Laparoscopic-Assisted Total Resection and Endorectal Pull-Through Technique for Congenital Megarectum with Anorectal Malformation.

BACKGROUND/PURPOSE: Congenital megarectum (CMR) is sometimes associated with anorectal malformations (ARM), although there is no established therapeutic strategy. This study aims to clarify the clinical features of ARM with CMR, and to demonstrate the effectiveness of a surgical treatment, namely laparoscopic-assisted total resection and endorectal pull-through technique. METHODS: We conducted a review of the clinical records of the patients with ARM with CMR treated at our institution between January 2003 and December 2020. RESULTS: Seven of 33 cases of ARM (21.2%) were diagnosed with CMR, four males and three females. The types of ARM were 'intermediate' in four, and 'low' in three patients. Five of the seven patients (71.4%) required resection of megarectum for intractable constipation and underwent laparoscopic-assisted total resection and endorectal pull-through technique. Bowel function was improved after resection in all five cases. All five specimens showed hypertrophy of the circular fibers, and three of them showed abnormal location of ganglion cells within the circular muscle fibers. CONCLUSIONS: CMR often causes intractable constipation and requires resection of the dilated rectum. Laparoscopic-assisted total resection and endorectal pull-through technique for ARM with CMR considered to be an effective, minimally invasive treatment for intractable constipation. LEVEL OF EVIDENCE FOR CLINICAL RESEARCH PAPERS: Level Ⅳ. TYPE OF STUDY: Treatment study.

Optimum therapeutic strategy for meconium-related ileus in very-low-birth-weight infants.

BACKGROUND/PURPOSE: therapeutic strategy for meconium-related ileus (MRI) in very-low-birth-weight infants (VLBWs) has not been established. This study aims to clarify the optimum therapeutic strategy for MRI in VLBWs. METHODS: MRI was defined as delayed meconium excretion and microcolon on contrast enema with Gastrografin (diatrizoate acid). Forty-two infants with MRI were treated at our institution between 2009 and 2019, and are reviewed here. They were classified into two groups: in group A (n=21), Gastrografin regurgitated into the dilated intestine during the first or second round of Gastrografin enema (GaE), while in group B (N = 21), Gastrografin did not regurgitate. Laparotomy was indicated if the intestine was perforated, or if abdominal distention was not relieved by two rounds of GaE. RESULTS: in group A, meconium was excreted in all cases within 24 h after GaE, and no cases required laparotomy. In group B, twelve cases (57%) underwent laparotomy (P < 0.01), six cases in this group (29%), showed free air on X-ray images (P < 0.01). The median hospital stay in groups A and B were 89.0 and 136.5 days, respectively (P < 0.05). Overall mortality was 2.4%. CONCLUSIONS: early therapeutic diagnosis by GaE followed by early surgery is suggested as the optimum strategy for MRI in VLBWs.

A case of pediatric duodenal transection caused by abuse successfully treated by duodenojejunostomy.

BACKGROUND: Abuse can be a cause of pediatric duodenal injury. Patients who have been injured by abuse tend to have delay before medical examination, they may therefore have especially poor prognosis. CASE PRESENTATION: A 3-year-old boy presented with abdominal pain and was diagnosed with duodenal perforation. He was urgently transferred to our hospital for surgery. There was no clear history of trauma according to initial parent interviews, but old bruises were observed in several places. Paternal remarks about the injury mechanism were contradictory to bruit findings. Eventually, the mother reported daily paternal domestic violence against the patient. Duodenal perforation was considered to be caused by physical abuse, and emergent surgery was carried out. Intraoperative findings revealed transection at the horizontal part of the duodenum. Primary repair was difficult due to severe damage, so duodenojejunostomy was undertaken. CONCLUSION: Duodenojejunostomy was successfully carried out as emergent surgery for severely damaged duodenal transection.

Lactobacillus reuteri DSM 17938 and Magnesium Oxide in Children with Functional Chronic Constipation: A Double-Blind and Randomized Clinical Trial.

OBJECTIVE: Chronic functional constipation is a frequent condition. The aim of the study was to evaluate the efficacy of the probiotic Lactobacillus (L.) reuteri DSM 17938 and magnesium oxide (MgO) for relieving chronic functional constipation in children. STUDY DESIGN: A prospective, double-blind, placebo-controlled, randomized, and parallel-group trial was conducted in five pediatric outpatient clinics in Japan. Sixty patients who were more than six months old and under six years of age with a diagnosis of functional constipation according to Rome IV criteria were randomly divided into three groups: group A (n = 20) received L. reuteri DSM 17938 and lactose hydrate as a placebo of MgO; group B (n = 19) received L. reuteri DSM 17938 and MgO; and group C (n = 21) received a placebo of L. reuteri DSM 17938 and MgO. RESULTS: All three groups exhibited significant improvement in defecation frequency in the fourth week compared with the baseline condition (group A: p < 0.05; group B: p < 0.05; group C: p < 0.05). The MgO group and combination group showed a significant decrease in stool consistency, but the L. reuteri DSM 17938 group did not (group A: p = 0.079; group B: p < 0.05; group C: p < 0.05). MgO significantly suppressed the presence of the genus Dialister. Defecation frequency negatively correlated with the frequency of Clostridiales-belonging bacteria among the gut microbiome. CONCLUSIONS: L. rueteri DSM 17938 and MgO were both effective in the management of functional constipation in young children. MgO caused an imbalance in the gastrointestinal microbiome, which was not the case in the probiotic group.

Associations of Local Social Engagement and Environmental Attributes With Walking and Sitting Among Japanese Older Adults.

This cross-sectional study examined associations of local social engagement with walking and sitting, and whether these associations were modified by local environmental attributes. Older residents (aged 65-84 years, n = 849), recruited from a regional city in Japan, reported walking frequency, sitting time, local social engagement, and local environmental attributes. Walk Score® was also used as an environmental measure. Analysis of data from 705 participants found that engaging in community activities was significantly associated with more frequent walking, but not with prolonged sitting. Interaction analyses between social engagement and environmental attributes did not show any significant interactions, suggesting that promoting local social engagement may increase walking frequency among older adults, regardless of local environmental characteristics. Community-level social initiatives that encourage older adults to participate in local meetings, events, and activities may be an effective physical activity promotion strategy among older adults.

A Community-Wide Walking Promotion Using Maps and Events for Japanese Older Adults.

Objective: This study evaluated the effect of a community-wide walking intervention (involving walking maps and events) on older residents' walking and environmental perceptions. Method: In this nonrandomized quasi-panel study, older adults living in an intervention and control site were recruited at baseline and follow-up. All households in the intervention site received maps and were invited to participate in a walking event monthly. The intervention lasted for 21 months. The outcomes were walking frequency, duration, and perceptions of local environments. Results: We did not find a significant improvement in walking frequency or duration in the intervention site relative to the control site. However, there was a significant increase in the awareness of others being active and a marginal increase in the perception of aesthetics in the intervention site. Discussion: The increased awareness of others being active may be a positive step in the stages of change toward engagement in physical activity.

The impact of a new exercise facility on physical activity at the community level: a non-randomized panel study in Japan.

BACKGROUND: Considering that building a sports facility is a major investment to promote population health, it is important to understand whether it is effective in increasing the level of physical activity (PA) in the community. This study examined the impact of building a new multipurpose exercise facility on community-level PA in Japan. METHODS: This non-randomised panel study compared two sites: an intervention site where a new exercise facility was built (opened after baseline data collection) and a control site where there was no such additional exercise facility. From each site, 3200 adult residents (aged 30-74 years) were randomly selected at baseline (2013) and at follow-up (2015). The number of participants retained for analysis was 845 at baseline and 924 at follow-up for the intervention site, and 821 at baseline and 1018 at follow-up for the control site. The outcomes were participants' self-reported PA, perceived availability of PA facilities, awareness of others being active, and willingness to engage in PA. We examined the interaction terms between the sites and time of measurement in regression analyses to examine whether the magnitude of change from baseline to follow-up differed between the two sites. RESULTS: The changes in the proportion of participants meeting the PA guideline and those engaging in moderate-to-vigorous intensity PA were not significantly different between the intervention and control sites. The intervention site had a greater increase in the proportion of participants who were aware of PA facilities from baseline to follow-up than in the control site. The odds ratio for awareness of others being active approached significance, suggesting that there was a tendency at the intervention site towards a greater increase in the proportion of participants who noticed physically active people. CONCLUSIONS: This study did not find community-level increases in PA after the construction of the exercise facility. However, a significant improvement in the awareness of PA facilities was observed in the intervention site. A sustained community-level effort to promote PA, possibly including social components, and a further tracking of residents' PA are needed to take a full advantage of the new exercise facility and to assess its long-term impact. TRIAL REGISTRATION: UMIN-CTR UMIN000034116 (retrospectively registered: 13 September 2018).

Japanese clinical practice guidelines for allied disorders of Hirschsprung's disease, 2017.

BACKGROUND: Despite the presence of ganglion cells in the rectum, some patients have symptoms similar to those of Hirschsprung's disease. A consensus has yet to be established regarding the terminology for these diseases. We defined this group of diseases as "allied disorders of Hirschsprung's disease" and compiled these guidelines to facilitate accurate clinician diagnosis and provide appropriate treatment strategies for each disease. METHODS: These guidelines were developed using the methodologies in the Medical Information Network Distribution System (MINDS). Of seven allied disorders, isolated hypoganglionosis; megacystis-microcolon-intestinal hypoperistalsis syndrome; and chronic idiopathic intestinal pseudo-obstruction were selected as targets of clinical questions (CQ). In a comprehensive search of the Japanese- and English-language articles in PubMed and Ichu-Shi Web, 836 pieces of evidence related to the CQ were extracted from 288 articles; these pieces of evidence were summarized in an evidence table. RESULTS: We herein outline the newly established Japanese clinical practice guidelines for allied disorders of Hirschsprung's disease. Given that the target diseases are rare and intractable, most evidence was drawn from case reports and case series. In the CQ, the diagnosis, medication, nutritional support, surgical therapy, and prognosis for each disease are given. We emphasize the importance of full-thickness intestinal biopsy specimens for the histopathological evaluation of enteric ganglia. Considering the practicality of the guidelines, the recommendations for each CQ were created with protracted discussions among specialists. CONCLUSIONS: Clinical practice recommendations for allied disorders of Hirschprung's disease are given for each CQ, along with an assessment of the current evidence. We hope that the information will be helpful in daily practice and future studies.

The incidence and outcome of allied disorders of Hirschsprung's disease in Japan: Results from a nationwide survey.

BACKGROUND: Allied disorders of Hirschsprung's disease (ADHD) have been proposed to be the concept of the functional obstruction of the intestine with the presence of ganglion cells in the terminal rectum. They are classified into two categories based on pathology: (1) abnormal ganglia, including immaturity of ganglia, hypoganglionosis (HG), and intestinal neuronal dysplasia; (2) normal ganglia, including megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS), segmental dilatation (SD), internal anal sphincter achalasia (IASA), and chronic idiopathic intestinal pseudo-obstruction (CIIP). Some of these show poor prognosis, therefore, the establishment of criteria and appropriate treatment strategies is required. METHODS: The questionnaires were sent to the 161 major institutes of pediatric surgery or gastroenterology in Japan, in order to collect the cases of ADHD during 10 years from 2001 and 2010. RESULTS: In total, 355 cases were collected. They included 28 immaturity of ganglia, 130 HG (121 congenital, 9 acquired), 18 intestinal neuronal dysplasia, 33 MMIHS, 43 SD, three IASA, and 100 CIIP. Of the 95 institutes, 69 (72.6%) had their own criteria for ADHD. Criteria were based on clinical symptoms and signs, and conventional pathological examinations. Prognosis was poor in congenital HG, MMIHS, and CIIP, while the others showed good survival rates. CONCLUSION: Almost all Japanese cases of ADHD in the past 10 years were collected. Congenital HG and CIIP showed relatively high incidence, whereas acquired HG and IASA were extremely rare in Japan. The criteria of each disorder were also collected and summarized. Prognosis was poor in congenital HG, MMIHS, and CIIP.

Human thioredoxin-1 attenuates the rate of lipopolysaccharide-induced preterm delivery in mice in association with its anti-inflammatory effect.

BACKGROUND: Maternal intrauterine infection/inflammation represents the major etiology of preterm delivery and the leading cause of neonatal mortality and morbidity. The aim of this study was to investigate the anti-inflammatory properties of thioredoxin-1 in vivo and its potential ability to attenuate the rate of inflammation-induced preterm delivery. METHODS: Two intraperitoneal injections of lipopolysaccharide from Escherichia coli were administered in pregnant mice on gestational day 15, with a 3-h interval between the injections. From either 1 h before or 1 h after the first lipopolysaccharide injection, mice received three intravenous injections of either recombinant human thioredoxin-1, ovalbumin, or vehicle, with a 3-h interval between injections. RESULTS: Intraperitoneal injection of lipopolysaccharide induced a rise of tumor necrosis factor-α, interferon-γ, monocyte chemotactic protein 1, and interleukin-6 in maternal serum levels and provoked preterm delivery. Recombinant human thoredoxin-1 prevented the rise in these proinflammatory cytokine levels. After the inflammatory challenge, placentas exhibited severe maternal vascular dilatation and congestion and a marked decidual neutrophil activation. These placental pathological findings were ameliorated by recombinant human thioredoxin-1, and the rate of inflammation-induced preterm delivery was attenuated. CONCLUSION: Thioredoxin-1 may thus represent a novel effective treatment to delay inflammation-induced preterm delivery.

Major neonatal surgery: psychosocial consequence of the patient and mothers.

PURPOSE: Our previous studies of long-term QOL after major neonatal surgery revealed an unexpectedly high incidence of mental retardation as well as emotional and psychosocial problems, and the patients' QOL correlated with maternal QOL and post-traumatic stress disorder (PTSD). The main purpose of this study was to clarify the risk factors affecting long-standing maternal PTSD. MATERIALS AND METHODS: Sixty-eight patients, aged between 6 and 17years, and their mothers were enrolled in this study. The underlying diseases included congenital diaphragmatic hernia in 21, anorectal anomalies in 25 and esophageal atresia in 22. Psychoanalysts carried out intelligence tests, the Child Behavior Checklist and QOL evaluation in the patients. QOL was evaluated with WHOQOL26 and PTSD with IES-R questionnaires in their mothers. The mothers were divided into two groups; low- and high-risk groups according to PTSD score. RESULTS: Patient QOL scores correlated positively with maternal QOL (p<0.05) and negatively with maternal PTSD scores (p<0.05). There were significant differences in total number of hospital admissions (p<0.05), feeling of economic burden (p<0.05), and satisfaction with husband's help (p<0.01) between the two groups. CONCLUSION: Risk factors for maternal PTSD include repeated hospitalization, a feeling of economic burden, and lack of satisfaction with husband's help.

Long-term outcomes of four patients with tracheal agenesis who underwent airway and esophageal reconstruction.

PURPOSE: The aim of this study was to evaluate the long-term outcomes of four patients with tracheal agenesis who underwent airway and esophageal/alimentary reconstruction. MATERIALS AND METHODS: We reviewed the medical records of four long-term survivors of tracheal agenesis and collected the following data: age, sex, type of tracheal agenesis, method of reconstruction, nutritional management, and physical and neurological development. RESULTS: The patients consisted of three boys and one girl, who ranged in age from 77 to 109months. The severity of their condition was classified as Floyd's type I (n=2), II (n=1), or III (n=1). Mechanical respiratory support was not necessary in any of the cases. Esophageal/alimentary reconstruction was performed using the small intestine (n=2), a gastric tube (n=1), and the esophagus (n=1). The age at esophageal reconstruction ranged from 41 to 55months. All of the cases required enteral nutrition via gastrostomy. Three of the patients were able to swallow a small amount of liquid and one was able to take pureed food orally. The physical development of the subjects was moderately delayed-borderline in childhood. Neurological development was normal in two cases and slightly delayed in two cases. CONCLUSIONS: None of the long-term survivors of tracheal agenesis required the use of an artificial respirator, and their development was close to normal. Future studies should aim to elucidate the optimal method for performing esophageal reconstruction to allow tracheal agenesis patients to achieve their full oral intake.

Megacystis microcolon intestinal hypoperistalsis syndrome: A report of a nationwide survey in Japan.

BACKGROUND: Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare condition with a poor outcome. A nationwide survey was conducted to identify the clinical features and outcomes of MMIHS in Japan. METHODS: Data were collected via a questionnaire, which was sent to 353 pediatric/pediatric surgical departments in Japan. RESULTS: Of the 28 patients registered as having a certain diagnosis of MMIHS, 19 (male/female, 3/16) patients were analyzed. All of the patients developed functional bowel obstruction in the neonatal period and showed a distended bladder and microcolon in the radiological examination. A histopathology assessment of the full-thickness of intestinal specimens showed no pathological abnormalities in all patients. Although various medications were given, the patients did not show significant improvement. Drainage stomas were created in the jejunum (n=11) and colon (n=5). Sixteen patients were maintained by parenteral nutrition (PN). Nine patients died of sepsis or liver failure. The five- and ten-year survival rates were 63% and 57%, respectively. CONCLUSIONS: MMIHS patients developed severe functional intestinal obstruction in the neonatal period and had no specific therapeutic intervention. The majority of MMIHS patients required long-term PN. Small bowel or multivisceral transplantation may be necessary to improve the outcome of this condition.

The evaluation of diastolic function using the diastolic wall strain (DWS) before and after radical surgery for congenital diaphragmatic hernia.

OBJECTIVE: The measurement of diastolic wall strain (DWS), a new method of evaluating cardiac diastolic function, was employed to evaluate ventricular diastolic function in patients with congenital diaphragmatic hernia (CDH). MATERIALS AND METHODS: Eighteen neonates with a CDH who were born and treated in our hospital between September 2009 and January 2013 were studied. The left ventricular posterior wall thickness during the systolic phase (PWs) and diastolic (PWd) phase was measured using M-mode imaging, and the DWS was calculated as (PWs-PWd)/PWs. The Tei index, the isovolumic relaxation time (IRT), and the fraction shortening (FS) were measured as indices of cardiac function in 14, 15, and 18 cases, respectively. Cardiac function was measured before and after surgery. Statistical analyses were performed using the paired t test. RESULTS: The pre- and postoperative DWS, Tei index, IRT and FS values were 0.19 ± 0.06 and 0.26 ± 0.11 (P < 0.01), 0.40 ± 0.12 and 0.31 ± 0.11 (P < 0.05), 48 ± 14 and 39 ± 5.0 ms (P < 0.05), 30 ± 7.7 and 34 ± 7.4 % (P < 0.05), respectively. CONCLUSION: The diastolic and systolic functions were not only measured by the Tei index, IRT and FS values, but also by the DWS value, which improved after surgery. The measurement of DWS is an easy and useful method for evaluating the diastolic function of CDH patients.

Parenteral nutrition-associated liver disease in extremely low-birthweight infants with intestinal disease.

BACKGROUND: The aim of this study was to investigate factors associated with the development of parenteral nutrition-associated liver disease (PNALD) and to examine the clinicopathological relationship of PNALD in extremely low-birthweight infants (ELBWI). METHODS: The subjects were 13 ELBWI who had received PN because of intestinal perforation or functional ileus between 2000 and 2013. We measured the serum levels of biochemical parameters, including aspartate aminotransferase, alanine aminotransferase, and direct bilirubin. Liver histopathology was examined in relation to outcome. The subjects were categorized into two groups on liver histopathology: F(+), development of hepatic fibrosis and necrosis with/without cholestasis; and F(-), no hepatic fibrosis. RESULTS: Of 13 ELBWI, five died of hepatic failure, five died of sepsis, and the other three were alive at the time of the study. Of the five infants who died of hepatic failure, two developed fulminant hepatitis without cholestasis, and the other three developed chronic cholestasis and finally hepatic failure. Postmortem histopathology in F(+) indicated not only massive hepatic necrosis, but also massive hepatic fibrosis. These histopathological findings explained the clinical presentation of portal hypertension. There were significant differences in the fasting period after intestinal disease onset between the two groups. CONCLUSION: The prolonged fasting with PN is responsible for severe hepatocellular necrosis with fibrosis and consequent lethal portal hypertension.

Mesenteric hernia causing bowel obstruction in very low-birthweight infants.

Internal hernia through a mesenteric defect, called mesenteric hernia, is an uncommon cause of acute intestinal obstruction in newborns. Strangulated mesenteric hernia results in intestinal necrosis or perforation and progressive deterioration with fatal outcome, especially when it occurs in low-birthweight infants. We report two very low-birthweight (VLBW) infants, who presented with acute intestinal obstruction related to mesenteric defect. The initial diagnosis was meconium obstruction in those cases, which is a common cause of bowel obstruction occurring in VLBW infants. Correct diagnosis of mesenteric hernia was difficult in these cases because of rapid deterioration and non-specific radiological findings. Awareness of the possibility of rare mesenteric hernia causing acute intestinal obstruction and surgical intervention in an appropriate timeframe are important for rescuing VLBW infants with such organic abnormalities.

Efficacy and safety of strong acid electrolyzed water for peritoneal lavage to prevent surgical site infection in patients with perforated appendicitis.

PURPOSE: Our previous experimental study of perforated peritonitis in rats proved that peritoneal lavage with strong acid electrolyzed water (SAEW) has no adverse effects, reduces the bacteria count in the ascitic fluid more effectively than saline, and increases the survival rate significantly. Thus, we conducted a randomized controlled study, applying SAEW in the treatment of perforated appendicitis in children. METHODS: Forty-four patients, aged 3-14 years, were randomly divided into two groups: Group S (n = 20), in which the peritoneal cavity was lavaged with 100 ml/kg saline and the wound was washed out with 200 ml saline; and Group E (n = 24), in which the peritoneal cavity was lavaged with 100 ml/kg SAEW and the wound was washed out with 200 ml SAEW. RESULTS: No adverse effect of SAEW was observed in Group E. There was no difference in the bacterial evanescence ratio of ascitic fluid after lavage between Groups S and E (11.1 and 15.8%, respectively). A residual abscess developed in one patient from each group (5.0 and 4.2%, respectively). The incidence of surgical site infection (SSI) was significantly lower in Group E than in Group S (0 and 20%, respectively; P < 0.05). There was no difference in the duration of pyrexia, positive C-reactive protein, leukocytosis, or hospital stay between the groups. CONCLUSION: Peritoneal lavage and wound washing with SAEW have no adverse effects and are effective for preventing SSI.

Fibroadenoma in Beckwith-Wiedemann syndrome with paternal uniparental disomy of chromosome 11p15.5.

Herein is described a case of breast fibroadenomas in a 16-year-old girl with Beckwith-Wiedemann syndrome (BWS) and uniparental disomy (UPD) of chromosome 11p15.5. She was clinically diagnosed with BWS and direct closure was performed for an omphalocele at birth. Subtotal and 90% pancreatectomy were performed for nesidioblastosis at the ages 2 months and 8 years, respectively. Bilateral multiple breast fibroadenomas were noted at the age of 16 and 17 years. In this case, paternal UPD of chromosome 11p15.5 was identified on microsatellite marker analysis. The relevant imprinted chromosomal region in BWS is 11p15.5, and UPD of chromosome 11p15 is a risk factor for BWS-associated tumorigenicity. Chromosome 11p15.5 consists of imprinting domains of IGF2, the expression of which is associated with the tumorigenesis of various breast cancers. This case suggests that fibroadenomas occurred in association with BWS.

Pediatric chronic intestinal pseudo-obstruction is a rare, serious, and intractable disease: a report of a nationwide survey in Japan.

BACKGROUND/PURPOSE: A nationwide survey was conducted to identify the clinical presentation of pediatric chronic intestinal pseudo-obstruction (CIPO) in Japan. METHODS: Data were collected via a questionnaire, ensuring patient anonymity, from facilities that treat pediatric gastrointestinal diseases in Japan. RESULTS: Ninety-two responses were collected from forty-seven facilities. Sixty-two patients (28 males, 34 females) met formal diagnostic criteria for CIPO. The estimated pediatric prevalence was 3.7 in 1 million individuals. More than half the children (56.5%) developed CIPO in the neonatal period. Full-thickness intestinal specimens were available for histopathology assessment in forty-five patients (72.6%). Forty-one (91.1%) had no pathological abnormalities and were considered to be idiopathic. Patients were treated according to the local protocol of each facility. Forty-one patients (66.1%) had restricted oral intake of ordinary diets, and twenty-nine (46.8%) depended on parenteral nutrition. No therapeutic intervention, including medication and surgery, successfully improved oral food intake or obstructive symptoms. Only three patients (4.8%) died from enteritis or sepsis. CONCLUSIONS: In Japan, pediatric CIPO is a rare, serious, and intractable disease. The prognosis with respect to survival is good, but unsatisfactory because of the need for prolonged parenteral nutrition and associated potential for restricted quality of life.