Basem A. Khalil

Intestinal rehabilitation and bowel reconstructive surgery: improved outcomes in children with short bowel syndrome.

Background and Aim: Short bowel syndrome poses a great challenge to pediatric teams. Several innovative techniques in the management of total parenteral nutrition (TPN) and bowel reconstructive surgery have improved the outcomes of these children. The authors present their experience during the last decade as a specialist unit using improved techniques and multidisciplinary approaches in the management of this condition. Methods: All of the children presenting with short bowel syndrome between 2000 and 2009 were identified. Diagnosis, length of residual gut, age at definitive surgery, length of gut prelengthening, length of gut postlengthening, TPN status, and survival were recorded. Median values were calculated. Results: Twenty-seven children were identified (14 boys, 13 girls). Overall survival was 92%. Two children died. Nineteen children required bowel lengthening and 8 children had simple bowel reconstruction while on our protocol. Overall median age at definitive surgery was 12 months. Overall median residual gut length for these was 35.5 cm, whereas the median residual gut length for patients undergoing bowel lengthening was 25 cm. Postbowel lengthening, the median gut length was 90 cm. TPN data were unavailable for 2 patients. Overall, excluding the 2 patients who died and the 2 we have no TPN data on, of 23 patients, 21 (91%) are now off TPN. Conclusions: Our series shows improved results not only with survival but also in the number of patients that are off TPN. Multidisciplinary approach consisting of both medical and surgical expertise is necessary in the management of these patients. The authors advocate centralisation of short gut services to experienced centers with multidisciplinary expertise.

Autologous intestinal reconstructive surgery to reduce bowel dilatation improves intestinal adaptation in children with short bowel syndrome.

Objectives: Intestinal failure (IF) is a common consequence of neonatal small bowel pathology. In our experience, bowel dilatation is often responsible for the IF state in patients who fail to adapt despite adequate residual bowel length. The aim of the present study was to investigate the role of surgery to reduce bowel dilatation, and thus favour PN independence, for these children. Methods: Data were collected prospectively for all of the patients referred to our unit for a 7-year period (2004–2011). Eight patients (2 congenital atresia, 2 gastroschisis with atresia, 1 simple gastroschisis, 3 necrotising enterocolitis) with gut dilatation who failed adaptation despite a bowel length >40 cm were identified. Preoperatively, all patients were totally dependent on parenteral nutrition (PN). Patients were managed by longitudinal intestinal lengthening and tailoring (n = 3), serial transverse enteroplasty (n = 2), or tapering enteroplasty (n = 3). Results: Median age at time of surgery was 273 days (103–1059). Mean gut length increased from 51 (35–75) to 73 cm (45–120) following surgery (P = 0.02). Incidence of sepsis (P = 0.01) and peak serum bilirubin levels (P = 0.005) were reduced postoperatively. PN was discontinued after a median of 110 days (35–537) for 7 patients; 1 patient remains on PN 497 days after surgery. Conclusions: These data indicate that reconstructive surgery to reduce bowel diameter may be an effective technique for treating IF in patients with short bowel syndrome, without sacrificing intestinal length. We suggest that this technique may reduce the need for bowel transplantation in this group of patients.

Controlled Tissue Expansion in the Initial Management of the Short Bowel State

The treatment of short gut syndrome has evolved dramatically during the past decade. The combination of surgical techniques and medical management in the context of a multidisciplinary approach has improved the outcomes of these children. The authors describe in detail their technique of controlled tissue expansion of the bowel before lengthening procedures. Monitoring of the child and troubleshooting actions during the controlled tissue expansion program also are discussed.

It is not what you do, it is the way that you do it: impact of a care pathway for appendicitis

BACKGROUND/PURPOSE Appendicitis is the most common surgical emergency in children. However, management varies widely. The aim of this study was to assess the impact of introducing a care pathway on the management of childhood appendicitis. METHODS Data were collected prospectively for 3 successive cohorts: All patients operated for suspected appendicitis were included. The pathway was modified after interim analysis of group B data. P < .05 was significant. RESULTS Six hundred patients were included. When compared with group A, group C patients were more likely to receive preoperative antibiotics (P < .0001), undergo formal pain assessment (P < .0001), and be operated before midnight (P = .025). There was a significant decrease in readmission rates from 10.0% to 4.2% (P = .023) despite an increase in cases of gangrenous and perforated appendicitis (P = .010). CONCLUSIONS The introduction of a care pathway resulted in improved compliance with antibiotic regimens, more frequent pain assessment, and fewer post-midnight operations. Postappendicectomy readmission rates were reduced despite an increase in disease severity. This was achieved by critical reevaluation of outcomes and pathway redesign where appropriate.

Morbidity and mortality in total esophagogastric dissociation: a systematic review.

BACKGROUND/PURPOSE Total esophagogastric dissociation has been described as both a primary and a rescue procedure for patients with severe gastroesophageal reflux. Although most commonly used in the neurologically impaired, it has also been used in those with no neurological impairment. The main objective of this study was to determine morbidity and mortality for this procedure. METHODS All published cases of esophagogastric dissociation in children were identified. Series were updated where possible by personal communication with the author. Patient characteristics, indications, morbidity, and mortality were analyzed. RESULTS One hundred eighty-one cases were identified. One hundred seventeen were primary operations and 64 were rescue procedures. There were 29 (16.0%) early complications and 28 (15.5%) late complications with 6 (3.3%) deaths related to the procedure of a total of 35 deaths. Twenty-one patients (11.6%) required re-operation in the study periods. CONCLUSIONS Esophagogastric dissociation has an acceptable morbidity and mortality. It is useful as both a primary and a rescue procedure.

External ventricular drains: is there a time limit in children?

Background/purposeThe time limit for the use of external ventricular drains (EVDs) has always been controversial. The purpose of this study is to find out if there is a time limit with regard to infection of EVDs and their duration of use in children.MethodsThe records of 28 patients who had a total of 46 EVDs over a 4-year period at the Regional Paediatric Neurosurgical Centre at the Royal Liverpool Children’s Hospital, Alder Hey, Liverpool, UK, were retrieved. The cerebrospinal fluid (CSF) white cell counts, CSF Gram stains and the CSF culture results were analysed.ConclusionThere is no time limit for EVDs in children. They can be left as long as clinically indicated provided strict protocols are followed in the handling of the set.

KTP laser: an important tool in refractory recurrent tracheo-esophageal fistula in children.

Secondary tracheo-oesophageal fistula in delayed primary repair of oesophageal atresia is rare. This paper reports the successful use of the KTP laser in the treatment of this condition in a refractory case. It also recommends the use of direct laryngotracheobronchoscopy (DLTB) in the diagnosis. We recommend the use of this laser in cases of recurrent tracheo-esophageal fistula especially when other means have failed.

Rectus sheath tunnels for continent stomas.

Continent stoma rectus sheath tunnel (CSRST) has been used in antegrade colonic enema (ACE) and urinary continent cutaneous diversion (UCCD) stomas to reduce leakage and to support a straight track for the continent conduit. All patients that underwent CSRST between 1995 and 2005 were identified and their case notes retrospectively reviewed. Patients were divided into two groups: the ACE group and the UCCD group. Demographic data, age at surgery and complications including stenosis and leakage were recorded. Forty patients underwent CSRST between 1995 and 2005. The mean age and standard deviation (SD) at surgery was 6.8 (2.1) years. Eighteen patients underwent ACE with a mean (SD) follow-up of 7.6 (3.1) years. No patient has faecal leakage. No patient had stomal revision, but one patient (5.5%) required an indwelling gastrostomy button to maintain patency. Twenty-two patients underwent UCCD with a mean (SD) follow-up of 8.1 (2.8) years. No patient developed urinary leakage. Two patients (9%) required revision of the stoma and one (4.5%) required an indwelling catheter because of recurring stomal stenosis. CSRST prevents stoma leakage. Revision surgery rate after CSRST is low, particularly after ACE reconstruction.

Mature cystic teratoma arising at the porta hepatis: a diagnostic dilemma

Mature cystic teratoma (MCT) is a tumor of embryological origin, which contains tissue derived from any or all germ cell lines found in an abnormal location. We present 2 cases of MCT both arising from the porta hepatis that were incorrectly thought perioperatively to be a choledochal cyst, which subsequently were demonstrated on histology to be a MCT.

Caregiver evaluation and satisfaction with autologous bowel reconstruction in children with short bowel syndrome.

Background and Aims: Short bowel syndrome (SBS) describes the malabsorptive state seen following extensive bowel resection. Management aims to optimise nutritional intake, promote intestinal adaptation, and prevent the development of complications. Surgical options such as autologous gastrointestinal reconstruction (AGIR) attempt to restore enteral autonomy to the patient. Although the literature focuses on the objective measurements of success following AGIR techniques, subjective assessment of its effect on the quality of life (QoL) should also be sought. Because children with SBS are reliant on caregivers, caregivers’ satisfaction with surgery and their perception of the effect of AGIR on their daily lives should be assessed. This is the first report of caregiver satisfaction following AGIR in children. Methods: All children with SBS who had AGIR between January 1999 and June 2010 were identified. A questionnaire was developed. Caregivers were asked to complete the questionnaire to rate their satisfaction with surgery and the pre- and postoperative daily care, medical needs, and subjective interpretation of QoL for their child. Data were analysed using SPSS version 18, using the Friedman nonparametric test and 2-way analysis of variance by rank. Statistical significance was set at ⩽0.05. Results: Thirty-two children were identified. Seven assessments were done outside our centre (5 outside the UK) and were not included in the study. One patient moved to another city and was not contacted. Two children died. Twenty patients were contactable and were included in the present study. Parents were satisfied with the AGIR and saw improvements in their childs physical condition, bowel habit, and subjective interpretation of QoL postoperatively. Parents perceived significant improvements in the ease of caring and in aspects of their relationship with their child, including the quality of time spent, their enjoyment, and the level of their frustration with them. Conclusions: The present study found that AGIR improved the physical well-being of the child and gave the impression of improvements on specific QoL aspects for both the child and the parents.