TurnerFertility trial: fertility preservation in young girls with Turner syndrome by freezing ovarian cortex tissue-a prospective intervention study.

OBJECTIVE: To evaluate which girls with Turner syndrome (TS) could benefit from fertility preservation by ovarian tissue cryopreservation on the basis of karyotype, puberty status, and hormonal data. DESIGN: Prospective intervention study; participants were included between 2018 and 2020. SETTING: Tertiary hospital in the Netherlands. PATIENTS: In total, 106 girls with TS aged between 2 and 18 years were included. Girls with minor X chromosome deletions, Y chromosomal content, active infections, or contraindications for surgery were excluded. INTERVENTION: A laparoscopic unilateral ovariectomy was performed to obtain ovarian cortical tissue for cryopreservation. One tissue fragment per participant was used to determine the number of follicles per ovary by serial sectioning and staining. Chromosome analysis was performed on lymphocytes and buccal cells. A blood sample was taken before the ovariectomy for hormonal analysis. MAIN OUTCOME MEASURES: The presence of follicles in ovarian cortex tissue from girls with TS in relation to karyotype, puberty status, and hormonal data. RESULTS: A unilateral ovariectomy was performed on 93 girls with TS. Complications after surgery occurred in 5 girls, including luxation of psychological symptoms in 2 girls. In 13 (14%) girls, a 46,XX cell line was found in buccal cells that was absent in lymphocytes. Follicles were observed in 30 (32%) of the 93 girls and were found mainly in girls with a 46,XX cell line in lymphocytes or buccal cells (Phi coefficient = 0.55). Spontaneous onset of puberty (Phi coefficient = 0.59), antimüllerian hormone (AMH; point-biserial correlation [r] = 0.82), inhibin B (r = 0.67), and follicle-stimulating hormone (r = -0.46) levels were also correlated strongly with the presence of follicles. Furthermore, AMH levels had a significant correlation with the number of follicles per ovary (r = 0.66). CONCLUSION: Favorable predictive markers for the presence of follicles included either a 46,XX cell line, spontaneous onset of puberty, or a combination of measurable AMH and normal follicle-stimulating hormone levels. Karyotyping of two peripheral cell lines in girls with TS is recommended to reveal hidden mosaicisms. Ovarian tissue cryopreservation should be offered with caution in a research setting to those with a sufficient ovarian reserve, considering the significant loss of follicles after ovarian tissue cryopreservation and autotransplantation. Physicians should pay attention to the mental health of the girls during the whole process. CLINICAL TRIAL REGISTRATION NUMBER: Trial registration number: NCT03381300- Preservation of Ovarian Cortex Tissue in Girls With Turner Syndrome - Full Text View - ClinicalTrials.gov. Registered on: December 21, 2017. First patient recruited on January 1, 2018.

COllaborative Neonatal Network for the first European CPAM Trial (CONNECT): a study protocol for a randomised controlled trial.

INTRODUCTION: Consensus is lacking on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM). For future studies, the CONNECT consortium (the COllaborative Neonatal Network for the first European CPAM Trial)-an international collaboration of specialised caregivers-has established consensus on a core outcome set of outcome parameters concerning respiratory insufficiency, surgical complications, mass effect and multifocal disease. These outcome parameters have been incorporated in the CONNECT trial, a randomised controlled trial which, in order to develop evidence-based practice, aims to compare conservative and surgical management of patients with an asymptomatic CPAM. METHODS AND ANALYSIS: Children are eligible for inclusion after the CPAM diagnosis has been confirmed on postnatal chest CT scan and they remain asymptomatic. On inclusion, children are randomised to receive either conservative or surgical management. Subsequently, children in both groups are enrolled into a standardised, 5-year follow-up programme with three visits, including a repeat chest CT scan at 2.5 years and a standardised exercise tolerance test at 5 years.The primary outcome is exercise tolerance at age 5 years, measured according to the Bruce treadmill protocol. Secondary outcome measures are molecular genetic diagnostics, validated questionnaires-on parental anxiety, quality of life and healthcare consumption-, repeated imaging and pulmonary morbidity during follow-up, as well as surgical complications and histopathology. This trial aims to end the continuous debate surrounding the optimal management of asymptomatic CPAM. ETHICS AND DISSEMINATION: This study is being conducted in accordance with the Declaration of Helsinki. The Medical Ethics Review Board of Erasmus University Medical Centre Rotterdam, The Netherlands, has approved this protocol (MEC-2022-0441). Results will be disseminated through peer-reviewed scientific journals and conference presentations. TRIAL REGISTRATION NUMBER: NCT05701514.

The Management of Asymptomatic Congenital Pulmonary Airway Malformation: Results of a European Delphi Survey.

Consensus on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM) is lacking, and comparison between studies remains difficult due to a large variety in outcome measures. We aimed to define a core outcome set (COS) for pediatric patients with an asymptomatic CPAM. An online, three-round Delphi survey was conducted in two stakeholder groups of specialized caregivers (surgeons and non-surgeons) in various European centers. Proposed outcome parameters were scored according to level of importance, and the final COS was established through consensus. A total of 55 participants (33 surgeons, 22 non-surgeons) from 28 centers in 13 European countries completed the three rounds and rated 43 outcome parameters. The final COS comprises seven outcome parameters: respiratory insufficiency, surgical complications, mass effect/mediastinal shift (at three time-points) and multifocal disease (at two time-points). The seven outcome parameters included in the final COS reflect the diversity in priorities among this large group of European participants. However, we recommend the incorporation of these outcome parameters in the design of future studies, as they describe measurable and validated outcomes as well as the accepted age at measurement.

Development of a core outcome set for congenital pulmonary airway malformations: study protocol of an international Delphi survey.

INTRODUCTION: A worldwide lack of consensus exists on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM) even though the incidence is increasing. Either a surgical resection is performed or a wait-and-see policy is employed, depending on the treating physician. Management is largely based on expert opinion and scientific evidence is scarce. Wide variations in outcome measures are seen between studies making comparison difficult thus highlighting the lack of universal consensus in outcome measures as well. We aim to define a core outcome set which will include the most important core outcome parameters for paediatric patients with an asymptomatic CPAM. METHODS AND ANALYSIS: This study will include a critical appraisal of the current literature followed by a three-stage Delphi process with two stakeholder groups. One surgical group including paediatric as well as thoracic surgeons, and a non-surgeon group including paediatric pulmonologists, intensive care and neonatal specialists. All participants will score outcome parameters according to their level of importance and the most important parameters will be determined by consensus. ETHICS AND DISSEMINATION: Electronic informed consent will be obtained from all participants. Ethical approval is not required. After the core outcome set has been defined, we intend to design an international randomised controlled trial: the COllaborative Neonatal NEtwork for the first CPAM Trial, which will be aimed at determining the optimal management of patients with asymptomatic CPAM.

Treatment of Congenital Microgastria.

INTRODUCTION: Congenital microgastria is an extremely rare birth defect. The aim of this study was to present an overview of existing literature on the treatment of microgastria. MATERIALS AND METHODS: The term "microgastria" was used in a PubMed and Medline search. Since merely case reports were found, only a narrative synthesis with limited statistical analysis can be given. Data of different treatment modalities were collected and divided into two groups: conservative or less invasive treatment (C/LT, i.e., modified diet or a gastrostomy/jejunostomy) and extensive gastric surgery (EGS, i.e., Hunt-Lawrence pouch or total esophageal gastric dissociation). Clinical outcome parameters (nutrition, growth pattern, and mortality) were compared. RESULTS: Out of 73 articles published from 1973 to 2019, 38 articles describing 51 cases were included. In four patients, microgastria was an isolated anomaly (8%). Type of treatment was described in only 46 patients, 19 were treated by C/LT. Mortality was 9/19 (47%) in the C/LT group versus 4/27 (15%) in the EGS group (chi-square = 5.829, p = 0.016, Fisher = 0.022). There was a negative correlation between the invasiveness of the treatment and both mortality (r = -0.356, p = 0.015) and comorbidity (r = -0.506, p <0.001). Patients in the C/LT group had significantly more comorbidity than in the EGS group (mean = 4.32 vs. 2.26, p = 0.001). There was a positive correlation between comorbidity and mortality (r = 0.400, p = 0.006). Median follow-up was 42 months (range: 1-240). Type and way of nutrition were poorly described. In at least 9 of the 33 surviving patients, oral feeding was reported as normal, of whom 8 belonged to the EGS group. In all patients, growth could be acknowledged, but in comparison to peers, final body length was less. There was no difference in final body length between the two treatment groups. CONCLUSION: In patients with congenital microgastria, only minimal differences in clinical outcome in terms of type of nutrition and body growth were found when C/LT was compared with treatment by EGS. Mortality was significantly higher in the first group as well as the amount of comorbidities.

Risk factors for refractory anastomotic strictures after oesophageal atresia repair: a multicentre study.

OBJECTIVE: To determine the incidence of refractory anastomotic strictures after oesophageal atresia (OA) repair and to identify risk factors associated with refractory strictures. METHODS: Retrospective national multicentre study in patients with OA born between 1999 and 2013. Exclusion criteria were isolated fistula, inability to obtain oesophageal continuity, death prior to discharge and follow-up <6 months. A refractory oesophageal stricture was defined as an anastomotic stricture requiring ≥5 dilations at maximally 4-week intervals. Risk factors for development of refractory anastomotic strictures after OA repair were identified with multivariable logistic regression analysis. RESULTS: We included 454 children (61% male, 7% isolated OA (Gross type A)). End-to-end anastomosis was performed in 436 (96%) children. Anastomotic leakage occurred in 13%. Fifty-eight per cent of children with an end-to-end anastomosis developed an anastomotic stricture, requiring a median of 3 (range 1-34) dilations. Refractory strictures were found in 32/436 (7%) children and required a median of 10 (range 5-34) dilations. Isolated OA (OR 5.7; p=0.012), anastomotic leakage (OR 5.0; p=0.001) and the need for oesophageal dilation ≤28 days after anastomosis (OR 15.9; p<0.001) were risk factors for development of a refractory stricture. CONCLUSIONS: The incidence of refractory strictures of the end-to-end anastomosis in children treated for OA was 7%. Risk factors were isolated OA, anastomotic leakage and the need for oesophageal dilation less than 1 month after OA repair.

Near-Infrared Spectroscopy to Predict the Course of Necrotizing Enterocolitis.

OBJECTIVES: To investigate whether cerebral, liver, and infraumbilical regional tissue oxygen saturation (rSO2) and fractional tissue oxygen extraction (FTOE) could be used to diagnose necrotizing enterocolitis (NEC) and complicated NEC (Bell's stage 3B or death) during its early stages. METHODS: A prospective observational cohort study of preterm infants with suspected or diagnosed NEC. We compared the mean eight-hour cerebral, liver, and infraumbilical rSO2 and FTOE values of infants with no NEC and definite NEC and of infants with uncomplicated and complicated NEC in the first forty-eight hours after onset of symptoms, suspicious for NEC. Furthermore, we determined cut-off values by generating receiver operating characteristics curves in case of significant differences in the first eight-hour mean values of rSO2 between infants with no NEC and definite NEC and between infants with uncomplicated and complicated NEC. RESULTS: We included 33 patients: 13 no NEC, 10 with uncomplicated NEC, and 10 with complicated NEC. We found no significant differences in the first twenty-four hours after onset of symptoms in rSO2 and FTOE between infants with no NEC and definite NEC. In preterm infants with complicated NEC, we observed significantly lower cerebral, liver, and infraumbilical rSO2 and higher FTOE within twenty-four hours after onset of symptoms compared with infants with uncomplicated NEC. A continuous cerebral rSO2 ≤ 71% and liver rSO2 ≤ 59% in the first eight hours after onset of symptoms predicted the onset of complicated NEC with a sensitivity of 1.0 and specificity of 0.8, and a sensitivity of 1.0 and specificity of 1.0, respectively. CONCLUSIONS: By measuring the cerebral and splanchnic oxygenation it is possible to differentiate complicated NEC from uncomplicated NEC. In our sample, NIRS monitoring did not proof useful for distinguishing between definite NEC and no NEC in preterm infants with clinical signs suspicious of NEC.

The relation between splanchnic ischaemia and intestinal damage in necrotising enterocolitis.

OBJECTIVES: The underlying pathophysiology of necrotising enterocolitis (NEC) remains incompletely understood, particularly the role of intestinal perfusion. We aimed to determine the relation between cerebral and splanchnic fractional tissue oxygen extraction (FTOE), a marker for tissue underperfusion, with intestinal fatty acid-binding protein in plasma (I-FABPp), a marker for intestinal damage, in infants with NEC. Furthermore, we investigated the combined courses of cerebral and splanchnic FTOE values and I-FABPp levels in uncomplicated (conservative treatment) and complicated NEC (surgery or death). DESIGN: This study was part of a prospective observational cohort study. PATIENTS: We included 19 preterm infants with NEC (9 uncomplicated, 10 complicated). INTERVENTIONS: Using near-infrared spectroscopy, we measured regional cerebral and splanchnic tissue oxygen saturations continuously for 48 h after NEC onset. We measured I-FABPp levels simultaneously. MAIN OUTCOME MEASURES: We used Spearman correlation tests to calculate correlation coefficients between FTOE values and I-FABPp levels in uncomplicated and complicated NEC. RESULTS: Median (range) gestational age was 28 (25-36) weeks and median (range) birth weight was 1290 (740-2400) g. Cerebral and splanchnic FTOE values correlated strongly with I-FABPp levels (rho between .745 and 0.900; p<0.001-0.037) during the first 16 h after NEC onset. Thereafter, in uncomplicated NEC, splanchnic FTOE values increased while I-FABPp levels decreased concomitantly. In complicated NEC both splanchnic FTOE values and I-FABPp levels decreased. CONCLUSIONS: Combining cerebral and splanchnic FTOE values with I-FABPp levels, gives insight in the pathological chain of events resulting in progression or recovery of intestinal ischaemia in NEC. TRIAL REGISTRATION NUMBER: NTR3239.

Assessing cerebrovascular autoregulation in infants with necrotizing enterocolitis using near-infrared spectroscopy.

BACKGROUND: We assessed cerebrovascular autoregulation (CAR) in preterm infants with definite necrotizing enterocolitis (NEC), Bell's stage 2 or 3, and infants without NEC, using near-infrared spectroscopy. We hypothesized that CAR would be more often impaired in infants with NEC compared with infants without NEC. METHODS: We measured cerebral regional tissue oxygen saturation, arterial oxygen saturation, and mean arterial blood pressure (MABP) during 48 h. We calculated the correlation between cerebral fractional tissue oxygen extraction and MABP for each patient. A statistically significant negative correlation reflected impaired CAR. RESULTS: We included 15 infants with definite NEC (median (range) gestational age 27.4 (25.6-34.7) wk; birth weight 1,070 (670-2,400) g) and 13 infants without NEC (gestational age 27.9 (26.3-34.7) wk; birth weight 980 (640-2,640) g). Fourteen infants had a statistically significant negative correlation (ρ -0.468 to-0.104), of whom five were infants without NEC (5/13; 38%) and nine with definite NEC (9/15; 60%). The difference in prevalence of impaired CAR was not statistically significant. CONCLUSION: Impaired CAR is present in a substantial proportion of infants with definite NEC, which may predispose them to NEC-associated neurological damage.

Intestinal fatty acid-binding protein as a diagnostic marker for complicated and uncomplicated necrotizing enterocolitis: a prospective cohort study.

BACKGROUND: Early NEC symptoms are non-specific and diagnostic tests lack discriminative power. Intestinal fatty acid-binding protein (I-FABP), mainly located in small bowel enterocytes, is released into the blood following NEC-associated enterocyte disruption. Aim of this prospective cohort trial was to determine the diagnostic value of I-FABP measured in plasma (I-FABPp) and urine (I-FABPu) for the presence of NEC, to evaluate I-FABP levels during NEC development, and to assess its prognostic value for the progression from suspected to complicated disease. METHODS: Between 2010 and 2012 we prospectively enrolled neonates with suspected NEC. We measured I-FABP levels eight-hourly from onset of suspected NEC for at least 48 hours, or until surgery. NEC diagnosis was confirmed radiologically or during operation. We defined NEC as complicated if it resulted in surgery and/or death. We determined disease course and diagnostic I-FABP cut-off points. RESULTS: The study comprised 37 neonates (24M, 13F), gestational age 28 (24-36) weeks, birth weight 1190 (570-2,400) grams. We found significantly higher I-FABPp and I-FABPu levels in NEC patients (n = 22) than in patients with other diagnoses (n = 15). Cut-off values for diagnosing NEC were 9 ng/mL I-FABPp and 218 ng/mL I-FABPu, with corresponding likelihood ratios (LRs) of 5.6 (95% CI 0.89-35) and 5.1 (95% CI 0.73-36), respectively. I-FABP levels were highest in the first eight hours after symptom onset and gradually decreased over time. Cut-off values for complicated disease were 19 ng/mL I-FABPp and 232 ng/mL I-FABPu, with LRs of 10 (95% CI 1.6-70) and 11 (95% CI 1.6-81), respectively. CONCLUSIONS: Both plasma and urinary I-FABP levels specifically identify NEC in preterm infants prior to appearance of diagnostic radiological signs suggestive for NEC. Moreover, serial I-FABP measurements accurately predict development of complicated disease.

[A girl with abdominal pain and progressive vomiting]. / Een meisje met buikpijn en progressief braken.

An 11-year-old girl with a history of trichophagia presented with abdominal pain and progressive vomiting. The MRI scan showed a gastric mass and multiple masses in the small intestine. A large trichobezoar was removed from the stomach by laparotomy.

[Infestation with Enterobius vermicularis mimicking appendicitis]. / Appendicitis-beeld door Enterobius vermicularis.

BACKGROUND: Gastrointestinal infestation with the parasite Enterobius vermicularis is common in humans and is usually harmless. Anal pruritus is the most characteristic symptom, but the parasites can cause severe abdominal pain mimicking appendicitis. Early recognition can prevent an unnecessary appendectomy. CASE DESCRIPTION: A six-year-old girl reported to the accident and emergency department with pain in the lower right abdominal region. She was admitted and treated for suspected perforated appendix, following physical examination supplemented with an abdominal CT scan. After antibiotic treatment the symptoms disappeared as did the abscess, apart from a minor amount of residual infiltrate. She was then readmitted twice with recurrent abdominal pain without radiological evidence of an abdominal focus. We decided to conduct a diagnostic laparoscopy and an elective appendectomy à froid. During this procedure living worms were found in the appendix. Treatment with the anthelminthicum mebendazol was effective. CONCLUSION: Gastro-intestinal infestation with E. vermicularis is very common, especially in young children. This infestation is usually harmless, but can mimic appendicitis. This infestation is easily treatable with mebendazol.

Intestinal fatty acid-binding protein in neonates with imminent necrotizing enterocolitis.

BACKGROUND: Intestinal fatty acid-binding protein (I-FABP) is a promising marker for necrotizing enterocolitis (NEC). It can be measured in plasma (I-FABPp) and urine (I-FABPu). Data on the best way to measure I-FABP (in plasma or urine) and the necessity of simultaneous measurement of the urinary creatinine concentration to correct for physiological variations in urine concentration are not available. This holds also true for the reciprocal relation between I-FABPp, I-FABPu and other more conventional laboratory parameters. OBJECTIVES: To evaluate the above-mentioned correlations of I-FABP measurements in neonates with suspected NEC. METHODS: All neonates with suspected NEC were prospectively included. I-FABPp and I-FABPu were analyzed at regular intervals during the first 24 h after onset of symptoms. Correlation and agreement were assessed between these and other parameters (i.e., IL-6, WBC, platelet count, CRP, pH and lactate). RESULTS: Included were 24 boys, 13 girls [median (range) GA 28 weeks (24-36), median birth weight 1,190 g (570-2,400)]. I-FABPu correlated strongly with I-FABPp (r 0.80, p < 0.001) with an adequate agreement. A very strong correlation between I-FABPu and I-FABPu/urine creatinine ratio (r 0.98, p < 0.001) existed. Correlations between I-FABPp/u and conventional parameters were moderate to strong until 8 h after onset of symptoms. CONCLUSION: In neonates with suspected NEC, I-FABPu correlates strongly with I-FABPp, offering an opportunity to choose the most appropriate way of measuring I-FABP. Calculating urinary IFABP/creatinine ratio seems redundant. Moderately strong correlations between I-FABPu and IL-6, WBC and lactate were found.

Abdominal near-infrared spectroscopy in preterm infants: a comparison of splanchnic oxygen saturation measurements at two abdominal locations.

BACKGROUND: Splanchnic tissue oxygenation monitoring has been performed at both the liver and the infra-umbilical regions. It is unknown whether these measurements could be substituted one for the other when interpreting splanchnic oxygenation since they have not been measured simultaneously before. AIMS: To evaluate the feasibility and safety of liver and infra-umbilical near-infrared spectroscopy (NIRS) monitoring in preterm infants with suspected necrotizing enterocolitis (NEC) and to assess the correlation and agreement between NIRS measurements performed simultaneously at the two abdominal locations. STUDY DESIGN AND SUBJECTS: This study was part of a prospective observational cohort study. Preterm infants who were suspected of NEC or who had been diagnosed with NEC were included. OUTCOME MEASURES: Liver oxygen saturation and infra-umbilical oxygen saturation were monitored simultaneously and continuously for 48h by NIRS. RESULTS: NIRS monitoring was performed in 20 out of 24 infants for the entire 48-hour study period. No adverse effects were observed. Values of liver and infra-umbilical oxygen saturation correlated weakly (Spearman's rho=0.244, P<.001). On the Bland-Altman plot liver oxygen saturation was higher than infra-umbilical oxygen saturation (mean difference 6.6%, SD 22.5%). CONCLUSIONS: Using NIRS as method for monitoring oxygen saturation simultaneously in both the liver and infra-umbilical regions is safe and feasible. Additionally, we demonstrated that values of liver and infra-umbilical oxygen saturation cannot be randomly substituted one for the other for the purpose of assessing splanchnic oxygenation.

Clinical importance of a fixed bowel loop in the treatment of necrotizing enterocolitis.

BACKGROUND: The need for surgical treatment in neonates with necrotizing enterocolitis (NEC) is associated with high mortality. Although pneumoperitoneum and progressive disease are generally accepted indications for surgery, it is unclear whether a fixed bowel loop (FBL) should prompt surgery. OBJECTIVE: To determine the relationship between an FBL, type of treatment, and death in the management of NEC. METHODS: Retrospective analysis (January 2000-December 2011) of all neonates with definite NEC in a tertiary neonatal intensive care unit. FBL was defined as a persistent (i.e. >24 h) dilated intestinal segment present on serial abdominal X-rays. RESULTS: NEC was diagnosed in 141 neonates (median gestational age 30 weeks; median birth weight 1,340 g). An FBL was reported in 38 (27%) patients, of whom 18 were treated surgically. Mortality among FBL patients was independent of the type of treatment (surgical versus conservative): 8/18 and 7/20, respectively (p = 0.55). Of the 103 patients without FBL, 37 (36%) were treated surgically, which is comparable to the FBL group. Again, mortality was not related to the type of treatment (surgical versus conservative): 5/37 and 6/66, respectively (p = 0.49). The presence of an FBL was associated with mortality: more patients with an FBL (15/38, 39%) died than without an FBL (11/103, 11%; odds ratio 5.45, 95% confidence interval 2.21-13.45; p < 0.01). CONCLUSIONS: In NEC patients, an FBL is associated with increased mortality. On its own it has moderate significance to guide treatment. Nevertheless, because it reflects disease severity, early recognition is important and prompt (surgical) treatment should be considered.

[Outcome of an intestinal rehabilitation program for children with short bowel syndrome]. / Uitkomsten darmrevalidatieprogramma voor kinderen met kortedarmsyndroom.

OBJECTIVE: To evaluate an intestinal rehabilitation program, introduced in 2001, for children who developed short bowel syndrome (SBS) after extensive intestinal resection. DESIGN: Retrospective cohort study. METHOD: General information on disease status and outcome of intestinal rehabilitation plus demographic data on all SBS patients, treated in the programme between 2001 and 2009, were collected. The outcome measures were intestinal autonomy, intestinal and/or liver transplantation and survival. RESULTS: A total of 10 boys and 9 girls, born at a median gestational age of 36 weeks, were treated according to intestinal rehabilitation programme guidelines. Eight of them had been referred to our centre from elsewhere, 3 times as many as in 1991-2000. The causes of SBS were: intestinal atresia (n = 3), gastroschisis (n = 2), volvulus (n = 9), necrotising enterocolitis (n = 3) or strangulation (n = 2). Median remaining small-intestinal length was 35 cm (range: 10-70 cm). In 14 patients the ileocecal valve was still present and in all 19 patients at least 25% of the colon remained. The median follow-up period was 25 months (range: 50 days-9 years). Following total parenteral nutrition lasting a median 138 days (range: 41 days-11 years), 16 patients (84%) reached intestinal autonomy. Central venous catheter-related complications occurred in all of the patients; liver function disorders in 13 (68%) and failure to thrive in 5 (26%). One patient underwent an intestinal lengthening procedure. None of the patients needed an intestinal transplantation, but one patient underwent a liver transplantation for intestinal failure associated liver disease. Overall mortality was 11%: 2 patients died of abdominal sepsis. CONCLUSION: The specialised intestinal rehabilitation programme led to intestinal autonomy in 84% of the patients with SBS.

Ostomy creation in neonates with acute abdominal disease: friend or foe?

BACKGROUND: An ostomy seems a safe alternative in neonates with an acute abdomen when immediate restoration of bowel continuity is deemed undesirable. Faced with several complications in our center, and the feeling we are not the only center with these complications, we decided to assess the rate and type of complications after both ostomy creation and closure. METHODS: All data regarding neonates (<30 days of age) who underwent a laparotomy for a suspected abdominal emergency in the period 2000 to 2010 were retrospectively analyzed. These data included demographics such as gender, gestational age, and birth weight. Disease etiology was defined and various features of the enterostomy were analyzed. These features included type, location, time to ostomy take down, and complications and mortality directly related to both creation and closure of the ostomy. RESULTS: A total of 155 patients who underwent a laparotomy for suspect acute abdomen were identified. Median gestational age was 33 weeks (range 25 to 40) and median birth weight was 1926 g (range 560 to 4380). Median age at laparotomy was 8 days (range 0 to 30). Indications for surgery were necrotizing enterocolitis (n = 38), spontaneous intestinal perforation (n = 11), intestinal atresia (n = 9) or obstruction (n = 5), and volvulus (n = 4). An ostomy was created in 67 patients (67/155: 43%): 38 boys and 29 girls. There were 8 jejuno-, 49 ileo-, and 10 colostomies created. In almost all cases (94%), a mucous fistula was also constructed.In 23 patients (23/67: 34%) ostomy-related complications occurred. Most frequent were high output ostomy (n = 10) and necrosis of the enterostomy (n = 7). Due to either one of the complications, nine patients (9/67: 13%) needed a reoperation.In this study, 11 patients died before ostomy closure could occur. In 53 patients, the ostomy was closed after a median of 107 days (range 4 to 299).After ostomy closure, complications occurred in 13 cases (13/53: 25%). Seven patients (7/53: 13%) needed another reoperation because of anastomotic leakage (n = 4), adhesions (n = 2), or incisional hernia (n = 1). There was no closure-related mortality. CONCLUSION: Although creating a temporary ostomy in newborns is preferable in certain situations, there is a considerable occurrence of complications and reoperations.