Surgery for children with Crohn's disease: indications, complications and outcome.

BACKGROUND: Outcome data for surgery in paediatric Crohn's disease are limited. We report 10 years' experience at a regional paediatric gastroenterology centre. METHODS: Children undergoing surgery for Crohn's disease (January 2002-December 2012) were identified from an onsite patient register. Patients were followed until transition to adult services. Data were obtained from medical records and are expressed as median (range). RESULTS: Sixty-nine children, aged 13.8 years (6.3-17.0) at diagnosis, were included. 42 were male (61%). Follow-up was 1.8 years (27 days-6 years). Surgery followed diagnosis by 9 months (0 days-7 years). 52 children (75%) received thiopurines and 5 (7%) anti-TNF (tumour necrosis factor) therapy preoperatively. 58 (84%) underwent intra-abdominal surgery (40 right hemicolectomy, 8 stoma formation, 2 subtotal colectomy, 2 small bowel resection, 6 other) and 10 (14%) underwent surgery for perianal disease. The commonest indications for intra-abdominal surgery were stricturing disease 35 (60%) and unresponsive luminal disease 13 (22%). There were 13 (22%) early, and 5 (8.6%) late, complications following intra-abdominal surgery. Nine children had disease relapse, five required further surgery. Height SD scores (SDS) did not increase between diagnosis; -0.5 (-3.4-2.1) and most recent follow up; -0.4 (-3.0-1.1). Body Mass Index (BMI) SDS increased from -1.0 (-6.3-1.5) to -0.3 (-3.3-2.0) (p<0.05). CONCLUSIONS: Surgery was associated with a 22% early complication rate and a 15% risk of relapse. 21% of patients required a second unplanned intra-abdominal procedure. Surgical intervention was associated with an increase in BMI SDS, but not in height SDS.

Scope and feasibility of operating on the neonatal intensive care unit: 312 cases in 10 years.

PURPOSE: To report the scope, feasibility and learning experience of operating on neonates on the neonatal intensive care unit (NICU). METHODS: (1) Review of all NICU operations performed by general neonatal surgeons over 10 years; (2) 6-month prospective comparison of procedures performed in NICU or operating room; (3) structured interviews with five surgeons with 1-13 years experience of operating on NICU. RESULTS: 312 operations were performed in 249 infants. Median birth weight was 1,494 g (range 415-4,365), gestational age 29 weeks (22-42), and age at operation 25 days (0-163). Nearly half (147) were laparotomy for acute abdominal pathology in preterm, very low birth-weight infants There were no surgical adverse events related to location of surgery. Surgeon satisfaction with operating on NICU for this population was high (5/5). Several factors contribute to making this process a success. CONCLUSIONS: This is the largest reported series of general neonatal surgical procedures performed on NICU. Operating on NICU is feasible and safe, and a full range of neonatal operations can be performed. It removes risks associated with neonatal transfer and is likely to reduce physiological instability. We recommend this approach for all ventilated neonates and urge neonatal surgeons to operate at the cotside of unstable infants.

Trans-anastomotic tubes reduce the need for central venous access and parenteral nutrition in infants with congenital duodenal obstruction.

PURPOSE: To determine the effect of trans-anastomotic tube (TAT) feeding on outcome following repair of congenital duodenal obstruction (CDO). METHODS: Retrospective comparative study of all infants with CDO over 10 years. Data are median (range). Mann-Whitney U test and Fisher's exact test were used. RESULTS: Of 55 infants with CDO (48 atresia, 7 stenosis), 17 were managed with a TAT, 38 without. Enteral feeds were commenced earlier in infants with a TAT compared to those without (TAT 2 days post-repair [1-4] vs. no-TAT 3 days post-repair [1-7]; p = 0.006). Infants with a TAT achieved full enteral feeds significantly sooner than those without (TAT 6 days post-repair [2-12] vs. no-TAT 9 days post-repair [3-36]; p = 0.005). Significantly fewer infants in the TAT group required central venous catheter (CVC) placement and parenteral nutrition (PN) than in the no-TAT group (TAT 2/17 vs. no-TAT 28/38, p < 0.0001). There were six CVC-related complications (5 infections, 1 PN extravasation) and four TATs became displaced and were removed before achieving full enteral feeds. One infant with a TAT with trisomy 21 and undiagnosed Hirschsprung disease developed an anastomotic leak and jejunal perforation requiring re-operation. CONCLUSIONS: A TAT significantly shortens time to full enteral feeds in infants with CDO significantly reducing the need for central venous access and PN.

Outcome of childhood ulcerative colitis at 2 years.

AIM: Ulcerative Colitis (UC) has an incidence of 1.4 per 100,000 in childhood. There is a paucity of data regarding outcome particularly with the increased use of early immunosuppression. This study reviews outcome at 2 years in a cohort with UC referred to a single centre. METHOD: Patients were recruited on the basis of a diagnosis made between 2000 and 2003 as a consecutive cohort. All had UC according to standard clinicopathological criteria. Children with indeterminate colitis were excluded. Follow-up data was collected at 2 years by case notes review. RESULTS: Thirty-two children are reported. The median age at diagnosis was 11 years (range 2-16). All were treated with corticosteroids and 5-ASA derivatives at diagnosis. The majority of patients (94%, 30/32) received more than one course of steroids. By 2 years azathioprine use was high with 75% (24/32) of patients on treatment for steroid-dependent disease. There were 6 extra-intestinal manifestations and 8 disease related complications occurring in 12 patients (38%). The colectomy rate was 9% (3/32) for unresponsive disease. CONCLUSION: There is a high need for Azathioprine in childhood UC. Colectomy rate at 2 years was around 10%. Extra-intestinal manifestations and disease related complications are common.

A new complication of the Malone antegrade continence enema.

The Malone antegrade continence enema now commonly known as the ACE procedure was described originally for the treatment of intractable fecal incontinence in 1990. Since this time it has been used widely in many centers for the treatment of constipation and faecal soiling in both adult and pediatric practice. This case report describes a previously unreported complication of an ACE that developed in a disused appendicocecostomy in a child.

The physiology of continence: idiopathic fecal constipation and soiling.

The anatomic and functional elements contributing to fecal continence and defecation are explored. The high incidence and importance of chronic idiopathic constipation as an entity in itself and a contributing factor to fecal incontinence are emphasized, and its management is discussed. The importance of a proper clinical history and examination is highlighted, and investigative modalities including radiology, transit studies, and anorectal manometry are described and placed in the context of practical clinical management.

Sleeve thrombus fibrosis causing neck pain.

BACKGROUND: Central venous catheter occlusion due to thrombus formation is the most common cause for malfunction of long-term indwelling catheters. The exact pathology and pathogenesis of this common complication is misunderstood because of an array of terms being used interchangeably in the literature. This article identifies the pathogenesis and symptoms and gives suggestions for the treatment of central venous catheter-related thrombosis. METHODS: Our case report is of a girl who had a percutaneously placed left subclavian central venous line attached to her vascuport. After 9 months she developed severe pain over the catheter tubing on injection into the port. Surgical exploration of the catheter tubing in the infraclavicular region demonstrated that the pain was due to retrograde flow around the catheter within a sleeve thrombus. CONCLUSIONS: In this type of patient, all precautions should be taken to minimize the risk of thrombus formation, including considering the prophylactic use of anticoagulants.

Rectal biopsy in the investigation of constipation.

AIMS: To develop criteria to prevent unnecessary rectal biopsies in constipated children. METHODS: A retrospective review of 186 rectal biopsies from 141 children, comparing the age at onset of symptoms with the diagnosis of Hirschsprung's disease. RESULTS: All of the 17 children with Hirschsprung's disease had the onset of symptoms before the age of 4 weeks. Twenty seven children had delayed passage of meconium (more than 48 hours) of whom 10 had Hirschsprung's disease. Three children with Hirschsprung's disease were referred after the neonatal period (2 months, 11 months, and 3 years) but all had the onset of symptoms before 4 weeks of age. CONCLUSION: If the age at onset of constipation is after the neonatal period, a rectal biopsy is unnecessary.

Prenatal diagnosis of laryngotracheoesophageal clefts.

Seven infants with laryngotracheoesophageal (LTE) clefts who had abnormal prenatal fetal sonographs were reviewed retrospectively to evaluate the antenatal scan characteristics, clinical features at presentation and associated malformations. The prenatal scans demonstrated polyhydramnios, lung cysts and an absent stomach in all. The mode of delivery was by emergency lower segment cesarean section for fetal distress, in all 7 babies. The mean gestational age at delivery was 36 weeks (range 33-38) and intrauterine growth retardation was seen in 4 neonates. A combination of endoscopy, surgery and autopsy confirmed LTE cleft type 4 in 5 patients, type 3 and type 2 in a patient each. Esophageal atresia and lower pouch tracheoesophageal fistula was present in all. Agastria was seen in 4 and microgastria in 3 children. Lung abnormalities were seen in all 7 infants and they included congenital lung cysts (4 patients), absent lung lobulation (3 patients), bronchogenic cysts (2 patients), cystic adenomatoid malformation (1 patient) and bronchoesophageal fistula (1 patient). The mortality was 86% and the sole survivor had a LTE cleft type 2 which was successfully repaired. The diagnosis of LTE cleft must be considered if a prenatal scan demonstrates the triad of: (1) polyhydramnios; (2) absent stomach, and (3) presence of lung cyst. This should lead to a detailed postnatal evaluation and early diagnosis of this uncommon anomaly resulting in early counseling and suitable management.

Urea production in normal breast-fed infants measured with primed/intermittent oral doses of [15N, 15N]urea.

Urea kinetics were measured on 11 occasions in six normal, breast-fed infants aged 29-88 days. Prime and intermittent oral doses of [15N, 15N]urea with measurement of enrichment of urea in urine were used. The rate at which urea appeared in the urea pool was 265 mgN/kg per hour, 85% of which derived from endogenous production and 15% from the diet. Urinary excretion of urea was 87 mgN/kg per hour. Therefore, 60% of the urea entering the pool each day was hydrolysed by the metabolic activity of the colonic microflora and the nitrogen was made available for further metabolic interaction. The rate of urea appearance and the extent to which urea nitrogen was salvaged were greater in infants under 6 weeks than in those over 6 weeks, indicating that urea kinetics is a more active process at an early age, and slows with time. With respect to factors influencing urea kinetics, the apparently conflicting results which have appeared in the literature may be explained. The results may help explain the growth of breast-fed infants on low protein intakes.

Opsite skin closure in day case paediatric surgery: is a subcuticular suture necessary?

Opsite skin closure without skin suture was compared with subcuticular Vicryl in a randomized trial in children undergoing day-case surgery for hernia, hydrocele or undescended testis. Ninety-nine groin closures were randomized, 47 to Opsite and 52 to subcuticular Vicryl. There was no difference in the duration of operation or in the cosmetic appearance of the wounds. Complications were all minor and similar in both groups. Opsite alone is suitable as a skin closure for the groin wounds in children.

Gastric volvulus and associated gastro-oesophageal reflux.

Between 1984 and 1994, 10 neurologically normal children between 2 and 24 months were diagnosed as having gastric volvulus with associated gastro-oesophageal reflux (GOR). The common features at presentation were episodic colicky abdominal pain, non-bilious vomiting, upper abdominal distension, haematemesis, and failure to thrive. Anterior gastropexy and conservative management of GOR was curative.

The Malone antegrade continence enema.

The previously reported Malone antegrade continence enema (MACE) was used in 21 children for intractable fecal incontinence or constipation. Twelve are completely clean, and three are much improved (71% success rate). However, only four of the 21 have not had some type of complication, either minor or major. Five of the 21 now have a colostomy, and one has abandoned the procedure.

Fiber levels and disease in workers from a factory predominantly using amosite.

The Cape Boards Plant at Uxbridge produced insulation board containing amosite asbestos between 1947 and 1973 with only small amounts of chrysotile. After 1973 only amosite was used. In this study we examined lung samples from 48 workers who had been employed at the plant and who had come to autopsy. The study investigated the fiber levels against the lung pathology including amount of interstitial fibrosis and numbers of ferruginous bodies. The degree of interstitial fibrosis and number of asbestos bodies were graded and the tissues were analyzed by transmission electron microscopy and energy dispersive X-ray analysis and the fibers counted and typed. The 48 cases included 5 mesotheliomas and 14 lung cancers. The mineral analysis results were dominated by the amosite fiber levels. The amounts of chrysotile were relatively small. There were higher levels in lung cancer cases than mesotheliomas and higher levels in mesothelioma cases than those who had died from nonasbestos related diseases. Analysis of the lung tissues showed a consistent pattern of high amosite levels, which confirms the impression that amosite was the predominant form of asbestos used and also indicates that the factory had been a very dusty one.

Urea kinetics in neonates receiving total parenteral nutrition.

Urea kinetics were measured on 10 occasions in eight neonates who had not received an oral intake from birth and were maintained on total parenteral nutrition. After a prime/intermittent oral dose of 15N15N-urea over 14 hours urine was collected every three to four hours, urea isolated, and kinetics determined from the plateau level of enrichment in urea, measured by isotope ratio mass spectrometry. The total parenteral nutrition provided 393 kJ (94 kcal)/kg/day and 360 mg nitrogen/kg/day. Urea production was mean (SD) 84 (44) mg nitrogen/kg/day, or 50% of intake. Urinary excretion of urea, 39 (16) mg nitrogen/kg/day, was 40% of production. Therefore 54% of urea production was salvaged through the lower bowel, 45 (35) mg nitrogen/kg/day. It is concluded that even in infants who have never had a regular dietary intake the microflora of the lower bowel is sufficiently developed to salvage urea nitrogen for further metabolic interaction, however it is not clear whether the rate of salvage is adequate to satisfy the metabolic demand.

Urea salvage in a neonate with cloacal exstrophy.

Urea kinetics were measured in a child with congenital absence of the colon on days 15, 19, and 23 of age. Urea salvage was 5% of urea production in the first study, increasing to 79% by the third. This provides evidence that the colonic microflora play a more active part in urea salvage than the mucosa and that the establishment of an active lower ileal microflora takes over some of the metabolic functions of the intact colon.