema reduction, 50.8% required bowel resection while 49.2% required only manual reduction. Bowel resection was necessitated in 26% of total admissions and 61.2% of those requiring surgery. Hospital stays ranged from 3-97 days (median 7 days). There were four complications (2%). CONCLUSION: This comprehensive study reveals a higher rate of surgical intervention and bowel resection than was anticipated from selective series in published literature. Institutional variation in outcome is likely multifactorial but incomplete data make comparisons difficult.

How Secure Is the Diagnosis of Ulcerative Colitis in Children, Even After Colectomy?

OBJECTIVES: We reviewed all children who have undergone a colectomy for ulcerative colitis (UC) in our tertiary referral centre in a 12-year period to assess the rate of reclassification as Crohn disease (CD). In contrast to CD, a colectomy is considered to be definitive treatment for patients with UC. Distinguishing between the 2 can be challenging when disease is manifest only within the colon-even histological examination of a colectomy specimen may be inconclusive. Historically, the recognised "rediagnosis" rate (post-colectomy) was reported at approximately 3% to 7%. A recent study suggested that a higher rate of 13% should be expected in children. This has implications in terms of pre-operative counselling and surgical decision making. METHODS: A retrospective case-note review of all patients who underwent a colectomy for UC between 2003 and 2014 in a single paediatric tertiary referral centre was performed. RESULTS: Of the 570 children diagnosed with inflammatory bowel disease in this period, 190 were diagnosed as UC. Of these 190 cases, 29 underwent a colectomy. None of these was re-classified following histological examination of the colectomy sample. Seven out of the 29 patients (24%) were subsequently diagnosed with CD (median follow-up 7.6 years). This is significantly higher than previously reported rates (P = 0.003). CONCLUSIONS: Our data suggest that later manifestation of CD is more common than previously thought (24%). Therefore, a diagnosis of UC in children should be regarded as provisional and a potential later diagnosis of CD taken into account when considering colectomy and J-pouch formation.

Single-center review of staged restorative proctectomy for ulcerative colitis.

AIM: The purpose of this study was review results of pediatric patients undergoing total colectomy and restorative surgery for ulcerative colitis (UC) in a regional pediatric surgical center. METHODS: A retrospective case note analysis of consecutive patients undergoing colectomy for UC between 1995 and 2014 was performed. Early complications were defined as occurring within 30 days of surgery. Data was expressed as median (range). RESULTS: Of 39 children who had colectomy (59% female), 21 J-pouch procedures (20 three-staged) were undertaken at our center at median age 13 years (6-17 years). Length of stay (LOS) after colectomy was 8 days (6-21) with five early and eight late complications. LOS after J-pouch ileo-anal anastomosis was 7 days (5-19 days) with two early and three late complications. After ileostomy closure LOS was 6 days (4-16 days) with three early and two late complications. The most frequent complication following each surgical stage was bowel obstruction. There was only one pouch loss, which was secondary to development of Crohn's disease (CD), and three other patients with J-pouches also subsequently developed CD 1.5 to 10 years after their original colectomy but retained their pouch. Bowel frequency at 1-2 years post J-pouch in 14 patients was a median of 4 per 24 h (2-7 per 24 h). CONCLUSION: When all three postoperative stages were considered, two thirds of patients developed complications resulting in 15 additional visits to the operating theater. One in five patients subsequently developed CD after J-pouch. This should be carefully considered during pre-operative counseling. TYPE OF STUDY: Retrospective Study. LEVEL OF EVIDENCE: Level IV.

Pedicled jejunal interposition for long gap esophageal atresia.

BACKGROUND/PURPOSE: Long gap esophageal atresia (LGEA) represents 10% of all esophageal atresias but can be complex to manage. Jejunal interposition (JI) has been recommended as the operative management of choice when esophageal ends cannot be opposed. We report our experience using the pedicled jejunal interposition technique with comparison to patients undergoing primary repair of LGEA. METHODS: This was a retrospective analysis of all patients managed for LGEA at our unit between 2003 and 2017 with comparison between pedicled jejunal interposition and primary repair. RESULTS: 10 patients were treated with pedicled jejunal interposition and 9 patients underwent primary repair (including one Foker procedure performed elsewhere). Patient demographics and short term outcomes were similar between the two groups, but less anastomotic stricturing and gastroesophageal reflux were observed in the JI group. CONCLUSION: This is the largest published series of pedicled jejunal interposition from the UK. Our results support continued use of this procedure with good long term graft function. It is our recommendation that pedicled interposition grafts are used in all patients requiring extensive dissection or tension to achieve opposition for primary repair. TYPE OF STUDY: Retrospective study. LEVEL OF EVIDENCE: Level III.

Incidence and Clinical Associations of Childhood Acute Pancreatitis.

OBJECTIVES: To establish the UK incidence and clinical associations of acute pancreatitis (AP) in children aged 0 to 14 years. METHODS: Monthly surveillance of new cases of AP in children under 15 years of age through the British Pediatric Surveillance Unit conducted from April 2013 to April 2014 (inclusive) followed by 1-year administrative follow-up for all valid cases. RESULTS: Ninety-four cases (48 boys) fulfilled the diagnostic criteria. The median age at diagnosis was 11.2 years (range 1.3-14.9). White children accounted for 61% of the cases compared with 28% from Asian and 5% from African ethnicities. Pakistani children accounted for 18 of 26 (69%) Asian patients and 19% of the total cohort. The incidence of AP in children in the United Kingdom was 0.78 per 100 000/year (95% confidence interval [CI] 0.62-0.96). The incidence in Pakistani children (4.55; 95% CI 2.60-7.39) was sevenfold greater than white children (0.63; 95% CI 0.47-0.83). Of the 94 cases, 35 (37%) were idiopathic; other associations were: drug therapy, 18 (19%); gallstones, 12 (13%); hereditary, 7 (7%); organic acidemias, 7 (7%); anatomic anomalies, 5 (5%); viral infections, 3 (3%); systemic diseases, 2 (2%); and trauma 1 (1%). The most common drug associations were asparaginase (28%), azathioprine (17%), and sodium valproate (17%). CONCLUSIONS: Although still relatively uncommon in the United Kingdom, on average there is >1 case of childhood AP diagnosed every week. The associations of AP have changed significantly since the 1970-80s. Overrepresentation of Pakistani children is worthy of further investigation.

Survival in a 2-year-old boy with hemorrhage secondary to an aortoesophageal fistula.

Aortoesophageal fistula (AOF) in children with no history of cardiac disease or trauma is extremely rare, and survival is even rarer. We present a case of a 2-year-old boy who presented with massive hematemesis secondary to a primary aortoesophageal fistula and was found at endoscopy to have a submucosal hematoma. He then suffered a cardiac arrest with further catastrophic bleeding only controlled by a Sengstaken-Blakemore tube. Subsequent cardiopulmonary bypass was instituted and allowed direct repair of the fistula. He was discharged home 4 weeks later. The etiology of the fistula remains unknown.

Rectal duplication cyst: a combined abdominal and endoanal operative approach.

Rectal duplication cysts are rare, comprising <5% of all gastrointestinal duplications. Early excision is the treatment of choice and a number of surgical approaches have been described. We present a 3-week-old infant with a 3 cm cyst that was excised using a previously unreported combined abdominal and endoanal approach.

Postnatal rupture of an antenatally diagnosed choledochal cyst: first case report.

UNLABELLED: A 7-mo-old girl with an asymptomatic antenatally diagnosed choledochal cyst was referred to the regional paediatric surgical centre for advice on management. Following further radiological investigation and discussion with the supra-regional centre, a date for elective surgery was arranged. However, in the interim, she was admitted as an emergency following rupture of the cyst. She made an uneventful recovery following urgent laparotomy. CONCLUSION: We recommend prompt paediatric surgical referral following antenatal diagnosis of a presumed choledochal cyst. Delay in surgery may permit expansion and rupture of the cyst, even after a period of asymptomatic stability.

Azygos lobe associated with esophageal atresia: a trap for the unwary.

Presence of an associated azygos lobe may cause problems during thoracotomy on the right side for esophageal atresia. Awareness of the anomaly allows its recognition and appropriate management.

Colostomy for treatment of functional constipation in children: a preliminary report.

OBJECTIVES: Surgery is indicated in very few children with intractable functional constipation. A number of operations have been described with unpredictable outcome and significant morbidity. The authors present a series of 10 children who underwent a Hartmann procedure with end colostomy formation. METHOD: Preoperative management, in addition to maximum conservative measures, included psychologic referral, rectal biopsy, transit studies, and contrast enemas. A standard Hartmann procedure was performed with on-table rectal washout, formation of a proximal sigmoid colostomy, limited anterior resection of hypertrophic proximal rectosigmoid, and oversewing of the rectal stump. RESULTS: The series includes 10 pediatric patients (4 female, 6 male), in whom constipation was first reported at a median age of 3 years (range, 2 months-7 years) and surgical referral was made at 8 years (range, 1-14 years). Surgery was performed at a median age of 9.5 years (range, 2-15 years), and the median postoperative stay was 5 days (range, 4-9 days). Complications occurred in four patients (transient mild rectal discharge in 2, stomal prolapse in 1, and an unrelated small bowel obstruction in 1 patient with an additional Mitrofanoff stoma). Median postoperative follow-up was 31 months (range, 9-56 months), and the children and parents were all completely satisfied with the stoma. CONCLUSION: Colostomy formation is a potential surgical option for severe functional constipation with low associated morbidity and high patient satisfaction.