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This article presents a case study of a neonate with a rare vascular tumor called a rapidly involuting congenital hemangioma (RICH). The patient developed a condition called Kasabach-Merritt phenomenon, which caused severe thrombocytopenia and coagulopathy. The case highlights the challenges in managing such cases and suggests that tumor size may correlate with thrombocytopenia. The study emphasizes the importance of a multidisciplinary team in making treatment decisions and the potential complications of large RICHs. The research was supported by the Italian Ministry of Health and the authors have no conflicts of interest. [Extracted from the article]

Background: The impact of perioperative care interventions on postreconstructive complications and short-term colorectal outcome in patients with anorectal malformation (ARM) type rectovestibular fistula is unknown., Methods: An ARM-Net consortium multicenter retrospective cohort study was performed including 165 patients with a rectovestibular fistula. Patient characteristics, perioperative care interventions, timing of reconstruction, postreconstructive complications and the colorectal outcome at one year of follow-up were registered., Results: Overall complications were seen in 26.8% of the patients, of which 41% were regarded major. Differences in presence of enterostomy, timing of reconstruction, mechanical bowel preparation, antibiotic prophylaxis and postoperative feeding regimen had no impact on the occurrence of overall complications. However, mechanical bowel preparation, antibiotic prophylaxis ≥48 h and postoperative nil by mouth showed a significant reduction in major complications. The lowest rate of major complications was found in the group having these three interventions combined (5.9%). Multivariate analyses did not show independent significant results of any of the perioperative care interventions owing to center-specific combinations. At one year follow-up, half of the patients experienced constipation and this was significantly higher among those with preoperative mechanical bowel preparation., Conclusions: Differences in perioperative care interventions do not seem to impact the incidence of overall complications in a large cohort of European rectovestibular fistula-patients. Mechanical bowel preparation, antibiotic prophylaxis ≥48 h, and postoperative nil by mouth showed the least major complications. Independency could not be established owing to center-specific combinations of interventions., Type of Study: Treatment study., Level of Evidence: III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose: Congenital diaphragmatic hernia (CDH) presents with a broad spectrum of severity, depending on the degree of pulmonary hypoplasia and persistent pulmonary hypertension (PPH). It is currently not clear whether pulmonary hypertension may affect late morbidity. Aim of the present study was to evaluate the influence of PPH on mid-term morbidity in high-risk CDH survivors., Methods: All high-risk (prenatal diagnosis and/or respiratory symptoms within 6 h of life) CDH survivors, treated between 2004 and 2008 in our Department were followed up in a multidisciplinary outpatient clinic as part of a longitudinal prospective study. Auxological, gastroesophageal, pulmonary and orthopedic evaluations were done at specific time-points (at 6, 12, and 24 months of age). Patients were grouped depending on the presence/absence of pulmonary hypertension (defined by expert pediatric cardiologists after echocardiography). Paired t-test and Fisher's exact test were used as appropriate. P < 0.05 was considered significant., Results: 70 survivors out of a total of 95 high-risk CDH infants treated in our Department attended our follow-up clinic and were prospectively evaluated. 17 patients were excluded from the present study because no clear data was available regarding the presence/absence of PPH during the perinatal period. Moreover, 9 infants were not enrolled because they did not reach at least 6 months of age. A total of 44 survivors were finally enrolled since they met the inclusion criteria. 26 infants did not present with PPH during the first hospital admission, while 18 had PPH. The 2 groups did not differ with regard to any of the outcomes considered at follow-up (p > 0.2)., Conclusion: In our cohort of high-risk CDH survivors, PPH was not found to affect late sequelae at mid-term follow-up. This may indicate that postnatal pulmonary development is not (always) influenced by perinatal PPH. Nevertheless, a longer follow-up and more patients are needed to properly quantify possible late problems in high-risk CDH survivors with associated neonatal PPH., (© Georg Thieme Verlag KG Stuttgart · New York.)

Background/purpose: As long as the survival rate of patients with abdominal wall defects (AWD) increases, information about long-term follow-up is becoming necessary. Even though quality of life in these patients, in absence of associated anomalies, appears to be unaffected, respiratory impairment soon after birth has been documented; therefore, participation in sports rarely is addressed., Methods: Eighteen patients, ranging in age from 7 to 18 years, operated on at birth for large abdominal wall defects (> 4 cm for gastroschisis; >6 cm for omphalocele) were asked to come for a stress test on a treadmill, with measurements of time of exercise (TE), maximal oxygen consumption (VO2 max) and continuous recording of vital parameters. Respiratory function also was assessed by Forced Vital Capacity (FVC)., Results: Ergometric data were compared with those of a normal pediatric population. All patients were able to perform the stress test with no cardiovascular abnormalities detected at rest or on exertion. Maximum heart rate was reached after a significantly shorter TE, and VO2 max was significantly reduced when comparing normal subjects with AWD subjects and AWD subjects in sports with those sedentary. FVC was only slightly reduced in AWD patients without reaching statistical significance., Conclusions: These findings indicate that patients operated on for AWD at birth exhibit a normal cardiorespiratory function; decreased TE and VO2 max are likely to reflect a lack of physical activity with a lower degree of fitness. Therefore, no limitations to motor performances should exist for these patients. Well-being may be greatly improved by regular exercise., (Copyright 2003, Elsevier Science (USA). All rights reserved.)

Gastrointestinal motility disorders are frequently found in several pathologies. The aim of this study was to assess, by means of electrogastrography, the presence of gastrointestinal motility abnormalities in children affected by Crohn's disease (CD) or Chronic Intestinal Pseudo-Obstruction (CIPO). Patients and Methods. We studied 34 subjects; 20 control subjects (M = 15, mean age = 10 +/- 3.5 yrs), 8 patients (M = 4, mean age = 18 +/- 7 yrs) with Crohn's disease in a quiescent phase and 6 patients (M = 6, mean age = 10 +/- 3.5 yrs) with Chronic Intestinal Pseudo-Obstruction. Results. Analysis of gastric electrical activity (GEA) parameters demonstrated that in the control group physiological post-prandial changes are represented by an increase of 3 Cycles Per Minute (3 CPM) activity, Period Dominant Power (PDP) and Period Dominant Frequency (PDF) and by the reduction of bradygastria. Crohn patients showed an insignificant increase of 3 CPM and PDP; CIPO patients showed an abnormal variation of 3 CPM, PDP and post-prandial bradygastria. Moreover, CD patients showed a significant difference in post-prandial values of PDP compared to normal subjects. CIPO patients revealed a significant difference in the values of either preprandial PDF with tachygastria or the post-prandial value of 3 CPM, compared to normal subjects. Conclusions. EEG is a non-invasive method to study gut motility related to GEA alterations present in CIPO as well as in CD patients.

Introduction: Recent literature indicates that laparoscopic techniques are considered the most appropriate approach for management of neonatal ovarian cysts (NOC). However, laparoscopic equipment may be unavailable in most centers, has high costs, and demands a long learning curve, especially in newborns., Methods: A minilaparotomy with exteriorization-aspiration ("catch and suck") approach was used in 14 female newborns with antenatally diagnosed ovarian cysts. Average operating time was 25 minutes (range 15-45 min.). Postoperative course was uneventful in all cases; no intra- or postoperative complications were recorded. Length of stay was 48 hours in all patients., Conclusions: Minilaparotomy with cyst aspiration and subsequent removal may prove a reliable and safe technique in the treatment of NOC: operating time and length of stay are not significantly longer than laparoscopic approach, making this procedure a valid alternative.

The Malone surgical procedure for antegrade continence enema (ACE) greatly improves the quality of life of many patients with fecal incontinence but has several complications. This report describes a technique of percutaneous endoscopic cecostomy (PEC) which was applied in three children with fecal incontinence secondary to anorectal malformation or spina bifida. Using a pediatric fiberscope, a colonoscopy was done which reached the right colon. An intraoperative ultrasound examination confirmed the position of the cecum just below the layers of the abdominal wall. The cecostomy tube was then percutaneously inserted, imitating the steps of percutaneous endoscopic gastrostomy (PEG) using the pull technique. The preliminary results are very encouraging: there are no procedure-related complications in this small series; all the patients are able to carry out the antegrade enema by themselves; and complete control of defecation has been obtained.

Objective: To obtain 'intestinal atresia-like' conditions in the fetal lamb model to subsequently allow in utero surgical repair., Methods: Six time-dated pregnant sheep underwent general anesthesia at 75 days of gestation (term 145 +/- 5 days). After maternal laparotomy and hysterotomy, the fetal abdomen was opened. Once the jejunoileal intestinal loop was identified, the mesenteric vessels were isolated, ligated, and sectioned in 2 fetuses, and in the remaining 5 fetuses the bowel loop was ligated. Two further fetuses were used as controls and underwent sole laparotomy. Of the group of 7 fetuses 2 were reoperated at 100-105 days of gestational age and underwent intestinal recanalization. Eight fetuses were delivered at term by cesarean section and the remaining 1 by spontaneous delivery. One newborn underwent neonatal entero-enteric anastomosis., Results: 4 out of 6 fetuses survived, in utero intestinal or vascular ligation having provoked an 'intestinal atresia-like' picture. The animal operated at birth died. The 2 control fetuses and the 2 fetuses with in utero intestinal recanalization survived until term., Conclusion: The present study shows that in utero treatment of intestinal obstruction is possible in an experimental model.

Objectives: To quantify colorectal neurogenic dysfunction in children with spina bifida and to evaluate the clinical efficacy of appropriate rehabilitation performed by the coloproctologist in the spina bifida team., Patients and Methods: The bowel function of 73 patients with congenital (67) and acquired (six) spinal lesions (age 7-25 years) was evaluated by one physician. Evacuation habit was classified as full bowel control, mild and severe constipation or incontinence. Fifty-two children had mild or severe incontinence or constipation, 22 of whom were treated by the coloproctologist using biofeedback or conventional therapy; 30 were not treated. The outcome was compared between the groups, Results: Bowel constipation remained stable in 90% and was complicated in 10% of the untreated patients, while it ameliorated in 59% of patients who received specialist treatment., Conclusion: Neurogenic bowel dysfunction needs specialist management to achieve better results, using the concept of controlled incontinence. There was no significant difference between conventional therapy and biofeedback methods.

From 1980 to 1996 we followed up 65 children with occult spinal dysraphism (OSD) out of 85 observed in our urodynamic laboratory. The average age at diagnosis was 4.7 years (range: 1-21 years). In 19 cases (22%) the OSD was associated with anorectal anomalies (ARA). Urinary incontinence or voiding anomalies with urinary tract infection were the presenting symptom in 34 children (49%). At diagnosis all children underwent complete neuro-urological and urodynamic evaluation. Thirty-eight patients underwent neurosurgical correction. Vesicoureteral reflux (VUR) was present or developed in 17 patients: 15 underwent endoscopic procedures and 2 required surgery; 2 needed bladder augmentation because of upper tract and renal function deterioration. Urinary incontinence was treated mainly by intermittent catheterization and anticholinergic drugs, but endoscopic treatment was performed in 3 children. At long-term follow-up (2 to 14 years), socially acceptable continence was achieved in 78% of 57 children (8 could not be assessed because they were less than 4 years old). Upper urinary tract deterioration occurred in 15% and renal failure in 7.5%.