Circumcision and Risk of Febrile Urinary Tract Infection in Boys with Posterior Urethral Valves: Result of the CIRCUP Randomized Trial.

BACKGROUND: Boys with posterior urethral valves (PUVs) have an increased risk of febrile urinary tract infections (fUTIs). Circumcision is believed to reduce the risk of fUTIs in boys, although there are no randomized trials demonstrating this. OBJECTIVE: To determine the effect of circumcision on the risk of fUTIs in boys with PUVs. DESIGN, SETTING, AND PARTICIPANTS: A clinical randomized trial that ran between August 2012 and July 2017 was conducted. The trial was multicentric, including 13 referral centers for pediatric urology. Male boys, aged 1-28 d, diagnosed with posterior urethral valves, confirmed by voiding cystogram, were included. The exclusion criteria included presence of a genital malformation contraindicating performing a circumcision. INTERVENTION: Participants were randomized to neonatal circumcision + antibiotic prophylaxis (CATB) or antibiotic prophylaxis alone (ATB), and followed for 2 yr. OUTCOME MEASUREMENTS AND STATISTICAL ANALYSIS: The primary outcome was a risk of presenting fUTIs in each group. An fUTI was defined as fever (>38.5 °C) with evidence of pyuria and culture-proven infection on urinalysis, obtained by urethral catheterization or suprapubic aspiration. A bivariate analysis of the primary outcome was performed using the Kaplan-Meier method. RESULTS AND LIMITATIONS: In total, 91 patients were included: 49 in group CATB and 42 in group ATB. The probability of presenting an fUTI was 20% in group ATB versus 3% in group CATB. The hazard ratio of presenting an fUTI within 2 yr in the ATB group compared with that in the CATB group was 10.3 (95% confidence interval: 1.3-82.5). Sixty-four children (70.3%) had a complete follow-up at 2 yr of age. CONCLUSIONS: Circumcision significantly decreases the risk of presenting an fUTI in boys with PUVs. PATIENT SUMMARY: In this report, we compared, in a multicentric trial, the number of febrile urinary tract infections (UTIs) in boys with posterior urethral valves who had either antibiotic prophylaxis alone or antibiotic prophylaxis and circumcision. We found that those who had a circumcision had a significantly lower risk of febrile UTIs.

Incidence and survival of childhood cancer in the French islands of Reunion and Mayotte (2005-2011).

The aim of this study is to describe childhood cancer incidence and survival in the French islands of Reunion and Mayotte for the period 2005-2011. Data were taken from the population-based Cancer Registry of Reunion Island. All incident cases of malignant tumours and benign tumours of the Central Nervous System diagnosed between 2005 and 2011 in children under the age of 15 and living in Reunion or Mayotte were included. A total of 236 cases were registered (176 in Reunion, 60 in Mayotte). Age-standardised incidence rates (ASRs, world standard) for all cancers were 125.0 and 101.8 per million for Reunion and Mayotte, respectively. ASRs for the main cancer groups were lower than those described in mainland France for the same period. The 5-year overall survival rate for all patients was 78.5% (95%CI 71.9- 83.7), slightly lower than that reported in mainland France.

Male infants with hypospadias and/or cryptorchidism show a lower 2D/4D digit ratio than normal boys.

BACKGROUND: In humans the ratio of the index finger to the ring finger is sexually dimorphic, with the mean ratio being larger in women than in men. It has been suggested that this difference is related to prenatal androgen exposure. This has been further demonstrated in children with congenital adrenal hyperplasia. Normal development of the male external genitalia is linked to androgen-mediated events during gestation. We therefore wanted to determine if the 2D:4D digit ratio was normal in boys with cryptorchidism or hypospadias. METHODS: We prospectively enrolled all prepubertal patients seen in the outpatient clinic for cryptorchidism or hypospadias between September and December 2012. We then compared their 2D:4D digit ratio with two control groups made up of normal boys and normal girls. Interobserver and intraobserver variability was evaluated. RESULTS: We included 57 boys with hypospadias and/or cryptorchidism, 79 boys without genital abnormalities and 25 girls without genital abnormalities. The mean 2D:4D ratio for both hands was significantly different between the three groups, with the digit ratio for boys with genital anomalies being lower than for normal boys and normal girls (p<0.0001). CONCLUSIONS: It appears that boys with genital abnormalities (cryptorchidism and/or hypospadias) have a lower 2D:4D digit ratio than boys without genital anomalies.

Assessment and outcome of children with intestinal failure referred for intestinal transplantation.

BACKGROUND & AIMS: Chronic intestinal failure (CIF) requires long term parenteral nutrition (PN) and, in some patients, intestinal transplantation (ITx). Indications and timing for ITx remain poorly defined. In the present study we aimed to analyze causes and outcome of children with CIF. METHODS: 118 consecutive patients referred to our institution were assessed by a multidisciplinary team and four different categories were defined retrospectively based on their clinical course: Group 1: patients with reversible intestinal failure; group 2: patients unsuitable for ITx, group 3: patients listed for ITx; group 4: patients stable under PN. Analysis involved comparison between groups for nutritional status, central venous catheter (CVC) related complications, liver disease, and outcome after transplantation by using non parametric tests, Mann-Whitney tests, Kruskal-Wallis, Wilcoxon signed rank tests and chi square distribution for percentage. RESULTS: 118 children (72 boys) with a median age of 15 months at referral (2 months-16 years) were assessed. Etiology of IF was short bowel syndrome [n = 47], intractable diarrhea of infancy [n = 37], total intestinal aganglionosis [n = 18], and chronic intestinal pseudoobstruction [n = 17]. Most patients (89.8%) were totally PN dependent, with 48 children (40.7%) on home-PN prior to admission. Nutritional status was poor with a median body weight at -1.5 z-score (ranges: -5 to +2.5) and median length at -2.0 z-score (ranges: -5.5 to +2.3). The mean number of CVC inserted per patient was 5.2 (range 1-20) and the mean number of CRS per patient was 5.5 (median: 5; range 0-12) Fifty-five patients (46.6%) had thrombosis of ≥2 main venous axis. At admission 34.7% of patients had elevated bilirubin (≥50 µmol/l), and 19.5% had platelets <100,000/ml, and 15% had both. Liver biopsy performed in 79 children was normal (n = 4), or showed F1 or F2 fibrosis (n = 29), bridging fibrosis F3 (n = 20), or cirrhosis (n = 26). Group 1 included 10 children finally weaned from PN (7-years survival: 100%). Group 2 included 12 children with severe liver disease and associated disorders unsuitable for transplantation (7-years survival: 16.6%). Group 3 included 66 patients (56%) who were listed for small bowel or liver-small bowel transplantation, 62/66 have been transplanted (7 years survival: 74.6%). Factors influencing outcome after liver-ITx were body weight (p < .004), length (p < .001), pre-Tx bilirubin plasma level (p < .001) and thrombosis (p < .01) for isolated ITx, Group 4 included 30 children (25.4%) with irreversible IF considered as potential candidates for isolated ITx. Four children were lost from follow up and 3 died within 2 years (survival 88.5%). Among potential candidates, the following parameters improved significantly during the first 12 months of follow up: Body weight (p.0001), length (p < .0001) and bilirubin (p < .0001). CONCLUSIONS: many patients had a poor nutritional status with severe complications especially liver disease. PN related complications were the most relevant indication for ITx, but also a negative predictor for outcome. Early patient referral for Tx-assessment might help to identify and separate children with irreversible IF from children with transient IF or uncomplicated long-term PN, allowing to adapt a patient-based treatment strategy including or not ITx.

Severe vaginal laceration in a 5-year-old girl caused by sudden hydro-distention.

BACKGROUND: Severe vaginal trauma in young girls is uncommon. We describe a 5-year-old girl who presented a vaginal laceration following sudden hydro-distention. CASE: A 5-year-old girl was transferred to our institution for vaginal trauma with severe bleeding. Her brother had directed the nozzle of a functioning garden hose against her vulva. She presented pain and persistent vaginal bleeding without external genital lesions. Surgical exploration revealed a laceration of the right vaginal wall. The vagina was sutured and the bleeding stopped. SUMMARY AND CONCLUSION: This case illustrates a rare mechanism of severe vaginal laceration, in a young girl, by hydro-distention. Though there are often no external lesions the internal lesions can be severe causing significant bleeding. Certainly in these cases aggressive diagnostic evaluation is necessary.

Cortical transit time as a predictive marker of the need for surgery in children with pelvi-ureteric junction stenosis: preliminary study.

INTRODUCTION: Postnatal management of prenatally detected hydronephrosis remains controversial. It has been suggested that cortical transit time (CTT) could successfully predict deterioration in children with pelvi-ureteric junction (PUJ) obstruction. We decided to conduct a retrospective study in our hydronephrosis population to evaluate whether initial CTT was significantly correlated with the need for surgery. PATIENTS AND METHOD: We reviewed the charts of all our patients managed for significant PUJ obstruction (>12 mm) between 2007 and 2010 and determined CTT retrospectively, on the first diuretic scan of each of these patients. We then determined the relationship between initial CTT and the need for surgery. RESULTS: We identified 37 patients with hydronephrosis (pelvic size >12 mm) of which 26 were diagnosed prenatally. Out of 22 patients with an initial abnormal CTT, 20 underwent surgery. Out of 15 children with a normal initial CTT, 4 underwent surgery (OR 27.5 (IC95%: 4.3-174.9)). CONCLUSION: Initial CTT could be a reliable prognostic factor for future evolution of renal function in children with hydronephrosis. CTT is easy to determine on diuretic renal scan. A prospective trial is being devised to confirm what role it could have in the management of children with hydronephrosis.

Combined laparoscopic-assisted nephrectomy, augmentation ureterocystoplasty and Mitrofanoff appendicovesicostomy.

Conventional and robotic-assisted laparoscopy is being used for more and more complex urological procedures in children. There have recently been reports of laparoscopic or laparoscopic-assisted appendicovesicostomies in children. We report a case of combined laparoscopic-assisted nephrectomy, augmentation ureterocystoplasty and Mitrofanoff appendicovesicostomy in a 5-year-old boy with valve bladder syndrome.

Delayed diagnosis of neurological bladder following spinal cord injury without radiological abnormality.

Spinal cord injuries are a well-known cause of neurological bladder and a well-known complication of high-velocity trauma. They can, however, be difficult to diagnose in young children, in whom the absence of initial neurological symptoms and normality of the radiological workup can be falsely reassuring. Management of associated injuries can further delay diagnosis. We present 2 cases of spinal cord injuries without radiological abnormality revealed by delayed bladder retention.

[Management of pediatric liver abscess]. / Abcès hépatique chez l'enfant : à propos d'un cas.

Liver abscess in the pediatric population remains uncommon in developed countries, except in cases of septicemia or in children with major debilitating diseases, granulocyte dysfunction, or immunosuppression. Although much is known about the etiopathogenesis of liver abscess, the gold standard of investigations and treatment is still debatable in developing countries. We report the case of a 6-year-old child living in Reunion Island, with no medical history, presenting with right and pyretic abdominal pain in the right upper quadrant. Ultrasound and CT scan showed a large hypodense nonenhanced area in segment IV. Final diagnosis was, by exclusion, pyogenic liver abscess based on negative serology, recent liver lesion, and normal tumor test results, even if blood culture remained negative. No percutaneous puncture was done because of positive outcome after 4 days of antibiotics. Treatment consisted in three intravenous antibiotics (ceftriaxone, aminoxide, and metronidazole) until complete biological normalization. Ultrasound remained normal 3 months later. Even if liver abscess is uncommon in developing countries, the diagnosis must be raised in cases of isolated liver tumor with fever. Management in the nonimmunosuppressed child must be discussed associating parenteral antibiotic therapy, percutaneous drainage, or surgery in very uncommon cases, according to the liver location and first day's progression. Etiological investigation such as colonoscopy in adults must be adapted to pediatric data.

Nutritional outcome in children with severe generalized recessive dystrophic epidermolysis bullosa: a short- and long-term evaluation of gastrostomy and enteral feeding.

BACKGROUND: Generalized recessive dystrophic epidermolysis bullosa (RDEB) is often complicated by high nutritional difficulties with risks of malnutrition. OBJECTIVES: To provide information regarding the benefits of enteral feeding by gastrostomy (GTF), energy and protein requirements, tolerance, growth and pubertal development in children with RDEB. METHODS: Twenty-four patients were referred over a 7-year period in a retrospective study. Gastrostomy placement was decided in patients unable to feed orally and/or presenting loss in weight and height of at least 1 SD compared with their best growth level, despite regular nutritional advice. Weight and height were expressed as Z-scores. Catch-up growth following GTF onset was studied. RESULTS: Gastrostomies were performed in 11 children (aged 9·0±5·8years), and one young man aged 18years. The body weight Z-score was -2·3±1·0, height Z-score 1·1±1·1, weight-for-height was 81±11% and height-for-age 95± 4%. At onset, GTF provided 74±21% and 180±81% of the recommended dietary allowance (RDA) for energy and proteins, respectively. At study update (53±20months), GTF provided 91±29% and 205±100% of RDA for energy and proteins, respectively. Weight-for-height reached 92±15% and height-for-age 98±5%. A normal puberty was obtained when GT was performed before the age of 10years. Skin was not improved. CONCLUSION: Malnutrition was observed in 50% of the children with generalized RDEB. Protein and energy needs are particularly high. GTF is well tolerated and helps with catch-up growth and puberty. It must be considered before malnutrition onset, and, if necessary, before puberty.

Penile length is comparable in boys with and without hypospadias.

INTRODUCTION: It has been suggested that penile length is negatively correlated to the degree of hypospadias; however, there are no studies in the literature actually comparing penile length in normal patients and patients with hypospadias. MATERIAL AND METHOD: Between January and May 2011, we measured penile length in two groups of children aged up to 5 years. The first group comprised all boys admitted for hypospadias (40 patients; 25 distal and 15 proximal hypospadias). The control group comprised 100 boys seen for other surgical procedures excluding those with endocrine disorders. We measured the length of the dorsal aspect of the penis in the flaccid state, without stretching. We then established, using our control group, a range of 'normal' values for penile length in our population and determined whether children with hypospadias were within this 'normal' range. RESULTS: Mean penile length in the control group was 4.07 ± 0.92 cm, mean penile length in the hypospadias group was 4.36 ± 0.9 cm (4.48 ± 0.89 cm for distal hypospadias and 4.21 ± 0.79 cm for proximal hypospadias). There was no difference in penile length between the hypospadias group and the control group regardless of degree of hypospadias. CONCLUSION: This study indicates that penile length in children with hypospadias whether proximal or distal is within the normal range.

Giant hepatic cysts: prenatal findings and uncommon postnatal outcome.

With modern prenatal imaging, liver cysts are being diagnosed more often. Although large cysts are usually asymptomatic, they may present as an abdominal emergency requiring surgery in the first weeks of life. We report a series of 3 patients with prenatal diagnosis of isolated cystic liver lesions diagnosed at 22, 31 and 33 weeks of gestational age. The hepatic origin of the cysts was confirmed prenatally by a MRI in 2 cases, with visualization of a normal gallbladder. The prenatal course was uneventful. Postnatal ultrasound confirmed the diagnosis of liver cyst, showed normality of the biliary tract and in one case, rupture of the cyst during delivery. Because of an uncommon rapid increase in size, the 3 children underwent surgical excision of the cysts within the first weeks of life. These were non-bile-containing intrahepatic cysts arising from segment IV. Long-term follow up was uneventful.

Normal oxidative phosphorylation in intestinal smooth muscle of childhood chronic intestinal pseudo-obstruction.

BACKGROUND: Chronic intestinal pseudo-obstruction (CIPO) is a severe disease of the digestive tract motility. In pediatric population, CIPO remains of unknown origin for most patients. Chronic intestinal pseudo-obstruction is also a common feature in the course of mitochondrial oxidative phosphorylation disorders related for some patients to mutations in TYMP, POLG1, mtDNA tRNA(leu(UUR)) or tRNA(lys) genes. We hypothesized that CIPOs could be the presenting symptom of respiratory chain enzyme deficiency and thus we investigated oxidative phosphorylation in small bowel and/or colon smooth muscle of primary CIPO children. METHODS: We studied eight children with CIPO and 12 pediatric controls. We collected clinical, radiological and pathological data and measured respiratory chain enzymatic activity in isolated smooth muscle of the small bowel and/or the colon. We also sequenced TYMP, POLG, mtDNA tRNA(leu(UUR)) and tRNA(lys) genes. KEY RESULTS: Neither pathological nor radiological data were in favor of a mitochondrial dysfunction. No respiratory chain enzyme deficiency was detected in CIPO children. In myogenic CIPO, respiratory enzymes and citrate synthase activities were increased in small bowel and/or colon whereas no abnormality was noted in neurogenic and unclassified CIPO. Levels of enzyme activities were higher in control small bowel than in control colon muscle. Sequencing of TYMP, POLG, mtDNA tRNA(leu(UUR)) and tRNA(lys) genes and POLG gene did not reveal mutation for any of the patients. CONCLUSIONS & INFERENCES: The normal enzymatic activities as the lack of radiological and genetic abnormalities indicate that, at variance with adult patients, oxidative phosphorylation deficiency is not a common cause of childhood CIPO.

The return of the solitary testis.

PURPOSE: To assess what is done and what is recommended concerning fixation of the solitary testis. MATERIAL AND METHOD: We conducted an e-mail survey of current practices in 28 pediatric surgery departments in 28 university or general hospitals in France. We then reviewed what evidence could be found in the literature. RESULTS: All surgeons fix the contralateral solitary testis following intravaginal torsion. Sixteen out of 28 fix the contralateral solitary testis following extravaginal torsion, 13/28 in cases of monorchia, and 8/28 following orchiectomy for trauma or tumour. Five surgeons have observed one case each of torsion of a solitary testis, and three have witnessed testicular necrosis following orchiopexy. CONCLUSION: There is no consensus regarding fixation of the remaining testis in the literature. Scientific evidence does not show clearly whether fixation is necessary, regardless of the clinical situation. However, if fixation is performed it should be done using the dartos pouch sutureless technique.

[Haemorrhagic shock after severe abdominal trauma in children: knowing when to change from conservative to surgical management]. / Choc hémorragique après traumatisme abdominal grave chez l'enfant : savoir opter pour l'attitude interventionnelle chirurgicale.

Abdominal vascular injuries following a serious falling out are quite rare in children. They can lead to haemorrhagic shock whose etiological diagnosis may be difficult in children in case of multiple trauma. The current management of abdominal injuries in the child is usually conservative, surgery being indicated in haemodynamically unstable patients. We report the case of a 7-year-old girl who presented with abdominal trauma with rupture of the hepatic artery and shredding of the splenic vein following a falling out of 10 meters. Aggressive resuscitation associated with early laparotomy for haemostasis, contrary to usual practices advocated in such a context, have helped control the hemorrhagic shock and stabilize the haemodynamic status of the child. The subsequent evolution was favourable, with full recovery. While a conservative attitude usually prevails in the management of traumatic intra abdominal bleeding in children an interventional attitude with emergency surgery must be sometimes considered.

[Fertility preservation before puberty: from mice to men]. / Préservation de la fertilité avant la puberté : de la souris à l'homme.

Malignant tumors account for 1% of childhood cancers. The incidence is to the order of 122 cases per million children. The five-year survival after cancer before the age of 16years has improved from 50 to 80% in 40years. Assessment of potential for preservation of fertility should thus be a systematic element of care for children treated for a malignant tumor (high-dose chemotherapy with alkylizing agents, radiation therapy including the gonads) or those receiving hematopoietic stem cell grafts for malignant or benign disease (sickle-cell anemia, immune deficit). Among the techniques proposed, cryopreservation of ovarian tissue appears to be the most promising, or perhaps the only one available before puberty with encouraging result. Nevertheless the uncertainties, or even risks, related to these treatments, should not be neglected.

Preserving fertility in prepubertal children.

BACKGROUND: As a result of advances in treatment, almost 80% of children and adolescents who currently receive a diagnosis of cancer become long-term survivors. Potential adverse consequences of treatment include impaired puberty and fertility due to gonadal removal, genital tract injury or damage to germ cells from adjuvant therapy. In recent years, treatment of solid tumors and hematological malignancies has been modified in an attempt to minimize damage to the reproductive system. Simultaneously, advances in assisted reproductive technologies have led to new possibilities for the prevention and treatment of infertility. We review experimental data in animal models and clinical experience in adults and discuss strategies to preserve fertility in prepubertal children. CONCLUSIONS: Fertility preservation should now be considered in children facing cancer treatment that has a high risk of gonadal toxicity including high-dose chemotherapy and bilateral irradiation of the gonads at toxic doses.

Is intestinal transplantation the future of children with definitive intestinal insufficiency?

UNLABELLED: Intestinal transplantation (IT) is the newest and most difficult of organ transplantations. The first ever (1987) and the longest surviving (1989) IT were performed in our institution. However, IT still has to demonstrate its benefit to children on long-term parenteral nutrition (PN). We tried to clarify this aspect by looking back at our 13 years' experience. PATIENTS: From 1994 to December 2007, 74 IT were performed in 69 children, 39 with an isolated small bowel (IT), 35 combined with a liver transplant (LITx). The indications were: short bowel syndrome (n = 25), congenital mucosal diseases (n = 22), and motility disorders (n = 22). Median age at transplantation was 5 years (1 - 17 years). Follow-up was 1 to 12 years (median 5 years). RESULTS: Thirty-one children have a functioning graft (42 %), 15/39 IT, 16/35 LITx. They are at home without PN, with a good quality of life. One child is PN-dependent 1.5 years post IT. Post IT, 16 children were detransplanted: 12 early on (1 for mechanical complications, 11 because of resistant rejection; 3 less than 3 years, one 9 years post SBT (chronic rejection). In 2 noncompliant teenagers, PN was reintroduced (one was detransplanted later on). Several years post LITx, 2 children underwent bowel detransplantation due to an acute viral infection complicated with rejection. Twenty-two children died (32 %, 8 IT, 14 LITx), 18 early on from infectious or surgical complications, 4 more than 1 year post IT, 3 after retransplantation (1 in another unit). Bad prognostic factors are multiple previous surgeries, an older age (> 7 y), and chronic intestinal pseudo-obstruction. DISCUSSION: Complications post IT are frequent and life-threatening, especially early on: rejection (IT), infections (LITx). Later on, the rate of complications decreases but remains significant, especially in noncompliant patients. However we describe here a 13-year learning curve; the recent results are encouraging with regard to control of rejection and viral infections. CONCLUSION: Intestinal transplantation is indicated only in selected patients in whom long-term PN cannot be performed safely any more. In every child with intestinal insufficiency, the therapeutic strategy must be discussed early on in order to perform IT at the right time under optimal conditions. IT should evolve from being a "rescue" procedure to becoming a true therapeutic option.

Emergency management and conservative surgery of ovarian torsion in children: a report of 40 cases.

INTRODUCTION: The authors describe and discuss the clinical and therapeutic features of 40 ovarian torsions (OT) in children with its urgent treatment that has advanced in recent years. MATERIALS AND METHODS: A retrospective study of 40 cases of OT in 38 children under 16 years of age, excluding adnexal torsions in neonates. RESULTS: Abdominal and/or pelvic pain was the presenting symptom ; 8 of these children had pain between 2 to 9 months prior to surgery and 27/40 (67.5%) had associated vomiting. Before the procedure, ultrasound (US) diagnosed 29 ovarian lesions, related to 14 mature teratomas (MTE) and 10 cystadenomas (CA), one association of MTE and CA in the same ovary, 2 functional cysts and 2 malignant neoplasms. 19/40 torsions could benefit from conservative management. Eleven torsions occurred, 10/11 of these ovaries had an increased volume, and 5/11 had US evidence of small subcortical cysts. Three detorsions with incomplete removal of CA were followed by enlargement of the tumor and re-torsion in 2 of them. Five children had bilateral ovarian pathology which led to unilateral ovariectomy, while the other benefited from conservative treatment. CONCLUSIONS: In any girl presenting with abdominal pain, the diagnosis of an ovarian torsion must be considered. US is performed emergently, but only surgery, most often a laparoscopic procedure, assures diagnosis. The treatment of the torsion is an emergency and must be as conservative as possible in order to preserve the ovarian function. Bilateral torsions are not unusual.

Long-term outcome, growth and digestive function in children 2 to 18 years after intestinal transplantation.

OBJECTIVE: Small bowel (SB) transplantation (Tx), long considered a rescue therapy for patients with intestinal failure, is now a well recognised alternative treatment strategy to parental nutrition (PN). In this retrospective study, we analysed graft functions in 31 children after SBTx with a follow-up of 2-18 years (median 7 years). PATIENTS: Twelve children had isolated SBTx, 19 had combined liver-SBTx and 17 received an additional colon graft. Growth, nutritional markers, stool balance studies, endoscopy and graft histology were recorded every 2-3 years post-Tx. RESULTS: All children were weaned from PN after Tx and 26 children remained PN-free. Enteral nutrition was required for 14/31 (45%) patients at 2 years post-Tx. All children had high dietary energy intakes. The degree of steatorrhoea was fairly constant, with fat and energy absorption rates of 84-89%. Growth parameters revealed at transplantation a mean height Z-score of -1.17. After Tx, two-thirds of children had normal growth, whereas in one-third, Z-scores remained lower than -2, concomitant to a delayed puberty. Adult height was normal in 5/6. Endoscopy and histology analyses were normal in asymptomatic patients. Chronic rejection occurred only in non-compliant patients. Five intestinal grafts were removed 2.5-8 years post-Tx for acute or chronic rejection. CONCLUSIONS: This series indicates that long-term intestinal autonomy for up to 18 years is possible in the majority of patients after SBTx. Subnormal energy absorption and moderate steatorrhoea were often compensated for by hyperphagia, allowing normal growth and attainment of adult height. Long-term compliance is an important pre-requisite for long-term graft function.