Frédérique Sauvat

Short bowel syndrome and intestinal transplantation in children.

Purpose of reviewThis review summarizes recent knowledge and clinical practice for pediatric patients suffering extensive intestinal resection causing short bowel syndrome. This condition requires the use of parenteral nutrition, as long as intestinal failure persists, and may be, in some selected cases, an indication for intestinal transplantation. Recent findingsBiological evaluation of intestinal failure is becoming possible with the use of plasma citrulline as a marker of intestinal mass. Few epidemiological data are available; some indicate an increased incidence of short bowel syndrome-related gastroschisis and persistent high incidence of necrotizing enterocolitis. Morbidity and mortality data in pediatric patients with short bowel syndrome are limited, while long-term outcome is better documented from recently reported cohorts. Non-transplant surgery is one of the best options for patients with unadapted short bowel syndrome. Isolated liver transplantation may be avoided. The use of trophic factors for enhancing mucosal hyperplasia still remains disappointing. SummaryThe management should include therapies adapted to each stage of intestinal failure, based on a multidisciplinary approach in centers involving pediatric surgery, pediatric gastroenterology, parenteral nutrition expertise, home- parenteral nutrition program, and liver-intestinal transplantation experience. If managed appropriately, the prognosis of short bowel syndrome is excellent, with limited indications for intestinal and/or liver transplantation. Timing for patient referral in specialized centers remains an issue.

Fetal Intestinal Obstruction Induces Alteration of Enteric Nervous System Development in Human Intestinal Atresia

Intestinal motility disorders are a major cause of morbidity after surgical repair of intestinal atresia of unknown mechanism. We hypothesized that interruption of antenatal peristalsis may disturb the normal development of the enteric nervous system. Using a series of neuronal (synaptophysin, neuronal nitric oxide synthase, neurofilaments) and nonneuronal markers (glial acidic fibrillary protein and c-Kit) and immunohistochemistry, we have defined developmental steps of the enteric nervous system in normal intestine (12 fetuses, 15 children, and 4 adults) and their alterations above and below the obstacle in 22 human intestinal atresia compared with age-matched controls. Antisynaptophysin antibody revealed the progressive conversion of the myenteric plexus from a continuous belt into regularly spaced ganglions during normal fetal gut development and, by contrast, the significantly delayed appearance of individual neuronal ganglions in the distal segments of atresia (p < 0.05). Staging using three other markers for neuronal (neurofilaments and neuronal nitric oxide synthase) and nonneuronal cells (glial acidic fibrillary protein) confirmed that maturation of the myenteric plexus was significantly delayed below atresia (p < 0.01). These results indicate that intestinal atresia impairs the development of the enteric nervous system and provide an anatomical substrate for the motility disorders observed after surgical repair. They point to the role of peristalsis in normal gut development and suggest that stimulation of peristalsis might be used to accelerate recovery.

MYO5B and bile salt export pump contribute to cholestatic liver disorder in microvillous inclusion disease

Microvillous inclusion disease (MVID) is a congenital disorder of the enterocyte related to mutations in the MYO5B gene, leading to intractable diarrhea often necessitating intestinal transplantation (ITx). Among our cohort of 28 MVID patients, 8 developed a cholestatic liver disease akin to progressive familial intrahepatic cholestasis (PFIC). Our aim was to investigate the mechanisms by which MYO5B mutations affect hepatic biliary function and lead to cholestasis in MVID patients. Clinical and biological features and outcome were reviewed. Pretransplant liver biopsies were analyzed by immunostaining and electron microscopy. Cholestasis occurred before (n = 5) or after (n = 3) ITx and was characterized by intermittent jaundice, intractable pruritus, increased serum bile acid (BA) levels, and normal gamma‐glutamyl transpeptidase activity. Liver histology showed canalicular cholestasis, mild‐to‐moderate fibrosis, and ultrastructural abnormalities of bile canaliculi. Portal fibrosis progressed in 5 patients. No mutation in ABCB11/BSEP or ATP8B1/FIC1 genes were identified. Immunohistochemical studies demonstrated abnormal cytoplasmic distribution of MYO5B, RAB11A, and BSEP in hepatocytes. Interruption of enterohepatic BA cycling after partial external biliary diversion or graft removal proved the most effective to ensure long‐term remission. Conclusion: MVID patients are at risk of developing a PFIC‐like liver disease that may hamper outcome after ITx. Our results suggest that cholestasis in MVID patients results from (1) impairment of the MYO5B/RAB11A apical recycling endosome pathway in hepatocytes, (2) altered targeting of BSEP to the canalicular membrane, and (3) increased ileal BA absorption. Because cholestasis worsens after ITx, indication of a combined liver ITx should be discussed in MVID patients with severe cholestasis. Future studies will need to address more specifically the effect of MYO5B dysfunction in BA homeostasis. (Hepatology 2014;60:301–310)

Intestinal transplantation for total intestinal aganglionosis: a series of 12 consecutive children

BACKGROUND Management of patients with total intestinal aganglionosis (TIA) is a medical challenge because of their dependency on parenteral nutrition (PN). Intestinal transplantation (ITx) represents the only alternative treatment for patients with irreversible intestinal failure for achieving intestinal autonomy. METHODS Among 66 patients who underwent ITx in our center, 12 had TIA. They received either isolated ITx (n = 4) or liver-ITx (LITx, n = 8) after 10 to 144 months of total PN. All grafts included the right colon. RESULTS After a median follow-up of 57 months, the survival rate was 62.5% in the LITx group and 100% in the ITx patients. The graft survival rate was 62.5% in the LITx group and 75% in the ITx group. All the surviving patients were fully weaned from total PN, after a median of 57 days. Pull through of the colon allograft was carried out in all patients. Fecal continence is normal in all but one of the surviving children. CONCLUSION These results suggest that ITx with colon grafting should be the preferred therapeutic option in TIA. Early referral to a transplantation center after diagnosis of TIA is critical to prevent PN-related cirrhosis and thereby to permit ITx, which is associated with a good survival rate.

Paraesophageal bronchogenic cyst: first case reports in pediatric

We described for the first time a paraesophageal intra-abdominal bronchogenic cyst, first considered as hepatic tumor, in a young boy. Resection was successfully realized by laparoscopy. Abdominal bronchogenic cyst and differential diagnosis are discussed.

Conservative management of blunt pancreatic trauma in children: a single center experience.

INTRODUCTION Blunt trauma of the pancreas represents a significant part of abdomen trauma in children with an incidence estimated at around 10%. If the conservative management is widely accepted concerning the stages I and II, it remains controversial concerning stages III and IV. The aim of our study was to perform a descriptive analysis of the nonoperative management, with a focus on the occurrence of pseudocysts. MATERIALS AND METHODS The charts of the patients treated in our center for pancreatic trauma from 1990 to 2010 have been reviewed. It was defined by an initial lipase greater than three times the norm and an abnormal computed tomography scan. RESULTS A total of 36 patients were included, with 26 boys (72%) and 10 girls (28%) with an average age of 8.7 years. The trauma was isolated in 13 cases (36.1%) and in 23 cases, there were other associated lesions (mainly liver [n = 9] and spleen [n = 5]). Pancreatic injuries were graded as follows: I (n = 21), II (n = 2), III (n = 7), and IV (n = 6). Pseudocysts occurred in 11 patients (30.5%) mainly in grades III (n = 3) and IV (n = 7), with an average delay of 17 days. Initial management of pseudocysts was conservative in six patients (54.6%), whereas five patients required mini-invasive procedures. CONCLUSION Nonoperative management remains a safe way to treat pancreatic injuries despite an average 30% rate of pseudocyst (PC) appearance. It allows a reduction in the number of children who required procedures to less than half of the patients where PC occurred. Furthermore, these procedures were exclusively mini-invasive.

Intestinal absorption rate in children after small intestinal transplantation.

BACKGROUND Small bowel transplantation has now become a recognized treatment of irreversible, permanent, and subtotal intestinal failure. OBJECTIVE The aim of this study was to assess intestinal absorption at the time of weaning from parenteral nutrition in a series of children after intestinal transplantation. DESIGN Twenty-four children (age range: 14-115 mo) received intestinal transplantation, together with the liver in 6 children and the colon in 16 children. Parenteral nutrition was slowly tapered while increasing enteral tube feeding. The absorption rate was measured from a 3-d stool balance analysis performed a few days after the child had weaned from parenteral nutrition to exclusive enteral tube feeding. Results were analyzed according to the resting energy expenditure (REE; Schofield formula). RESULTS All children were weaned from parenteral nutrition between 31 and 85 d posttransplantation. Median intakes were as follows: energy, 107 kcal · kg(-1) · d(-1) (range: 79-168 kcal · kg(-1) · d(-1)); lipids, 39 kcal · kg(-1) · d(-1) (range: 20-70 kcal · kg(-1) · d(-1)); and nitrogen, 17 kcal · kg(-1) · d(-1) (range: 11-27 kcal · kg(-1) · d(-1)). Median daily stool output was 998 mL/d (range: 220-2025 mL/d). Median absorption rates were 88% (range: 75-96%) for energy, 82% (range: 55-98%) for lipids, and 77% (range: 61-88%) for nitrogen. The ratios for ingested energy to REE and absorbed energy to REE were 2.2 (range: 1.6-3.6) and 1.8 (range: 1.3-3.3), respectively. CONCLUSION These data indicate a suboptimal intestinal graft absorption capacity with fat malabsorption, which necessitates energy intakes of at least twice the REE.

Seventeen years after successful small bowel transplantation: long term graft acceptance without immune tolerance

The first attempts at small bowel transplantation (SBT) in humans were disappointing1–3 and characterised by severe graft rejection in spite of initially encouraging experience in animals.3–5 Major difficulties were encountered as: (1) the intestine is an immune organ harbouring most immune competent cells of the body; and (2) it is particularly vulnerable to ischaemia, not allowing time consuming HLA matching prior to transplantation (Tx). Therefore, Tx of intestinal allografts into fully immune competent recipients results almost inescapably in terminal graft rejection. With the introduction of tacrolimus in the 1990s, this difficulty was partially overcome, improving small bowel graft survival rates (70% at one year post Tx).6–8 However, on follow up, a large number of grafts are still lost, raising the question of long term success and benefit after SBT. Here we present the first patient with a 17 year follow up after isolated …

Successful management of congenital bronchial stenosis using an expandable stent.

Congenital bronchial stenosis is a very rare cause of neonatal dyspnea. Surgical management remains challenging in small children. We report successful implantation of a bronchial stent in a 3-month-old female infant presenting with congenital right bronchial stenosis and 18 months of follow-up. Use of stents in children remains controversial because of the problem of size mismatch as the child grows. Nevertheless, expandable stent implantation could be an interesting alternative to complex surgery for localized bronchial stenosis in neonates.

Comments on “Challenges in Transition of Care for Pediatric Patients after Weight-Reduction Surgery: a Systematic Review and Recommendations for Comprehensive Care”

Obesity is a global health problem that can affect adults as well as children. Bariatric surgery has revolutionized the management of this pathology in adults. In children, it is more and more practiced, but follow-up and management are still not codified [1]. The treatment of obesity is merely palliative, which is why a multidisciplinary approach is needed. Sarah B. Cairo et al. recently published an article outlining a timeline and milestones in the follow-up of children undergoing bariatric surgery in order to harmonize follow-up and facilitate the transition from the Binfant^ phase to the Badult^ phase during which it will be followed by specialist practitioners for adults [2]. We found the article very interesting; however, we do not agree with some points because the child is not a miniaturized adult. The care must take into account the child in his globality: