Long-term urological outcome of cloaca patients with multidisciplinary management.

PURPOSE: Urological management of Cloacal Malformation (CM) focuses on preserving renal function and continence. Study aim was to analyze urinary and intestinal outcomes in CM patients, considering the length of common channel (CC) and presence of occult spinal dysraphism (OSD). METHODS: Retrospective review of CM treated at our institution by a multidisciplinary team from 1999 to 2020. Patients with follow-up < 2.5 years were excluded. Length of CC, renal function, urinary and bowel outcomes, presence of associated anomalies (especially OSD) were evaluated. RESULTS: Twenty patients were included, median age at follow-up: 8 years (4-15). A long CC > 3 cm was described in 11 (55%). Chronic kidney disease was found in 3 patients. Urinary continence was achieved in 8/20 patients, dryness (with intermittent catheterization) in 9/20. Fecal continence was obtained in 3/20, cleanliness in 14 (under bowel regimen). OSD was present in 10 patients (higher prevalence in long-CC, 73%). Among OSD, 1 patient reached fecal continence, 7 were clean; 2 achieved urinary continence, while 6 were dry. CONCLUSIONS: Length of CC and OSD may affect urinary and fecal continence. An early counseling can improve outcome at long-term follow-up. Multidisciplinary management with patient centralization in high grade institutions is recommended to achieve better results.

Short versus mid-long-term outcome of transanal irrigation in children with spina bifida and anorectal malformations.

PURPOSE: We assessed short- and mid-long-term clinical efficacy of transanal irrigation (TAI) and its effect on the quality of life of children with spina bifida (SB) and anorectal malformations (ARM). METHODS: Seventy-four pediatric patients (age 6-17 years) with SB and ARM with neurogenic bowel dysfunction were enrolled for a prospective and multicentric study. Patients were evaluated before the beginning of TAI (T0), after 3 months (T1) and after at least 2 years (range 24-32 months) (T2) using a questionnaire assessing bowel function, the Bristol scale, and two validated questionnaires on quality of life: the CHQ-PF50 questionnaire for the parents of patients aged 6-11 years and the SF36 questionnaires for patients aged between 12 and 18 years. RESULTS: Seventy-two patients completed TAI program in T1, and 67 continued into T2. Bowel outcomes (constipation and fecal incontinence) improved in both the SB and the ARM groups in the short and mid-long term. In both groups at T1 and T2, parents and children reported an improvement in quality of life and there was a significant increase of stool form types 4 and 5 as described by the Bristol scale. Common adverse effects during the study were similar at T1 and T2 without serious complications. CONCLUSIONS: We observed a sustained improvement in bowel management and quality of life in SB and ARM children during the study, more significant in the short term than in mid-long term. To maintain success rates in the mid-long term and to reduce the dropout rate, we propose patient training and careful follow-ups.

Peristeen(®) transanal irrigation in paediatric patients with anorectal malformations and spinal cord lesions: a multicentre Italian study.

AIM: In paediatric and adult patients with neurogenic bowel, transanal irrigation (TAI) of the colon has gained popularity due to the introduction of a specifically designed device. The aim of this pilot study was to present the results of TAI using the Peristeen(®) TAI system in a group of paediatric patients with anorectal malformation (ARM) and congenital or acquired spinal cord lesions (SCLs). METHOD: Eight Italian paediatric surgery and spina bifida centres participated in the study. The inclusion criteria were age between 6 and 17 years, weight above 20 kg and unsatisfactory bowel management. Patients with chronic inflammatory bowel disease, mental disability and surgery within the previous 3 months were excluded. At the beginning of treatment (T0) and after 3 months (T1) the Bristol scale, a questionnaire assessing bowel function, and two questionnaires on quality of life (QoL) for patients aged 6-11 years (CHQ-pf50) and 12-17 years (SF36) were administered. RESULTS: Eighty-three patients were enrolled, and seventy-eight completed the study (41 ARMs, 37 SCLs). At T1, constipation was reduced in ARMs from 69% to 25.6% and in SCLs from 92.7% to 41.5%, faecal incontinence in ARMs from 50% to 18.6% and in SCLs from 39% to 9.8% and flatus incontinence in ARMs from 20.9% to 9.8% and in SCLs from 31.7% to 10%. At T0, the Bristol Stool Scale types were 1-2 in 45% of ARMs and 77.5% of SCL patients, whereas at T1 types 1-2 were recorded in only 2.5% of SCL patients. QoL improved in both groups. In the younger group, a significant improvement in QoL was recorded in ARM patients for eight of nine variables and in SCL patients for seven of nine variables. CONCLUSION: This study showed that Peristeen TAI resulted in a significant time reduction in colonic cleansing, increased independence from the carer and improved QoL in paediatric patients with ARMs and SCLs.

Epidydimo-orchitis and anorectal malformations: when and in whom?

PURPOSE: Epididymo-orchitis (EO) is infrequently reported in anorectal malformation (ARM) cases. Therefore, it is difficult to assess its risk factors. METHODS: A total of 110 male patients who were operated on for ARM at the same Institution over a period of 13 years were contacted. Association was assessed between EO and the following: spinal dysraphism (SD), symptomatic VUR (VUR), and bowel management (BM) requiring enemas. The data were analyzed with the Chi-square test. RESULTS: A total of 89 patients were contacted. Ten cases of EO were found, and all occurred in patients with recto-urethral (RU) fistula after reconstruction. The patients' age at first episode ranged between 4 and 11 years. RU fistula patients experiencing EO (Group A, 10 patients) were compared with those without EO (Group B, 33 patients). VUR occurred in 9/10 cases in Group A and in 13/33 cases in group B (Chi-square 7.8658, p = 0.005038). SD was present in 4/10 cases in group A and in 13/33 cases in Group B (Chi-square 0.0434, p = 0.83491). A total of 8/10 cases in Group A and 12/33 cases in Group B were on BM (Chi-square 5.87, p = 0.0015). CONCLUSIONS: EO occurs in approximately in 20 % of male cases with ARM, and recto-urinary communication and should be considered the primary diagnosis in the presence of testicular pain. This could avoid unnecessary surgical exploration, and the family should be counseled about this subject.

The impact of perioperative care on complications and short term outcome in ARM type rectovestibular fistula: An ARM-Net consortium study.

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.

Exteriorization-aspiration minilaparotomy for treatment of neonatal ovarian cysts.

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.

Long-term urological follow-up of occult spinal dysraphism in children.

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%.

Role of electrogastrography in detecting motility disorders in children affected by chronic intestinal pseudo-obstruction and Crohn's disease.

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.

[pH-metric parameters potentially predictive of asthmatic symptomatology: clinical and statistical research]. / Parametri pH-metrici potenzialmente predittivi della sintomatologia asmatica: ricerca clinica e statistica.

The gastro-esophageal reflux (GER) usually causes digestive symptoms, failure to trive and/or respiratory symptoms. Furthemore the association between GER and asthma is well known. Nevertheless, the relationship between two pathologies and role of GER in aggravation of asthma are not well known. The aims of our study is to identify the peculiar pH-metric caracteristics of GER may be responsable of asthmatic symptoms in children. The study was conducted in 32 children. The patients were divided into two groups: Group A composed of 16 children suffering from non-allergic asthma characterized by prevalent nocturnal manifestation; Group B composed of 16 children suffering from GER, without respiratory symptoms. All patients underwent to 21 pH-monitoring. The pH-metric data collected in two groups are submitted to statistic analysis using the Student's "t" Test.

Pulmonary hypertension in neonates with high-risk congenital diaphragmatic hernia does not affect mid-term outcome.

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.

Isolated fetal ascites secondary to persistent urogenital sinus.

Objective. To present a case of isolated ascites secondary due to urogenital abnormalities (urogenital sinus) without any other prenatal ultrasound marker. Method. A 36-year-old woman with prenatal isolated ascites delivered a female baby, weighing 2.285 g; ascites was drained at birth and the baby underwent several episodes of urinary retention prior to undergoing X-ray investigations. Results. A voiding cystourethrogram revealed a short urogenital sinus: a vesicostomy was performed. A vaginoscopy revealed double vagina with a large posterior vagina. A posterior sagittal anorectal pull-through with genitoplasty was performed at 2 years old with 1-year follow-up. Conclusions. Though rare, a urogenital abnormality is to be suspected in fetal ascites cases with negative viral tests and no cardiac anomalies. The most common ultrasound marker of such abnormalities (fluid filled cavity) may be missing because of complete drainage of urine through the tubes into peritoneum.

[Surgical therapy of chronic constipation in pediatric age]. / Terapia chirurgica della stipsi in età pediatrica.

The authors report their experience in the treatment of the chronic constipation in paediatric age. During the last 3 years (1991-1994), 230 children presenting chronic constipation have been studied at the Strumental Gastroenterologic Department of Children Hospital Bambino Gesù; 19 of them (8%), 10 male and 9 female, were studied with anorectal manometry, defecography and Intestinal Transit test. All the patients had a overtone (high squeeze) of the anal sphincter and the medical treatment was unsuccessful. They underwent sphincteromyectomy by posterior approach. In 17 patients it has been obtained a notable improvement of the symptomatology with regularization of the evacuations. The simplicity of the surgical technique, the absence of complications and the obtained results confirm the validity of sphinteromyectomy in the therapy of the chronic constipation in paediatric age. Interoperating anorectal manometry proved to be essential both in modulating sphincteromyectomy and in its eventual complications.

Percutaneous endoscopic cecostomy for antegrade colonic irrigation in fecally incontinent children.

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.

Colorectal dysfunction and faecal incontinence in children with spina bifida.

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.

Cardiopulmonary performances in young children and adolescents born with large abdominal wall defects.

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.

An experimental animal model of intestinal obstruction to simulate in utero therapy for jejunoileal atresia.

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.