Intestinal bowel lengthening in children with short bowel syndrome: systematic review of the Bianchi and STEP procedures.

BACKGROUND: It has been thirty years since Bianchi introduced the technique that made intestinal lengthening possible. The last three decades have seen lengthening procedures established as vital components of intestinal rehabilitation programs. The goal of the present study was to use a systematic literature review to determine patient outcomes for the two most commonly used lengthening procedures, the Bianchi procedure and the serial transverse enteroplasty procedure (STEP). METHODS: Pubmed and Embase were searched using the terms "intestinal lengthening" and "bowel lengthening." Patient outcomes were extracted from each relevant journal article on the basis of a set proforma. The results were combined to create overall mean outcomes. Mean outcomes were also calculated separately for the Bianchi procedure and STEP. Significance was tested with the independent t-test. RESULTS: Overall survival for the last thirty years is 83%. However, survival for the last fifteen years has been 89%, with no significant difference between the two procedures. The Bianchi procedure has a higher rate of weaning patients who were static on parenteral nutrition with conservative measures: 55% versus 48%. In addition, the Bianchi procedure was associated with a higher rate of patients receiving transplants: 10% versus 6%. The STEP has a higher rate of complication. Length of follow-up is significantly longer for the Bianchi procedure: 76 versus 22 months. The impact that this differential could have had on our results must be considered. CONCLUSIONS: Outcomes for intestinal lengthening procedures are very good, and increasingly so. However, further analysis is required in order to fully understand the relative strengths and weaknesses of each procedure.

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.

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 child's 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.

Clinical practice: management of biliary atresia.

Biliary atresia is a rare, serious and challenging disease in newborn children. Its aetiology remains unknown. Optimal management at specialist centres with resultant better overall outcomes is achieved through a multidisciplinary team approach. The Kasai portoenterostomy performed early in life remains the only surgical repair procedure. Two thirds of patients will clear their jaundice after a Kasai procedure, but only about one third will retain their livers after the first decade of life. Failure of this procedure leaves liver transplantation as the only chance for survival, and this disease is the commonest indication for liver transplantation in children. With modern medical care and refinements in surgical techniques, survival after either or both of these procedures is about 90%. Early referral to specialist centres and long-term specialist care remains the key to successful treatment of this condition.

Surgical intervention in children with vesicoureteric reflux: are we intervening too late?

BACKGROUND: Vesicoureteric reflux is usually managed medically. When medical management fails, the patient is referred for surgical intervention. The aim is to protect the kidneys from progressive damage. This study investigates if significant kidney damage has occurred during medical treatment prior to surgical intervention. MATERIALS AND METHODS: Case notes of all children treated with ureteric re-implantation for vesicoureteric reflux in a 5-year period were reviewed. Demographic details, radiological investigations, surgery and follow-up were recorded. Indication for surgery was failure of medical treatment. Kidney damage was defined as the presence of a scar on the DMSA scans and/or kidney function below 45% in one kidney. RESULTS: Forty-two patients underwent ureteric re-implantation with 24 having a bilateral procedure resulting in a total of 66 renal units. Mean age at surgery was 7.4 years. Thirty-eight kidneys (58%) showed reduced function prior to medical treatment. Twenty-four kidneys (36%) had deterioration of renal function associated with recurrent urinary tract infections during the course of medical treatment with the overall mean function of the worst affected kidney being 28%. Thirty-five patients (83%) demonstrated scarring on their kidneys on DMSA scan prior to surgery. CONCLUSIONS: More than half of patients who eventually need ureteric re-implantation for vesicoureteric reflux have already suffered kidney damage prior to start of medical treatment. A third will have progressive deterioration of their renal functions. Early referral for medical management coupled with early surgical intervention in selected cases should hopefully reduce the number of children with renal damage due to VUR.

Long-term outcomes of surgery for malignant sacrococcygeal teratoma: 20-year experience of a regional UK centre.

BACKGROUND: The timing of surgery for malignant sacrococcygeal teratoma is controversial. The long-term outcomes and complications of surgery for this rare tumour are presented. METHODS: All cases of malignant sacrococcygeal teratoma in the 20-year period 1987-2006 were identified and the case notes retrieved. The age at diagnosis, investigations, presentation, type of surgery, early complications, recurrence rates, long-term complications and outcomes were recorded. RESULTS: Twelve patients (three males, nine females) were identified. Mean age at presentation was 20.8 months (range: 12-39 months). All had the Carboplatin-Etoposide-Bleomycin chemotherapeutic protocol. The average time of follow-up was 10.6 years (range: 1-17 years). Ten patients had excision of their tumours following chemotherapy, whilst two patients had excision prior to chemotherapy. Two patients had recurrence of their tumours. There was one death (8%), which was due to disseminated metastasis. The other 11 children were all well at the last follow-up. CONCLUSION: Surgery for malignant sacrococcygeal teratoma is safe and has a low complication rate. The long-term outcomes are favourable with minimal side effects.

Radiologic Image-Guided Tube Stoma Insertion in Neonatal Short Bowel Syndrome: First Case Report in the Literature.

Background: The management of neonatal short bowel syndrome can be challenging, and it is critical that these babies are managed in a multidisciplinary team setting with specialists who are experienced in the complex management of these children. One of the surgical strategies, initially published by the Bianchi team in Manchester, UK, is controlled tissue expansion program (CTE) which is done via the insertion of catheters into the proximal and distal bowel in the form of tube stomas. The clamping of the proximal tube allows for an increase in length and circumferential diameter of the proximal bowel for a period of time, whilst the distal tube stoma allows for easy refeeding of proximal bowel contents into the distal bowel. Method: CTE is associated with the risk of dislodgement and exposing patients to further surgical procedures with the risk of losing more bowel length. This article describes a new method in the management of such a complication through a less invasive approach of an image-guided procedure by interventional radiologists. Conclusion: Radiologically guided tube stoma reinsertion in a child with ultrashort bowel syndrome is a promising technique and should be considered in a CTE program in the management of short bowel syndrome.

Bridging the gap: functional healing of embryonic small intestine ex vivo.

The ability to grow embryonic organs ex vivo provides an opportunity to follow their differentiation in a controlled environment, with resulting insights into normal development. Additionally, similar strategies can be used to assess effects on organogenesis of physical and chemical manipulations. This study aimed to create an organ culture model with which to test physical manipulations to enhance healing of gut segments, thus generating a single functional organ. Embryonic mouse jejunum was isolated and cut into 2-3 mm tubes, which were placed in pairs, separated by a small gap, on semi-permeable supports. Each pair was linked by a nylon suture threaded through their lumens. After 3 days in organ culture fed by defined serum-free media, the rudiments differentiated to form tubes of smooth muscle surrounding a core of rudimentary villi. Of 34 such pairs, 74% had touching and well aligned proximate ends. Of these joined structures, 80% (59% of the total pairs) had a continuous lumen, as assessed by observing the trajectories of fluorescent dextrans injected into their distal ends. Fused organ pairs formed a single functional unit, as assessed by spontaneous contraction waves propagated along their lengths. In these healed intestines, peripherin(+) neurons formed a nexus in the zone of fusion, linking the rudiment pairs. In future, this system could be used to test whether growth factors enhance fusion. Such results should in turn inform the design of novel treatments for short bowel syndrome, a potentially fatal condition with a currently limited and imperfect range of therapies.

Short bowel syndrome in children: surgical and medical perspectives.

The main cause of intestinal failure in children is due to short bowel syndrome (SBS) resulting from congenital or acquired intestinal lesions. From the first lengthening procedure introduced by Bianchi, the last three decades have seen lengthening procedures established as fundamental components of multidisciplinary intestinal rehabilitation programs. Debate on indications and timing of the procedures is still open leaving SBS surgical treatment a great challenge. However, enteral autonomy is possible only with an individualized approach remembering that each SBS patient is unique. Current literature on autologous gastrointestinal reconstruction technique was reviewed aiming to assess a comprehensive pathway in SBS non-transplant management.

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.

Transanoproctoplasty: a 21-year review.

BACKGROUND: Transanal anorectoplasty was developed through the 1980s by the senior author (AB) as an alternative approach to posterior sagittal anorectoplasty for the management of imperforate anus. This study evaluates this surgical approach and its longer-term results. METHODS: Case notes of all patients treated from 1984 to 2005 were reviewed. Operative procedures, colostomy requirement, complications, and long-term outcome were recorded. Patients were grouped according to the status of the pelvic floor, the location of the rectal fistula, and the sacral ratio. RESULTS: A total of 245 patients (175 male, 70 female) underwent transanal anorectoplasty. The perineum was well formed in 208 patients (85%), moderately formed in 15 (6%), and poorly formed in 22 (9%) patients. Two hundred three patients (82.8%) had a visible perineal or vulval fistula, 24 (9.8%) had a prostatic urethral fistula, whereas 18 (7.4%) had a bulbourethral fistula. Overall, 6 patients (2.5%) had wound infection or breakdown. As continence is age related, 32 patients were excluded from the study. Of the remaining 212 patients, 182 are continent with no soiling or only minimal staining. Thirty patients born with a poor perineum are incontinent. CONCLUSION: Transanal anorectoplasty is a safe procedure with limited morbidity. It is sphincter sparing and permits accurate placement of the rectum with its internal sphincter within the anal canal. The anus lies accurately placed at the center of the external anal sphincter muscle complex. Given a well-performed surgical intervention, eventual continence relates to the original anatomy and neurology of the pelvic floor. Transanal anorectoplasty should be regarded as an alternative rather than as a substitute to posterior sagittal anorectoplasty for reconstruction of most forms of imperforate anus.

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.

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.

Gastroschisis associated with cleft lip and palate.

Gastroschisis is a common congenital abdominal wall defect. Rarely, it is associated with extraabdominal midline congenital anomalies. Oral clefts are the commonest craniofacial congenital midline defects. They can be associated with other midline defects like omphalocele. We believe these are the first 3 case reports of gastroschisis and cleft palate occurring in the same patient.

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

BACKGROUND/PURPOSE: The 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. METHODS: The 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. CONCLUSION: There 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.

Hidden pathologies associated with (and concealed by) early gestational isolated fetal hydrothorax.

Isolated fetal hydrothorax is an uncommon finding on routine prenatal ultrasound and is associated with a variable prognosis. We believe this is the first report of a patient whose antenatal hydrothorax was associated with both congenital diaphragmatic hernia and congenital pulmonary lymphangiectasia. This rare combination caused significant problems in diagnosis and subsequent treatment.