Combined procedures for surgical short bowel syndrome: experience from two European centres.

BACKGROUND: Autologous gastrointestinal reconstructive surgery (AGIR) has become a key component of intestinal rehabilitation programs. However, the best surgical option for short bowel syndrome (SBS) remains unknown. This paper presents our experience using combined procedures as primary treatment. METHODS: We collected data on SBS patients who underwent surgery from 2008 to 2021 in two tertiary European Centres. Combined procedures were defined as more than one technique used on the same patient. Charts were reviewed for demographics, type of procedures, complications, and outcomes. Data are presented as median and IQR. Wilcoxon signed rank was used for all paired analyses. RESULTS: Twenty-one children (12 females) underwent combined procedures. Preoperative median small bowel length was 20 cm (IQR: 15-35 cm); after lengthening, it was 35.5 cm (IQR: 30.75-50.50 cm) (P < 0.001). Combined procedures were simultaneous in 15 patients and sequential in 6. At a median of 9.2 years (IQR: 7.55-9.78 years) follow-up, complications were three bowel obstructions after strictures of anastomosis and two wound infections. Two patients achieved enteral autonomy, and others followed a weaning home parenteral nutrition regimen with a median of 4 nights off (IQR: 3-4 nights) starting with a median of 7 nights (IQR: 7-7 nights). CONCLUSIONS: Combined AGIR techniques are practical and safe in SBS treatment when tailored to meet patients' needs, combining lengthening, tailoring, and reducing transit time procedures. Therefore, combined AGIR may be considered a resource in intestinal rehabilitation units' armamentarium.

Early Bowel Lengthening Procedures: Bi-Institutional Experience and Review of the Literature.

Early bowel lengthening procedure (EBLP) has been defined as any bowel lengthening procedure performed before six months of age. The purpose of this paper is to compare our experience with literature on this subject to identify common indications. A bi-institutional retrospective analysis was performed. Diagnosis, type of surgery, age at procedure and outcomes were analysed. Eleven EBLP were performed in Manchester and Florence from 2006 to 2021. The median age at surgery was 126 days (102-180), pre-operative median short bowel (SB) length was 28 cm (17-49) with a post-operative median increase of 81%. Furthermore, a PubMed/Embase search was undertaken regarding bowel lengthening procedures performed in the last 40 years. Sixty-one EBLP were identified. The median age was 60 days (1-90). Serial transverse enteroplasty (STEP) was the most frequent procedure used, with a median increased bowel length of 57%. This study confirms that no clear consensus on indication or timing to perform early SB lengthening is reported. According to the gathered data, EBLP should be considered only in cases of actual necessity and performed in a qualified intestinal failure centre.

Messy Play Therapy in the Treatment of Food Aversion in a Patient With Intestinal Failure: Our Experience.

BACKGROUND: Food aversion (FA) is an eating behavior where children refuse solid or fluid intake. FA can compromise the weaning off parenteral nutrition (PN) in children with intestinal failure (IF), reducing their quality of life (QoL). Around 25% of children with IF experience FA, but few data are available on interventions to get over FA. Messy play therapy (MPT) uses sensory activities to provide another meaningful avenue for learning in children by creating a fun way to experience new textures. This study aims to assess the efficacy of MPT in FA. METHODS: Demographic data and MPT intervention were retrospectively recorded between 2004 and 2017. Food was categorized by tastes and textures. Data are expressed as median and interquartile range (25%-75%). RESULTS: Twelve children were identified. MPT was started at 9 (6-16) months with an enrolling time within the program of 10.11 (7.75-12.5) months. MPT was ended after 19.5 (16.75-28.5) months, and all patients achieved tolerance to oral diet. Significant improvement in savory (P = .001), sweet (P = .002), and mixed texture (P = .001) of food intake was reported. Better QoL and mealtimes with family were reported at median follow-up of 39 (24-56) months. CONCLUSIONS: MPT seems to be a positive intervention to overcome FA. In our experience, the children have gone from not tolerating any intake to tolerating an oral diet, which means enjoying their mealtimes. Further studies are needed to evaluate the effectiveness of MPT in a larger scale of patients.

Feeding Problems Are Persistent in Children with Severe Congenital Hyperinsulinism.

BACKGROUND: Congenital hyperinsulinism (CHI) is a rare but severe disorder of hypoglycemia in children, often complicated by brain injury. In CHI, the long-term prevention of hypoglycemia is dependent on reliable enteral intake of glucose. However, feeding problems (FPs) often impede oral glucose delivery, thereby complicating the management of hypoglycemia. FPs have not been systematically characterized in follow-up in a cohort with CHI. AIMS: We aimed to determine the prevalence, types, and persistence of FPs in a cohort of children with CHI and investigate potential causal factors. METHODS: FPs were defined as difficulty with sucking, swallowing, vomiting, and food refusal (or a combination) in an observational study in 83 children in a specialized CHI treatment center. The prevalence of FPs at diagnosis, 6, and 12 months after diagnosis were noted. Genetic mutation status and markers of severity of CHI were tested for association with FPs. RESULTS: A third of children with CHI had FPs (n = 28), of whom 93% required antireflux medication and 75% required nasogastric and gastrostomy tube feeding. Sucking and swallowing problems were present at diagnosis but absent later. Vomiting was present in 54% at 6 months, while food refusal was present in 68% at 6 months and 52% at 12 months. The age at commencing and stopping nasogastric tube feeding did not correlate with FPs frequency at 6 and 12 months. Children with FPs had severe hypoglycemia at diagnosis and required glucagon infusion more often [odds ratio (OR) (95% confidence intervals) (95% CI) 28.13 (2.6-300.1), p = 0.006] to normalize glucose levels. FPs were more frequent in those with diffuse CHI undergoing subtotal pancreatectomy [n (%) = 10 (35%) vs. 0 (0%), p < 0.001], in contrast to those with spontaneous resolution [6 (22%) vs. 32 (58%), p = 0.002]. Those undergoing focal lesionectomy also had reduced FPs at 6 months after diagnosis [OR (95% CI) 0.01 (0.0-0.2), R (2) = 0.42, p = 0.004]. These observations suggest that persistence of hyperinsulinism was associated with FPs. CONCLUSION: FPs occur in a significant proportion of children with CHI. Severe hyperinsulinism, rather than nasogastric tube feeding or medications, is the main factor associated with FPs.

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.

Nutritional problems in children treated for medulloblastoma: implications for enteral nutrition support.

BACKGROUND: The aim of this study was to identify the nature and severity of nutritional problems associated with the current treatment of medulloblastoma and to identify any risk factors for nutritional morbidity during treatment. PROCEDURE: A multicentre retrospective audit of medical and dietetic notes of 41 children treated for medulloblastoma in three UK paediatric oncology centres was undertaken. Data on nutritional status, nutritional support, mutism, swallowing and common toxicity criteria (CTC) scores for vomiting, constipation and mobility were collected at defined points in treatment from diagnosis until 12 months post-treatment. RESULTS: Significant problems including weight loss, vomiting and constipation were highlighted early on in treatment. The majority of patients were well nourished at diagnosis with a mean percentage weight: height of 99.8%, however nutritional status started to decline early in treatment during radiotherapy, coinciding with 49% of patients having grade 1 or above CTC score for vomiting and constipation. The decline in nutritional status continued, peaking by course 2 of chemotherapy with a mean weight loss of 8.2% since diagnosis. Proactive supplementary feeding early in treatment by one of the three centres demonstrated a superior nutritional outcome when compared statistically to the two centres that fed only as a response to nutritional decline. CONCLUSION: The study highlighted significant morbidity associated with the current treatment of medulloblastoma. Findings suggest the need to consider earlier proactive nutritional intervention to prevent nutritional decline during treatment. These early nutritional problems may be related to toxicities of radiotherapy and concomitant vincristine.