Intestinal transplantation for short bowel syndrome secondary to gastroschisis

Background/Purpose

Gastroschisis is the most frequent cause of pediatric intestinal transplantation. This study reviews our experience of intestinal transplantation secondary to gastroschisis to elucidate those factors affecting the outcome of children with short bowel syndrome.

Methods

A retrospective review was performed for children who underwent intestinal transplantation for gastroschisis at the University of Miami between June 2003 and August 1994.

Results

Thirty-two transplants were performed in 28 children with gastroschisis during the study period. Associated intestinal anomalies were present in 22 infants (atresia [n = 14], volvulus [n = 3], and/or ischemia [n = 16]). Spontaneous prenatal closure of gastroschisis, a rare anomaly associated with bowel atresia and ischemia because of a very small abdominal defect, was seen in 9 patients. Most of the patients had a complicated course and required multiple abdominal surgeries before transplant. Fifteen (53.6%) patients are currently alive at a median follow-up of 23.5 months. Short-term survival rate has significantly improved in recent years.

Conclusions

Patients with complex gastroschisis and intestinal anomalies have a significant risk for progression to short bowel syndrome. Intestinal transplantation can be a lifesaving option and provides a satisfactory outcome for children with short bowel syndrome secondary to gastroschisis.     

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.     

Experience performing 64 consecutive stapled intestinal anastomoses in small children and infants

Background/Purpose

Intestinal anastomosis in children has traditionally been performed using hand-sewn techniques. Little data exist evaluating the efficacy of stapled intestinal anastomoses in the infant and pediatric populations.

Methods

A review of a 5-year experience using a mechanical stapler to treat 64 consecutive children requiring intestinal anastomoses was performed. An intestinal stapler was used to complete a side-to-side functional end-to-end anastomosis. Postoperative outcomes and modifications made to the technique were identified.

Results

Since 2004, 64 children (median age, 3 months; range, newborn to 24 months) underwent procedures requiring intestinal anastomosis. Twenty-six children (41%) were 1 week or less in age. Twenty-seven children (42%) underwent a stoma closure using a stapler. Thirty-seven children (58%) underwent bowel resection and stapled anastomosis in treating a variety of surgical disorders. Complications included wound infection (n = 2) and anastomotic stricture (n = 1). No issues suggesting anastomotic dilatation and subsequent stasis/overgrowth were identified.

Conclusions

These results suggest that stapled bowel anastomosis is an effective approach applicable to a variety of surgical diseases in newborns and infants.     

Evolutionary experience with immunosuppression in pediatric intestinal transplantation

Background/Purpose

Intestinal transplantation has developed to become the standard of care for patients with irreversible intestinal failure who are not responding to total parenteral nutrition. Once considered experimental, it has taken time and much effort for the procedure to become a clinical reality, with final acceptance primarily because of the vastly improved outcomes. Advances and novel modifications in immunosuppression have been at the forefront of these improvements. The authors review their evolutionary experience with intestinal transplantation, particularly relating changes in immunosuppression protocols to improved outcomes.

Methods

From July 1990 to December 2003, 122 children received 129 intestinal containing allografts (70 liver/intestine, 42 isolated intestine, 17 multivisceral). Mean age was 5.3 ± 5.2 years, and 55% were boys. Indications for transplantation were mostly short gut syndrome. The allografts were cadaveric, ABO identical (except one), with no immunomodulation. Bone marrow augmentation was used in 29% of the recipients since 1995. T-cell lymphoctytotoxic crossmatch was positive in 24% cases. Immunosuppression protocols can be divided into 3 categories: (i) maintenance tacrolimus and steroids (n = 52, 1990-1995, 1997-1998); (ii) addition of induction therapy with cyclophosphamide (n = 16, 1995-1997) then daclizumab (n = 24, 1998-2001). A third immunosuppressive agent was added in either group where increased immunosuppression was indicated; (iii) pretreatment/induction with antilymphocyte conditioning and steroid-free posttransplantation tacrolimus monotherapy (n = 37, 2002-2003). In this later group, if clinically stable at 60 to 90 days posttransplantation, and no recent rejection, the tacrolimus was weaned by decreasing frequency of dosing.

Results

The overall Kaplan-Meier patient/graft survival was 81%/76% at 1 year, 62%/60% at 3 years, and 61%/51% at 5 years. Survival continues to improve, with 1-year patient/graft survival being 71%/62%, 77%/75%, and 100%/100% for groups (i), (ii), and (iii), respectively. Acute intestinal allograft rejection has decreased markedly in group (iii). The rate of infectious diseases, such as cytomegalovirus and Epstein-Barr virus, is lowest in group (iii). Graft-versus-host disease has not significantly increased with the latest protocol. Most importantly, the overall level of immunosuppression requirements has decreased markedly, with most patients in group (iii) being on monotherapy. Of these, most had their monotherapy weaned down to spaced doses, something never systematically attempted or achieved in pediatric intestinal transplantation.

Conclusions

Intestinal transplantation has progressed markedly over the last 13 years. Although there have been modifications in all aspects of the procedure, the story of intestinal transplantation has been the evolution of successful immunosuppression regimens. Our latest pretreatment/induction conditioning and posttransplantation monotherapy strategy improves graft acceptance and lowers subsequent immunosuppression dosing requirements. It is expected this will overcome many of the complications related to the previously high immunosuppression requirements. Minimization of immunosuppression with avoidance of steroid therapy offers profound long-term benefits, especially in the pediatric population. The patients still remain challenging and complex in every aspect; however, these advances offer significant hope to both patients and caregivers alike.     

A comparison of circumumbilical and transverse abdominal incisions for neonatal abdominal surgery

Purpose

Although neonatal bowel surgery traditionally involves a transverse abdominal incision, several authors have reported that the circumumbilical incision is effective and cosmetically appealing. We report the first study comparing the circumumbilical incision to the transverse abdominal incision for a variety of neonatal abdominal operations.

Methods

Retrospective cohort analysis comparing the circumumbilical incision to the transverse abdominal incision for neonates who underwent surgical repair of malrotation, duodenal atresia/web, or intestinal atresia/web was performed between 1999 and 2009.

Results

One hundred thirty-two patients underwent a laparotomy through a transverse abdominal incision (n = 106) or a circumumbilical incision (n = 26). Baseline characteristics between groups were similar. No differences were found when comparing operative time, postoperative days on a ventilator, narcotic infusion, time to full feeds, length of hospital stay, incidence of surgical site infection, and bowel obstruction. Although more incisional hernias occurred in the circumumbilical incision group (38%) than the transverse abdominal incision group (6%), all hernias in the circumumbilical group resolved without intervention, whereas 33% required surgical repair in the transverse abdominal group.

Conclusions

Because of its cosmetic advantages and similar outcomes to the transverse abdominal incision, the circumumbilical incision should be considered as an alternative to the transverse abdominal approach in neonatal surgery.     

Serum growth factors and growth indices pre- and post-pediatric intestinal transplantation

Background/purpose

Although intestinal transplantation (ITx) has succeeded in liberating children with intestinal failure from total parenteral nutrition (TPN), positive growth has yet to be achieved in the majority of patients. This investigation aims to evaluate levels of serum growth factors as they relate to growth parameters and nutritional outcomes.

Methods

Serum measures of insulin-like growth factor 1 (IGF-1) and insulin-like growth factor binding protein 3 (IGFBP-3) that had been obtained before and after transplantation were reviewed (with Institutional Review Board approval) in a subset of pediatric ITx recipients. Z-scores for weight and height were calculated at transplant and biannually thereafter for 2 years.

Results

Five children received a small bowel/liver transplant between August 1996 and March 2000 (median age, 1.3 years). Before transplantation, levels of IGF-1 and IGFBP-3 were low in 60% and 67% of patients, respectively. Posttransplant levels of these growth factors were within normal limits or elevated in all but 2 patients (IGFBP-3 only). A positive trend in z-scores was observed in just one of 5 patients for weight and in 2 of 5 for height/length during the follow-up period. Of the 3 patients who experienced negative linear growth velocity over time, 2 had low pretransplant levels of both IGF-1 and IGFBP-3. All patients were weaned from TPN within 3 months after transplant.

Conclusions

Pretransplant levels of growth mediators may be predictive factors in children who will require an intensive regimen of nutritional rehabilitation posttransplant to promote the growth process. Absorption studies may aid in determining the appropriate nutrient substrates for the post-ITx population.     

Definitive surgical management of antenatally diagnosed exomphalos

Background/Purpose

The management of exomphalos is controversial with many centers in the United Kingdom and elsewhere advocating a conservative nonsurgical approach for the larger examples. Nevertheless, this approach is not without problems or complication. The aim of the study was to ascertain the outcome of all infants with an antenatally diagnosed exomphalos treated recently at our institution using a policy of aggressive abdominal wall closure.

Methods

This is a retrospective review of all infants with exomphalos treated from January 1995 to September 2002.

Results

There were 35 infants, all of whom underwent surgery. These were separated into 3 groups: group A (all exomphalos minor) underwent primary closure (n = 11), group B (exomphalos major) underwent primary closure (n = 13), and group C (exomphalos major) underwent staged closure involving a silo (n = 11). Infants in group C had a lower birth weight (P = .05) and were less mature (P = .06). They required longer periods of ventilation (P < .001), a longer hospital stay (P = .001), and a longer period to achieve full enteral feeds (P < .001). Overall survival was 34 (97%) of 35 infants. One premature infant who was born with a ruptured exomphalos sac (birth weight, 862 g) died of nonsurgical complications (sepsis and respiratory failure) early after the creation of a silo.

Conclusions

An aggressive surgical approach in infants with exomphalos is a safe option resulting in effective abdominal wall closure. This requires a skilled multidisciplinary approach and possibly greater resources than other options.     

Intestinal and multivisceral transplantation in children with severe gastrointestinal dysmotility

Background/Purpose

Severe gastrointestinal dysmotility (GID) impairs patients' quality of life and is almost uniformly fatal after complications of parenteral nutrition. Intestinal and multivisceral transplants have been used as alternative treatment of these disorders. We studied patients with GID treated with transplantation in our center, and reviewed their outcome to determine the therapeutic efficacy of multivisceral transplants.

Methods

The transplant database was searched for patients with GID from 1994 to 2001. We excluded patients with Hirschsprung disease, scleroderma, and diabetic enteropathy. We reviewed explanted organs, histochemistry, and immunohistochemistry and classified cases by etiology.

Results

We selected 12 children with GID from 124 patients transplanted. Nine presented before 1 year and 3 started with symptoms between 2 and 8 years. By combined clinical and histopathological features, 6 were classified as megacystis microcolon intestinal hypoperistalsis syndrome, 4 as chronic idiopathic intestinal pseudoobstruction, and 2 as intestinal neuronal dysplasias. Six patients died during the follow-up from 21 to 546 days after transplant. The Kaplan-Meier actuarial survival rates were 66.7% at 1 year and 50% at 3 years.

Conclusions

Multivisceral transplantation is a valuable therapeutic alternative for children with severe GID who cannot be adequately managed with parenteral nutrition.     

Antenatal magnetic resonance imaging is useful in providing predictive values for surgical procedures in abdominal wall defects

Background

Antenatal magnetic resonance imaging (MRI) is useful for the diagnosis of abdominal wall defects. Its predictive value concerning the possibility of primary closure of the abdominal wall, however, has so far not been reported.

Methods

Between August 2001 and November 2004, antenatal MRI was performed on 9 patients with abdominal wall defects in whom surgical repair was performed immediately after birth. Areas of the abdominal cavity and exteriorized viscera were manually traced from both sagittal and axial MR images, and the data were further transmitted to a Workstation for MRI Volumetry (Advantage Windows 4.1, General Electric Medical Systems, Milwaukee, Wis). We examined the exteriorized ratio (ER), which is calculated by dividing the absolute volume of the abdominal cavity by that of the exteriorized viscera, and evaluated the predictive value by a retrospective comparison with surgical procedure.

Results

In the primary closure group (n = 5), mean values of ER were 0.33 ± 0.31 from axial and 0.45 ± 0.31 from sagittal MR images. In contrast, in the staged closure group (n = 4), mean values of ER were 1.39 ± 0.40 from axial and 1.34 ± 0.42 from sagittal MR images. There was a significant difference (P < .05) between the 2 groups for both sets of images. The ER obtained from antenatal MRI correlated well with surgical procedure.

Conclusions

The ER might be useful for antenatal counseling, planning for delivery, and prediction of the most likely surgical procedure.     

No free ride? The hidden costs of delayed operative management using a spring-loaded silo for gastroschisis

Purpose

The ideal management of gastroschisis (primary vs staged closure) has not yet been established. Despite the ease of silo placement, anecdotal experience shows that silos do not always offer benefit. The aim of this study was to highlight concerns regarding use of spring loaded silos and compare outcomes to primary closure.

Methods

Thirty-seven neonates with gastroschisis treated with either primary (n = 10) or staged closure with a spring-loaded silo (n = 27) were reviewed (1998-2007). Variables included ventilator days, daily intravenous fluid, hospital days, and complication rates. SPSS (SPSS Inc, Chicago, Ill) was used to perform t test and χ² analyses (significance P < .05).

Results

Survival for primary closure was 100% (10/10) compared to 89% (24/27) for staged closure (P = .548). Patients managed with silos required prolonged ventilation (16.1 ± 4 days vs 3.6 ± 1 days; P ≤ .05) and greater intravenous fluids on days 3, 4, and 5 of life (132 ± 25 mL/kg per day vs 104 ± 18 mL/kg per day; P ≤ .01). Although there was no difference in the complication rates between the groups, several problems were evident in the silo group: 15% (4/27) required silo replacement, 44% (12/27) required fascial defect enlargement for silo placement, and 19% (5/27) required mesh at closure. No significant differences in recovery of intestinal function were observed. Three silo patients developed ischemic complications because of vascular insufficiency at the level of the abdominal wall, leading to significant intestinal loss, ventilator and total parenteral nutrition dependence, and increased hospital stay.

Conclusions

Patients managed with a silo had longer ventilator requirements and greater fluid needs. This Specific technical complications leading to bowel ischemia were notable in the silo group. The silo should be carefully placed to avoid bowel twisting and the funnel effect. Larger prospective studies should be performed to provide decision-making criteria for the use of a silo vs primary closure.     

Staged reduction of gastroschisis using preformed silos: practicalities and problems

Purpose

Previous single-center studies have reported favorable outcomes when preformed silos (PFS) are used for the staged reduction of gastroschisis. The aim of this study was to assess the frequency and nature of complications associated with PFS in a large population and provide an insight into the practicalities of their routine use.

Methods

A retrospective review was carried out of all cases of gastroschisis managed with PFS in 4 UK neonatal surgical units for a 6-year period.

Results

One hundred fifty infants were included, and 139 (92.7%) silos were applied at cot side (no sedation, n = 93). Median silo size was 4 cm, and time of application was 2.5 hours. Enlarging the defect by incision of fascia was required in 17 (11%). Defect closure was performed at a median of 4 days (0-47) with 93 (62%) being at cot side. Methods of closure were adhesive strips/dressings (n = 94), sutures (n = 48), and patch (n = 8). Discoloration of the viscera occurred in 16 (11%), managed successfully by simple methods (change of PFS, aspirating the stomach, or incision of the defect fascia) (n = 8), conversion to operative silo (n = 3), and operative reduction (n = 1). Four required bowel resection. Other complications included missed atresia (n = 5; 3.3%) and nectrotizing enterocolitis (n = 11; 7%). There were 5 deaths in the series (3.3%).

Conclusions

Staged reduction of gastroschisis with PFS is simple, convenient, and safe. The low rates of associated complications and mortality appear favorable when compared to infants managed with more traditional techniques. We recommend that PFS should be used for the routine management of gastroschisis.     

High diagnostic yield of gastrointestinal endoscopy in children with intestinal failure

Purpose

Children with intestinal failure (IF) often have gastrointestinal (GI) symptoms, including bleeding, increased stool output, and feeding intolerance. The use of endoscopic assessment of these symptoms has not been previously reported. This report evaluates the diagnostic yield of GI endoscopy in the setting of IF.

Methods

After institutional review board approval, we reviewed the medical records (including endoscopy, pathology and microbiology data) of patients with IF who underwent GI endoscopies between September 1999 and March 2007.

Results

Twenty-seven patients underwent 61 GI endoscopies: 34 esophagogastroduodenoscopies, 17 colonoscopies, 7 flexible sigmoidoscopies, and 3 ileoscopies. Indications for endoscopy, which were not mutually exclusive, included chronic diarrhea (39%, n = 24), GI bleeding (36%, n = 22), suspected bacterial overgrowth (36%, n = 22), and suspected peptic disease (15%, n = 9). Based on gross endoscopic appearance, histopathology, or microbiology, 43 (70%) procedures yielded abnormalities. These included infectious (20%, n = 12), anatomical (18%, n = 11), peptic (15%, n = 9), allergic (15%, n = 9), and other (2%, n = 1) findings. Eleven (73%) of 15 duodenal cultures grew a spectrum of 17 bacterial species. Overall, 24 (89%) of 27 patients had gross endoscopic, histopathologic, or microbiologic abnormalities.

Conclusions

In pediatric patients with IF, diagnostic upper and lower GI endoscopies yield high rates of abnormalities and can help guide clinical management.     

Abdominal foregut perforations in children: a 10-year experience

Purpose

Intraabdominal foregut perforations in children are rare. We conducted a retrospective review with the aim of defining their etiologies, treatment, outcomes, and prognosis.

Methods

Abdominal foregut perforations treated during a 10-year period were reviewed. Perforations secondary to blunt or penetrating trauma and inadvertent perforations sustained, recognized, and repaired during surgical procedures, were excluded.

Results

Fourteen perforations were identified, including 1 esophageal, 8 gastric, and 5 duodenal perforations. Seven perforations (50%) occurred in the neonatal period. Of the 7 neonates, 3 (43%) were premature. Nine patients (64%) had significant comorbidities. Six (43%) perforations were spontaneous, 5 (36%) were iatrogenic, and 3 (21%) were direct complications of underlying conditions. Primary repair of the perforation was completed in 11 patients (79%), and staged repair in 1 patient. Significant morbidities related to the perforation occurred in 36% of patients. Twelve patients (86%) survived to discharge. The 10 long-term survivors are on full oral feeds.

Conclusions

Pediatric abdominal foregut perforations have diverse etiologies. Half occur in neonates, evenly divided between term and premature babies. Major comorbidities are common. One third are iatrogenic and potentially preventable. Primary repair should be performed when feasible. Patients who survive the initial complications have excellent long-term outcomes.     

Comprehensive Surgical Intestinal Rescue and Transplantation Program in Adult Patients: Bologna Experience

Introduction

Surgical approaches to complicated benign intestinal failure are accepted worldwide, especially in the pediatric population. Intestinal transplant surgery is thought to rescue patients in whom complications of total parenteral nutrition (TPN) develop.

Objective

To report our experience with surgical intestinal rescue in an adult population with intestinal failure.

Patients and Methods

An intestinal rehabilitation program initiated at our institution included comprehensive medical rehabilitation, surgical bowel rescue, and transplantation. From 2000 to 2009, of 81 adult patients referred by our gastroenterologists for bowel rehabilitation, 42 (51,8%) underwent 43 transplantations (32 isolated intestinal grafts and 11 multivisceral grafts). Underlying diseases were primarily short-bowel syndrome, Gardner syndrome, and intestinal pseudo-obstruction. Thirty-nine patients (48,2%) underwent surgical rescue (40 cases) consisting of bowel resection, adhesiolysis, stricturoplasty, liver transplantation with portocaval hemitransposition (6 cases in 5 patients). Underlying diseases were primarily intestinal fistulas, stenosis, or perforations, short-bowel syndrome, cocoon syndrome, and complete portal thrombosis.

Results

After a mean (SD) follow-up of 1043 (1016) days, in the transplantation population, 21 patients (50%) are alive, with a 1-, 3-, 5-year patient survival of 76%, 59%, and 52%, respectively, and graft survival of 66%, 54%, and 48%, respectively. After 901 (404) days in the rescue population, 32 patients (82%) are alive (2 died, and 5 were lost to follow-up); in 75%, TPN 25% was discontinued, and are receiving oral feeding with TPN support. The 1- and 3-year survival rate was 100% and 83%, respectively.

Conclusions

Deaths occurred primarily in the transplantation population. Intestinal surgical rescue, when possible, is optimal.     

Sentinel lymph node biopsy in the pediatric population

Background

Sentinel lymph node biopsy (SLNB) has only been recently used for childhood neoplasms.

Methods

We reviewed all patients younger than 19 years who underwent SLNB for 5 years.

Results

Twenty patients were identified (11 male, 9 female). Sentinel lymph node biopsy was performed for 10 sarcomas (5 synovial, 3 rhabdomyosarcoma, 1 epitheliod, 1 other); 9 skin neoplasms (4 melanomas, 3 Spitz nevi, 2 melanocytomas); and 1 acinic cell carcinoma. All patients underwent Technetium 99m sulfur microcolloid injection and 4-quadrant subdermal injection with Lymphazurin 1% (Autosuture, Norwalk, Conn). Six patients required either sedation for lymphoscintigraphy. Intraoperative gamma probe was used. Primary lesions were found in lower extremity (n = 8), upper extremity (n = 6), trunk (n = 3), and head and neck (n = 3). The lymphatic basins were inguinal (n = 8), axilla (n = 8), neck (n = 3), and both inguinal and axilla (n = 1). At least one lymph node was identified in each procedure. Of 20 patients, 5 (25%) had metastatic disease (4 skin neoplasms and 1 sarcoma). There were no complications in our series, and all patients are alive with no recurrence at an average follow-up of 2.2 years.

Conclusions

Sentinel lymph node biopsy allows for an accurate biopsy in children. However, some younger patients may require sedation, and it may be more challenging to isolate the sentinel node.     

Recurrent pancreatitis caused by pancreatobiliary anomalies in children with annular pancreas

Purpose

Annular pancreas (AP) is usually associated with duodenal obstruction in neonates. Pancreatitis with AP occurs frequently in adults but is rare in children. This article describes pancreatitis in children with AP and pancreatobiliary anomalies and its surgical treatment.

Patients and Methods

Six children who underwent duodenal bypass for AP subsequently developed recurrent pancreatitis. Three had trisomy 21. Duodenoduodenostomy had been performed in 5 patients and gastrojejunostomy in 1 patient for neonatal duodenal obstruction. We reviewed overall management, imaging, and surgical treatment in these children.

Results

All children subsequently complained of recurrent abdominal pain. Pancreatitis developed in 6 children, and magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography (ERCP) revealed associated pancreatobiliary anomalies such as pancreas divisum, pancreatobiliary malunion, choledochocele, and intraluminal duodenal diverticulum. In 5 cases, surgery for recurrent or chronic pancreatitis was performed. The range of follow-up was 11 to 54 months, and all children who underwent surgery had excellent results.

Conclusions

Children with AP occasionally require reoperation for recurrent pancreatitis because of associate pancreatobiliary anomalies. Magnetic resonance cholangiopancreatography and ERCP provide excellent images of pancreatobiliary anomalies. Intraoperative cholangiopancreatography is also essential for accurate depiction of the ductal structure and selection of the appropriate surgical procedure.     

Evaluation of intestinal absorption after longitudinal intestinal lengthening for short bowel syndrome

Purpose

The aim of this study was to answer if the longitudinal intestinal lengthening and tailoring (LILT) by Bianchi, modified by Aigrain, can allow the child to be weaned from parenteral nutrition (PN) and if the length of the bowel after the procedure can influence the results of the absorption test such as Schilling or d-xylose test.

Patients and Methods

We reviewed the files of 7 children who have had LILT from 1980 to 2003. We performed to explore 2 intestinal function tests: the d-xylose and the Schilling tests. Both were performed early (during the first year after the procedure) and late (during the second year) after the LILT. We used the χ² and Bartlett's correlation tests for statistical analysis.

Results

There were 6 boys and 1 girl. The surgical indication was short bowel syndrome with parenteral nutrition owing to multiple intestinal atresia (2 cases), severe necrotizing enterocolitis with volvulus (1 case), necrotizing enterocolitis (1 case), intestinal atresia with gastroschisis (2 cases), and volvulus owing to malrotation (1 case). The length of the bowel was significantly different before and after LILT (P < .0001). After LILT, the length of the bowel was significantly correlated with the percentage of PN on energy at 6 months (P = .02) and at 12 months (P = .001). Moreover, the length of the bowel after the procedure was significantly correlated with the results of the d-xylose test during the first year (P = .002) but not with the results after the second year. The length after lengthening influenced neither the results of the Schilling test during the first nor those of the second year after. Four patients were weaned from the PN 21 months in average after the LILT (57%); 1 was not because we had only a 2-month follow-up. The average follow-up was 111 (5 months; range, 4- 206).

Conclusion

Longitudinal intestinal lengthening and tailoring for short bowel syndrome is a good option to allow children to be weaned from the PN. The length of the bowel after the procedure can influence the absorption test such as d-xylose during the first postoperative year but not during the second and does not influence the Schilling test. We think it is not necessary to perform these tests during the follow-up of these patients.     

Morbidity and mortality of colostomy and its closure in children

Background

This study evaluated the complications of colostomy and its closure in infants and children.

Methods

One hundred forty-six colostomies were performed in 86 neonates, 23 infants, and 37 children older than 1 year. These children underwent colostomies for anorectal malformation (84), Hirschsprung’s disease (47), and other miscellaneous (15) conditions like colonic atresia, volvulus, rectal tuberculosis, traumatic rectal perforation, and intestinal obstruction caused by ascariasis.

Results

Of these, 17 (11.6%) had early complications, and 80 (69.8%) had stomal complications. Three patients died, but only 1 death was directly related to colostomy. Colostomy prolapse, peristomal excoriation, and malnutrition were the major complications. The complications were not dependant on the children’s age or primary indication. Sigmoid colostomy had a lower malnutrition rate than transverse colostomy (34.9% v 16.9% P = .009). Among the 56 children who underwent colostomy closure, major complications include death (1.8%), anastomotic leak (7.1%), and wound infection (12.6%).

Conclusions

A divided sigmoid colostomy should be performed whenever possible. Proper stomal care, regular nutritional assessment, and early closure of the colostomy would minimize morbidity and mortality of colostomy and its closure.     

Correlation of complex ascites with intestinal gangrene and perforation in neonates with necrotizing enterocolitis

Background/Purpose

The purpose of this study was to determine whether a correlation exists between the finding of complex ascites on ultrasound (US) and the presence of intestinal perforation or gangrene in neonates with complicated necrotizing enterocolitis (NEC).

Methods

Charts of neonates with NEC (n = 76) whose care involved consultations with the pediatric surgery service between 2005 and 2008 were reviewed. Twenty-three babies with NEC without free air had a bedside abdominal US. Neonates with pneumoperitoneum were excluded from the study because this was an absolute indication for surgical intervention.

Results

Twelve of the 23 neonates who had a bedside abdominal US were found to have ascites with debris or complex ascites. One of these 12 patients improved with medical management, and the ascites resolved. One infant with complex ascites had an initial laparotomy that revealed extensive bowel necrosis and gangrene that required intestinal resection and ostomy creation. This infant survived and is currently doing well.Ten patients were critically ill and were managed with bedside peritoneal drainage. Of those, 7 had drainage of intestinal contents after placement of the drain. Two of the babies who had a drain placed for complex ascites subsequently died of progressive disease. Five neonates with ascites with debris improved after peritoneal drainage and were subsequently subjected to laparotomy. All had gangrene with intestinal perforation. Three infants with complex ascites and intestinal contents were not observed during the initial peritoneal drainage. They improved after peritoneal drainage and had laparotomy. Free intestinal perforation was not demonstrated. The 3 infants in this group survived.

Conclusions

The presence of complex ascites with debris correlated well with intestinal gangrene or perforation. This correlation may also be a predictor of mortality. Neonates with complicated NEC without clear indication for surgical intervention would benefit from bedside abdominal US evaluation.     

Isolated liver and multivisceral transplantation for total parenteral nutrition-related end-stage liver disease

Purpose

Total parenteral nutrition (TPN) has prolonged survival in children with intestinal failure; however, end-stage liver disease owing to TPN-induced cholestasis (ESLD-TPN) may preclude its use. ESLD-TPN is an indication for isolated liver transplantation (ILT) or multivisceral transplantation (MVT). Isolated liver transplantation for ESLD-TPN should only be considered in patients who have the potential for enteral autonomy.

Methods

We retrospectively reviewed the records of patients with ESLD-TPN who underwent ILT (n = 7) or MVT (n = 5) between 1994 and 2005. The median age at the time of transplantation was 10.0 months. Intestinal failure followed necrotizing enterocolitis (n = 3), gastroschisis (n = 3), gastroschisis with volvulus (n = 3), gastroschisis with atresia (n = 1), malrotation (n = 1), and megacystis microcolon intestinal hypoperistalsis syndrome (n = 1).

Results

Isolated liver transplant patients had a median length of small bowel of 70 cm and tolerated a median of 50% of enteral calories. The median length of small bowel in patients who underwent MVT was 29 cm, and none tolerated more than 30% of goal enteral feeds. Reduced-size (n = 5) and whole-liver (n = 2) allografts were used for patients undergoing ILT. Patients undergoing MVT received liver-small bowel-pancreas (n = 4) or liver-small bowel-pancreas-colon (n = 1). Overall patient survival was 57% in ILT (median follow-up = 25.1 months); 3 survivors are TPN independent, and the fourth patient requires TPN 3 days/wk. Patient survival was 40% after MVT (median follow-up = 13.0 months); 1 MVT patient died of abuse 16.9 months after transplant and was TPN independent at the time of death. Both survivors are TPN independent. Bilirubin levels are within normal range in all survivors.

Conclusion

Isolated liver transplantation for ESLD-TPN in the setting of intestinal failure is a viable option in patients who have the potential for enteral autonomy. Multivisceral transplantation is the only alternative in patients without the potential for intestinal recovery. Survival can be achieved in patients with ESLD-TPN, but mortality remains high for both procedures.