Alterations in smooth muscle contractile and cytoskeleton proteins and interstitial cells of Cajal in megacystis microcolon intestinal hypoperistalsis syndrome

Background/Purpose: Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is characterized by decreased or absent peristalsis. Gastrointestinal motility depends on the enteric nervous system, smooth muscle cells (SMCs), and the interstitial cells of Cajal (ICCs). Contractile and cytoskeleton proteinase are important structural and functional components of SMCs. The aim of study was to examine the expression of contractile and cytoskeleton proteins in SMCs and distribution of ICCs in MMIHS bowel. Methods: Full-thickness bowel specimens were obtained from 4 infants with MMIHS and 4 controls. Specimens were processed as whole-mount preparations and frozen and paraffin sections. Combined staining of NADPH-d histochemistry/c-kit immunohistochemistry, single and double immunohistochemistry using [alpha ]-smooth muscle actin ([alpha ]-SMA), calponin (CALP), caldesmon (CALD), desmin (DES), protein gene product 9.5 (PGP 9.5) and c-kit antibodies were performed and examined using light and confocal scanning microscopy. Results: [alpha ]-SMA, CALP, CALD, and DES immunoreactivity were reduced markedly in MMIHS bowel compared with controls. Combined NADPH/c-kit staining showed dense network of ICCs around myenteric plexus in MMIHS bowel. In contrast, the intramuscular ICCs either were absent or reduced in MMIHS bowel. Conclusions: Marked reduction of contractile and cytoskeleton proteins in SMCs combined with reduced expression of intramuscular ICCs in the gut may be responsible for the motility dysfunction in MMIHS. J Pedriatr Surg 38:749-755. [copy ] 2003 Elsevier Inc. All rights reserved.     

Abnormalities of C-Kit-positive cellular network in isolated hypoganglionosis

Background/Purpose: C-Kit-positive interstitial cells of Cajal (ICCs) have a key role in the normal motility function and development of the bowel. They are pacemaker cells, which facilitate active propagation of electrical events and neurotransmission in the bowel wall. ICCs are present in the bowel as myenteric ICCs (ICC_myS) and muscular ICCs (ICC_musS). The aim of this study was to examine the distribution of c-Kit-positive ICCs and their relationship to the autonomic intrinsic innervation in bowel specimens from patients with isolated hypoganglionosis. Methods: Full-thickness large bowel specimens were obtained from 6 patients with hypoganglionosis and from 4 patients during bladder augmentation (controls). Frozen sections and whole-mount preparations were stained using c-Kit immunohistochemistry, nicotinamide adenine dinucleotide phosphate (NADPH)-diaphorase, and acetylcholinesterase (AChE) histochemistry and evaluated using normal brightfield and confocal laser scanning microscopy. Results: NADPH-diaphorase and AChE histochemistry findings showed characteristic histologic features of hypoganglionosis, eg, sparse and small myenteric ganglia and low or absent AChE activity in the lamina propria. Myenteric plexus in the normal bowel was surrounded by a dense network of c-Kit-positive ICC_myS, whereas in hypoganglionosis sparse isolated ICC_myS were found. C-Kit-positive ICC_musS were reduced markedly in the longitudinal and circular muscle layer and at the innermost part of the circular muscle in hypoganglionosis. Conclusion: Deficient expression of c-Kit-positive myenteric and muscular ICCs in the hypoganglionic colon may contribute to the motility dysfunction in the affected bowel.     

Preoperative enterocolitis is associated with poorer long-term bowel function after Soave-Boley endorectal pull-through for Hirschsprung's disease

Background/Purpose: The purpose of the investigation was to apply a semiquantitative scoring system for bowel function to patients who had undergone endorectal pull-through (ERPT) for Hirschsprung's disease (HD) and to use this to analyse the clinical factors relating to functional outcome. Methods: The case note details of 63 patients undergoing ERPT for HD were reviewed. A questionnaire using a semiquantitative scoring system for 6 aspects of bowel function was sent to parents of 55 patients. The total functional score (TFS) for each patient was calculated, and a statistical analysis was performed to determine which clinical factors were significantly related to bowel functional outcome. Results: Fifty (91%) of the parents returned the questionnaire. TFS was [ldquo ]good[rdquo ] in 45%, [ldquo ]fair[rdquo ] in 33% and [ldquo ]poor[rdquo ] in 22%. There was a statistically significant difference in the scores of those who had preoperative Hirschsprung's associated enterocolitis (HAEC) TFS = 8.0 and those who did not, TFS 12.7 (P [lt ] .01) The sex of the patient, length of aganglionic segment, timing of ERPT (early/late), staging of ERPT (1 or 2), presence/absence of anastomotic stricture, and presence or absence of Down's syndrome did not statistically significantly affect TFS. Conclusions: Preoperative HAEC was the most important factor in relation to functional outcome after ERPT for HD. The reason remains unclear. J Pediatr Surg 38:69-72.     

Submucosal pressure-air insufflation facilitates endorectal mucosectomy in transanal endorectal pull-through procedure in patients with Hirschsprung's disease

Background: Most children with Hirschsprung's disease (HD) can be treated with a transanal endorectal pull-through (TEP) procedure. The authors have developed a simple technique of submucosal pressure-air insufflation (SI) to facilitate the submucosal dissection, which is one of the crucial parts of the operation. Methods: Six patients with HD were treated by using TEP in one year. After adequate positioning and exposure, anal mucosa was incised 1 cm above the dentate line, and 4-quadrant SI with a simple system of scalp-vein needle connected to a 20-mL syringe was used in all of the patients. Submucosal proctectomy, aganglionic and dilated segment colectomy, and coloanal anastomosis were completed transanally, but, in 2 of the patients, laparoscopic assistance to release the colon was required. Results: There were no intraoperative and postoperative complications related to SI. Submucosal dissections were completed smoothly in all of the patients with negligible amount of bleeding. The only complication during the submucosal dissection was mucosal perforation at the site of previous rectal biopsy in 2 patients. Average operating time was 2.7 hours (range, 90-180 min), and mean length of resected bowel was 22.5 cm (range, 12 to 42 cm). Follow-up is 8 to 14 months. Frequent bowel movements ([gt ]8 times per day) and perianal dermatitis were observed in 2 patients but returned to acceptable limits in 3 months. One patient had to undergo reoperation for adhesive intestinal obstruction. Conclusions: SI is simple, and offers a safe and faster dissection with minimum amount of bleeding during the endorectal mucosectomy in TEP procedure. J Pediatr Surg 38:188-190.     

Combined abdominal and posterior sagittal approach for redo pull-through operation in Hirschsprung's disease

Background/Purpose: In Hirschsprung's disease (HD) redo pull-through (PT) is indicated for anastomotic complications and retained aganglionosis after a previous operation. Duhamel or Swenson method is used commonly for redo operations. The pelvic dissection may be difficult, especially in Swenson's type of operation, because of fibrosis resulting from previous surgery or its complications. To overcome this, the authors used a combined abdominal and posterior sagittal approach to perform redo pull-through of Swenson's type in 4 children. Methods: Four boys (2.5 to 12 years) underwent redo pull-through for failed endorectal pull through (n = 2), persistent symptoms after 2 myectomies (n = 1) and late anastomotic disruption after Swenson's PT (n = 1). Abdominal dissection was done first to mobilize colon and resect aganglionic segment as far as the mid pelvis. The mobilized ganglionic colon was tacked to the pelvic rectal stump, hemostasis checked, and the abdomen closed. The lower pelvic dissection was done through the posterior sagittal route, under direct vision. The remainder of diseased rectum was excised, and the pull-through colon was retrieved and anastomosed to the anal stump. No covering colostomy was done. Results: A rectocutaneous fistula developed in one patient, which healed spontaneously. All patients had increased stool frequency in the early postoperative period but improved with time. All patients have attained normal voluntary bowel actions, but one child has infrequent minor soiling. There was no anastomotic narrowing in any case. Conclusions: Posterior sagittal approach is a useful alternative in difficult redo pull-through surgery. It offers excellent exposure, precise dissection, and direct anastomosis. There are minimal chances of complications, and continence is retained. J Pediatr Surg 37:1156-1159.     

Delayed maturation of interstitial cells of Cajal in meconium obstruction

Background/Purpose: The etiology of meconium obstruction without cystic fibrosis is unclear. Interstitial cells of Cajal (ICC) function as pacemakers in gut motility and may play a role in the pathophysiology of the disease. Methods: The ICC were examined by immunohistochemical staining with anti-c-kit antibody in the bowel walls of 6 neonates who had meconium obstruction without cystic fibrosis, and the results were compared with specimens from normal neonates (n = 2). Results: Six patients underwent ileostomy between 2 and 15 days after birth, and 5 of them presented with microcolon. Ganglion cells were present in the ileum and colon. Whereas ICC were evenly distributed in the control specimens, they were not seen at the time of ileostomy in the colons of 2 patients, and the other 4 showed scanty distribution in muscle layers. However, ileum showed normal distribution of ICC in all patients. The ileostomies were closed between 39 and 104 days of age, and the ICC distribution was changed to a normal pattern in the colons of all 6 patients. Their bowel movements were restored to normal after closure. Conclusion: The findings of this study suggest that delayed maturity of ICC may be a cause of meconium obstruction without cystic fibrosis.     

Congenital pouch colon: Massive redilatation of the tubularized colonic pouch after pull-through surgery

Background/Purpose: Children with a type I/II congenital pouch colon (CPC) malformation associated with imperforate anus usually are treated by subtotal excision of the colonic pouch, tubularization of the remaining portion, and pull-through of the tubularized colon during definitive surgery. The authors report 3 patients treated in this fashion who presented 2 to 10[frac12] years later with massive redilatation of the previously tubularized colon and enterocolitis Methods: There were no anal strictures or malpositioning of the pulled through bowel. Contrast enema showed massive redilatation of the colonic pouch. Near-total excision of the redilated pouch with anastomosis of normal proximal ileum/colon with the retained distal portion of the pouch was performed by the abdominal approach. Results: Anastomotic leaks occurred in 2 patients but were treated successfully. Postoperatively, the patients had relief from their abdominal symptoms and improvement in fecal continence. Conclusions: The colonic pouch in CPC has a marked tendency to undergo redilatation, even after tubularization. The surgical procedure described here for the treatment of these patients appears to be satisfactory. J Pediatr Surg 37:1376-1379.     

Hirschsprung's disease in cartilage-hair hypoplasia has poor prognosis

Purpose: Cartilage-hair hypoplasia (CHH) is a chondrodysplasia with growth failure, impaired immunity, and high incidence of Hirschsprung disease (HD). This study describes the outcome of CHH patients with HD. Methods: Among 147 patients with CHH, 13 were identified to have HD. Their medical records were analyzed for treatment, outcome, and complications of HD and compared with a control group of 169 patients with HD but not CHH. Results: Eight CHH patients had classic HD with rectosigmoid involvement, 2 had long segment colonic disease, and 3 patients had total colonic aganglionosis. Six of the 13 CHH patients (46%) had episodes of enterocolitis before the first surgery. Enterocolitis was complicated by colonic perforation in 2 cases. Eleven CHH patients (85%) had at least one episode of postoperative enterocolitis. Five patients (38%) with CHH and HD had died; 4 of enterocolitis-related septic infection and one of non-Hodgkin's lymphoma. In the control group, preoperative enterocolitis occurred in 14% and postoperative enterocolitis in 8%. Two controls (1.2%) had died. Conclusions: HD associated with CHH has poor prognosis in terms of postoperative morbidity and risk of death. These patients require particular attention during postoperative follow-up to detect potentially lethal complications.     

Long-term outcome and quality of life after the Swenson procedure for Hirschsprung's disease

Background/Purpose: The aim of this study was to investigate long-term outcome and quality of life after the Swenson operation for rectosigmoid Hirschsprung's Disease (HD). Methods: Forty-five patients who underwent the Swenson procedure for HD underwent follow-up for 8 to 16 years. Long-term outcome and quality of life were assessed by interviews and questionnaires including scoring systems. Forty-four healthy children with similar age, sex, and education level distributions used as controls. Results: In 45 patients, 23 (51.1%) had bowel dysfunction. Seventeen patients (37.8%) suffered from fecal soiling. According to the clinical bowel function scoring system, the patients' scores (7.6 [plusmn] 2.1) were significantly lower than those of the controls (11.4 [plusmn] 0.6; P [lt ] .05). Because of poor fecal continence, 25 patients (55.7%) had to restrict their foods. School absence occurred in 6 (13.3%) patients. Seven patients (15.6%) had problems in peer relationships. According to the Quality-of-Life Scoring Criteria, 86.7% patients had good or fair quality of life. The patients' scores (7.7 [plusmn] 2.9) were significantly lower than those of the controls (11.6 [plusmn] 0.7; P [lt ] .05). And the scores of patients who had fecal soiling and incontinence (6.3 [plusmn] 2.7) were significantly lower than those of patients without fecal soiling and incontinence (8.4 [plusmn] 2.6; P [lt ] .05). Conclusions: Although most patients had good or fair quality of life after surgical correction for HD, the long-term outcome and quality of life are not as good as surgeons expected. The bowel function and quality of life of the patients were poorer than those of healthy children. Fecal soiling is very common and affects patients' quality of life. Long-term regular follow-up is indispensable. Close attention should be paid to minimizing bowel dysfunction for patients with HD postoperatively to improve their quality of life.     

Left-colon antegrade continence enema (LACE) procedure for fecal incontinence

Purpose: Antegrade continence enemas (ACE) are an efficacious therapeutic option for patients with fecal incontinence. The authors review their institution’s experience with a variation of the Monti-Malone ACE procedure using the left colon as a source of an intestinal conduit and enema reservoir.Methods: From 2000 to 2002, 18 patients with fecal incontinence or intractable constipation underwent left-colon ACE (LACE) procedure. Concomitant Mitrofanoff appendicovesicostomy was performed in 15 patients and bladder augmentation in 9. The majority of patients had neural tube defects. A segment of left colon was tubularized, tunneled into the muscular wall of the distal colon, and exteriorized through the left upper quadrant or midabdomen. Stomal catherization and enema installation were started one month postoperatively.Results: Fifteen patients (83%) achieved fecal continence, 2 remain incontinent of stool, and 1 experienced stomal closure (mean follow-up was 24 ± 9 months). Two patients had stomal stenosis that required revision. The mean enema volume in patient’s achieving continence was 360 ± 216 mL, and the mean transit time was 18 ± 12 minutes.Conclusions: LACE is an efficacious procedure for fecal incontinence that can be performed safely at the time of major urologic reconstruction. Administration of enemas into the left colon has several physiologic advantages that result in predictable bowel evacuation.     

Proctocolectomy and J-pouch ileo-anal anastomosis in children

Background/Purpose: The choice of ileo-anal reconstruction method in children undergoing proctocolectomy remains controversial. Although in adults ileo-anal pouch reconstruction has gained overall acceptance, many paediatric surgeons still advocate straight ileo-anal pull-through. The aim of this study was to assess the outcome and long-term functional results in children who have undergone proctocolectomy and ileo-anal anastomosis (IAA) with a J-pouch. Methods: Medical records of 40 consecutive children who had proctocolectomy and J-pouch IAA between 1991 and 1999 were reviewed for early and late complications, fecal frequency, day- and night-time continence, and pouchitis. The indication for surgery was ulcerative colitis (UC) in 29 (median age at operation, 13 years; range, 9 to 16), Hirschsprung's disease (HD) in 10 (median age at operation, 1.5 years; range, 1 month to 5 years), and familial adenomatous polyposis (FAP) in 1 (age at operation, 6 years). Six of the HD patients had primary pull-through for total colonic aganglionosis and 4 a redo operation for failed primary reconstruction of long segment aganglionosis. Results: There were no fatalities. Early complications (wound infection, early bowel obstruction, prolonged fever) occurred in 12 of 29 (41%) and late complications (bowel obstruction 9, pouch fistula 2) in 11 of 29 (38%) of the UC patients. Overall, 16 of 29 (53%) of the UC patients had complications. All patients with early complications were on systemic steroids at the time of the operation. Pouchitis occurred in 30% of the patients. None of the pouches had to be removed. At last follow-up all patients were continent during the day, 2 patients used protective pads during the night because of occasional staining. The median bowel frequency per 24 hours was 4 (range, 2 to 7); only 2 patients (7%) had to empty their bowel during the night. One (10%) of the HD patients had wound infection, and 3 had episodes of postoperative enterocolitis. Pouchitis-type symptoms have not occurred in HD patients. The median bowel frequency for 24 hours was 3 (range, 2 to 5). None of the HD patients needs to evacuate during the night. The 4 HD patients who are older than 3 years of age are continent. Conclusions: J-pouch IAA is a feasible method of reconstruction in children requiring proctocolectomy. Major complication are common but occur mainly in immunosuppressed patients suffering from UC. Despite high incidence of complications, long-term functional results in terms of continence and bowel frequency are excellent and ensure good quality of life in the great majority of patients.     

Cajal间质细胞在先天性巨结肠和巨结肠同源病结肠中的分布研究

目的研究先天性巨结肠（Hirschsprung’s disease，HD）和巨结肠同源病（allied Hirschsprung’s disease，AHD）肠壁内Cajal间质细胞（interstitial cells of Cajal，ICCs）的分布状态，探讨HD和AHD的发病机制。方法选择确诊为HD和AHD的患者各20例，取巨结肠根治术吻合口远端的全层肠壁作为实验组，另取16例正常结肠标本作为对照组。用鼠抗人c—kit单克隆抗体（CD117）标记ICCs，Image Pro-Plus图像分析系统检测ICCs。结果对照组中大量ICCs分布在肌间神经丛周围和环纵肌层内，ICCs包绕神经丛周围，肌层间ICCs连续分布；HD组远端肠管中肌层间和各肌层内ICCs明显减少甚至缺如，与对照组比较差异有统计学意义，P〈0．01；AHD组远端肠管中神经丛大小不一，ICCs分布差异大，大多数神经丛区ICCs减少，环肌层内ICCs明显减少，与AHD组和对照组比较差异有统计学意义，P〈0.01。HD组远端结肠中ICCs减少比AHD组显著，差异有统计学意义，P〈0．01。结论HD、AHD病变肠管中除了神经节细胞的异常外，同时存在ICCs异常；ICCs在HD和AHD的分布不同可能与两者临床症状差异有关；肠管中ICCs数量可能与临床症状及预后有一定关系。     

Improved quality of life by combined transplantation in Hirschsprung's disease with a very long aganglionic segment

Background/Purpose: The treatment of children with Hirschsprung's disease beginning in the proximal jejunum remains a challenge for the pediatric surgeon. These patients need a definitive parenteral nutrition, which could lead to a liver impairment. The goal of this work is to assess the quality of life after combined liver, intestine, and right colon transplantation. Methods: This is a retrospective study of 3 patients. Data regarding symptomatology, radiographic and operating findings, postoperating recovery, and quality of life were analyzed and compared with the quality of life before the transplantation. Results: The suspicion of a very long intestinal aganglionosis should be derived from the intestinal biopsies. Three combined liver, intestine, and right colon transplantation operations have been performed. The immunosuppression included steroids, tacrolimus, and azathioprine. An abdominal pull-trough (Duhamel procedure 2, Swenson procedure 1) was performed from 6 to 24 months after the transplantation. The follow-up after the transplantation ranges from 2 to 6 years. These 3 patients are completely off total parenteral nutrition with bowel movements 2 to 3 times a day. Two patients are continent day and night, and one is continent during the day only. Conclusions: Intestinal transplantation is feasible with good results even when a liver impairment needs a combined intestine and liver transplantation. The right colon transplantation, in our experience, does not impair the results. The quality of life after the transplantation is better than before. J Pediatr Surg 38:422-424.     

Evaluation of anorectal functions of children with anorectal malformations using fecoflowmetry

Background/Purpose: A simple and objective method evaluating the bowel functions of patients with anorectal anomalies is necessary. The authors applied fecoflowmetry and saline enama test for patients with anorectal anomalies to evaluate the fecodynamics and anorectal motilities in these children. Methods: The bowel functions of 16 patients who underwent repair for anorectal malformations and 5 normal controls were evaluated by saline enema test and fecoflowmetry. The correlations between the clinical scores for the bowel functions and the parameters in fecodynamic studies were investigated. Results: Seven of 16 patients exhibited periodical contractions of the rectum synchronized with relaxations of the anal canal during saline infusion as did the controls and had significantly higher clinical scores than the other patients. Two patients with severe chronic constipation lacked rectal contractions. Among the fecoflowmetric parameters, the maximum flow, average flow, and tolerable volume of saline infused into the rectum were significantly lower in the patients with low clinical scores than those of the controls. The maximal squeeze pressure and resting anal pressure were not significantly different between the patients and controls. Conclusion: Fecodynamic studies, such as fecoflowmetry and saline enema test, help in obtaining clinical indicators for the bowel functions of patients with anorectal anomalies.     

Immunolocalization of the gap junction protein Connexin43 in the interstitial cells of Cajal in the normal and Hirschsprung's disease bowel

BACKGROUND: Interstitial cells of Cajal (ICC) are pacemaker cells between gastrointestinal smooth muscles; they generate spontaneous slow waves of the smooth muscle layers and mediate neurotransmission. The cellular network of ICC is connected by Gap junctions to each other and to the smooth muscle cells. Although there have been several studies reporting distribution of ICC in the normal bowel and pathological conditions such as Hirschsprung's disease, there is little information on the crucial role of Gap junctions in the intercellular communication in the gut musculature. The aim of this study was to investigate the immunolocalization of the Gap junction protein Connexin43 in the normal and Hirschsprung's disease (HD) bowel using whole-mount preparation technique and confocal laser scanning microscopy. METHODS: Full-thickness bowel specimens were collected at pull-through operation from 8 patients diagnosed as having HD. Normal control large bowel specimens were collected from 12 patients during bladder augmentation operation. Whole-mount preparation was performed on all specimens and double immunostaining was carried out using anti c-kit and antiConnexin43 antibodies. The immunolocalization was detected with the help of confocal laser scanning microscopy. RESULTS: Connexin43 immunoreactivity appeared in and between the c-kit-positive cells and along the smooth muscle fibers of the normal bowel and ganglionic part of HD bowel. In the aganglionic part of HD bowel there was no expression of Connexin43. In the transitional zone of HD the Connexin43 staining was weak and colocalized only in the processes of the c-kit-positive Cajal cells. CONCLUSIONS: Results of this study show for the first time that Gap junctional protein Connexin43 is present in the ICCs, which form a 3-dimensional network in the normal bowel wall. The lack of expression of Connexin43 in the aganglionic bowel and reduced expression in the transitional zone of HD suggest that the impaired intercellular communication between ICCs and smooth muscle cells may partly be responsible for the motility dysfunction in HD.     

The left Monti-Malone procedure: Preliminary results in seven cases

Background/Purpose: The management of children who have congenital intestinal dysfunction and continuous feces soiling has been improved significantly by the Malone continent caecostomy. When the appendix is not available, the Monti tube represents a good alternative to create a catheterizable conduit. The authors report here 7 cases of left continent colonic access (left Monti-Malone). Methods: From July 1999 to January 2001 7 patients have been operated on in our unit: 6 spina bifida, 1 cloacal exstrophy. Technically, a ring of descending colon of 1.5 to 2 cm width is isolated with its meso, the Monti's tube fashioned, and implanted into the left colon according to the Malone's technique. Antegrade bowel washouts start 3 weeks after surgery with standard saline (200 to 500 mL) [plusmn] phosphate enemas. A period of 1 to 3 months is needed to adjust to the right enema regimen to get a satisfactory result. Results: The follow-up range is 18 months to 1.5 months. One patient is excluded. The duration of the enemas varies between 10 and 30 minutes, and the number of enemas varies from 1 to every 2 or 3 days. Five children are clean and perform their antegrade enemas after dinner to avoid the embarrassment of post enema leakages. Conclusion: Although it is a short series with a short follow-up, the first results collected show a significant shortening of the duration of the enema with excellent outcomes in terms of continence.     

Nutritional outcome and growth of children after intestinal transplantation

Purpose: The aim of this study was to determine nutritional outcome and growth in children after successful intestinal transplantation. Methods: Case-record review was conducted of all children who underwent intestinal transplantation at a single center and retained their grafts for at least 1 year. Supplementary data were obtained from outpatient charts and computerized database. Results: Forty-seven intestinal transplants were carried out in 46 children. There were 19 isolated small bowel and 29 combined liver[ndash ]small bowel transplant procedures. Median age at transplantation was 3.7 years (range, 0.4 to 16.6 years), and median graft survival time was 1,084 days (range, 368 to 3308 days). Nine patients died, and there were 11 graft losses, including those of the nonsurvivors. All survivors with functioning grafts receive all of their calories via the enteral route. There was significant inhibition of linear growth at the time of transplant in the majority of recipients. After successful transplantation, pretransplant growth velocity appeared to be maintained, but there was no evidence of catch-up growth. Conclusions: Intestinal transplantation allows an opportunity for full enteral feedings to be established. There is evidence of severe inhibition of linear growth at the time of transplantation with no evidence of catch-up after transplantation.