Antenatal ultrasonographic features associated with segmental small bowel dilatation: an unusual neonatal condition mimicking congenital small bowel obstruction.

Segmental dilatation of the intestine (SDI) is an unusual condition presenting during the neonatal period, with symptoms of obstruction. Late diagnosed cases are also reported. The clinical polymorphism, and the lack of specificity of radiological investigations, make diagnosis difficult. Prenatal detection of abdominal cystic lesions or bowel dilatation has occasionally been reported to be associated with SDI. We herein report two cases of SDI, with a prenatal ultrasonographic suspicion of intestinal abnormality. In both infants, a dilatation of the ileum was found at surgery, without any evident site of obstruction or abnormal histology. SDI must be taken into consideration when a prenatal alert of possible bowel obstruction is not followed by postnatal clinical signs.

Delivery in a tertiary Center with co-located surgical facilities makes the difference among neonates with prenatally diagnosed major abnormalities.

OBJECTIVE: Impact of prenatal diagnosis (PD) of major abnormalities on neonatal outcome is matter of debate. Unfortunately cases with and without PD may sometimes not be comparable. This is generally related to a lower maturity (GA) and weight (BW) secondary to a high rate of preterm cesarean sections (CSs) for clinical convenience. Present study tried to find out if in utero transfer to a Center with co-located surgical facilities reduces these potential risk factors. METHODS: 152 cases with prenatally detectable conditions were studied and divided according to PD; the following data were compared: GA, BW, obstetrical complications, associated malformations, mode and site of delivery, outcome. Cases with PD delivered in our Center (Inborn, IB) or transferred after birth (Outborn, OB) were compared. RESULTS: 61 cases had a PD (IB/OB ratio 34/27); GA and BW were lower respect to no-PD cases and a higher CS rate was found among OB cases, not justified by complicated pregnancies. No differences in outcome were observed. CONCLUSIONS: Elective preterm CS is still largely practiced for fetuses with PD of a major congenital anomaly in Centers without co-located surgical facilities either in the presumption of safer delivery or to facilitate postnatal transfer. This leads to a lower GA and BW and may spoil potential impact of PD on outcome. This may be avoided promoting prenatal transfer to a Center with co-located surgical facilities.

Jejunoileal Atresia: Factors Affecting the Outcome and Long-term Sequelae.

CONTEXT: Jejunoileal atresia (JIA) is a common abnormality. The outcome is conditioned by several variables. Nutritional problems, and long-term sequelae are described among those who survive. AIM: To correlate the type of JIA and its management to the outcome and long-term quality of life. SETTINGS AND DESIGN: Forty-three cases over a 17-year period (1992-2009). Perinatal data, management, and outcome were extracted from the clinical notes. The cases that had survived were contacted to get information about their present condition. MATERIALS AND METHODS: Morbidity and mortality were matched to maturity, birth weight, mode of diagnosis, type of JIA, associated anomalies, and management and duration of parenteral nutrition. Growth and quality of life in 34 cases were evaluated via a telephone interview at a minimum of one year from surgery. STATISTICAL ANALYSIS: Fisher test, Linear regression test, Kruskal-Wallis test, Dunn's comparison test. RESULTS: Male/Female ratio was 25/18 and median birth weight was 2.644 g. Prenatal diagnosis was recorded in 34%. Six patients (14%) had associated anomalies. Primary surgery was resection and anastomosis in 88% and temporary stoma in 12%. Length of the resected bowel ranged from 3 to 65 cm. Whenever multiple atresia was found, the bowel length was saved by multiple anastomosis. Three dehiscences and three adhesive obstructions required a reoperation. Two patients (4%) died due to a central catheter-related sepsis. Prenatal diagnosis did not influence the outcome and was associated with a higher rate of Cesarean deliveries. Interview, at a median of nine years, showed normal growth in 85%. One case of short bowel syndrome is still on parenteral support at 22 months. CONCLUSIONS: Preserving bowel length and reducing the recourse to stoma is the key to good outcome and growth. Sequelae are correlated with the type of atresia and length of residual bowel; however, they decrease their severity with time.

Meconium obstruction in absence of cystic fibrosis in low birth weight infants: an emerging challenge from increasing survival.

BACKGROUND: Meconium abnormalities are characterized by a wide spectrum of severity, from the meconium plug syndrome to the complicated meconium ileus associated with cystic fibrosis. Meconium Related Ileus in absence of Cystic Fibrosis includes a combination of highly viscid meconium and poor intestinal motility, low grade obstruction, benign systemic and abdominal examination, distended loops without air fluid levels. Associated risk factors are severe prematurity and low birth weight, Caesarean delivery, Maternal MgSO4 therapy, maternal diabetes. In the last 20 yrs a new specific type of these meconium related obstructions has been described in premature neonates with low birth weight. Its incidence has shown to increase while its management continues to be challenging and controversial for the risk of complicated obstruction and perforation. MATERIALS AND METHODS: Among 55 newborns admitted between 1992-2008 with Meconium Related Ileus as final diagnosis, data about Low Birth Weight infants (LBW < 1500 g) were extracted and compared to those of patients ≥ 1500 g. Hischsprung's Diseases and Cystic Fibrosis were excluded by rectal biopsy and genetic probe before discharge. A softening enema with Gastrografin was the first option whenever overt perforation was not present. Temporary stoma or trans appendiceal bowel irrigation were elected after unsuccessful enema while prompt surgical exploration was performed in perforated cases. NEC was excluded in all operated cases. Data collected were perinatal history and neonatal clinical data, radiological signs, clinical course and complications, management and outcome. RESULTS: 30 cases with BW ≥ 1500 g had an M/F ratio 16/14, Mean B.W. 3052 g, Mean G.A. 37 w Caesarean section rate 40%. There were 10 meconium plug syndrome, 4 small left colon syndromes, and 16 meconium ileus without Cystic Fibrosis. Five cases were born at our institution (inborn) versus 25 referred after a mean of 2, 4 Days (1-7) after birth in another Hospital (outborn). They were managed, after a Gastrografin enema with 90% success rate, by 1 temporary Ileostomy and 2 trans appendiceal irrigation. 25 cases with BW< 1500 g (LBW) had M/F ratio 11/14, Mean B.W. 818 g, Mean G.A. 27 w, Caesarean section rate 70%, assisted ventilation 16/25. There were 8 inborn and 17 outborn. Gastrografin enema was successful in 6 out 8 inborn infants only, all referred within one week from birth. There were 12 perforations mainly among late referred LBW outborn. CONCLUSIONS: Meconium Related Ileus without Cystic Fibrosis responds to conservative management and softening enema in most of mature infants. In LBW clinical course is initially benign but as any long standing bowel obstruction management may present particular challenges. Clinical and plain radiographic criteria are reliable for making diagnosis and testing for Cystic Fibrosis may not be indicated. Enema may be resolutive when performed in a proper environment. Perforated cases may be confused with NEC which is excluded by clinical history, no signs of sepsis, lab signs missing, abdominal signs missing, typical radiological signs missing. The higher complication rate is recorded among cases delivered and initially managed in Neonatal Units without co-located Surgical Facilities. Early diagnosis and aggressive medical therapy may lead to higher success rate and help avoiding surgical interventions. Surgical therapy in uncomplicated cases, unresponsive to medical management, should be minimally aggressive.

Prenatal diagnosis of duodenal obstruction selects cases with a higher risk of maternal-foetal complications and demands in utero transfer to a tertiary centre.

OBJECTIVES: It was the aim of this study to determine if prenatal diagnosis of congenital duodenal obstruction (CDO) selects high-risk pregnancies and demands special perinatal attention. METHODS: Medical records of 62 neonates with intrinsic CDO, admitted since 1981 in 2 institutions, were reviewed and divided into 2 groups: 39 cases, detected in utero by a prenatal ultrasonogram (group A), and 23 diagnosed at birth (group B). Prevalence of complete CDO, mean gestational age, mean birth weight, hydramnios, as well as maternal-foetal complications requiring emergency Caesarean section and associated with premature delivery were compared in the 2 groups. RESULTS: A complete CDO was found in 77% of patients in group A versus 48% of patients in group B (p < 0.02). Differences in mean gestational age and mean birth weight in the 2 groups were non-significant. Prenatally diagnosed patients presented a higher prevalence of hydramnios, maternal-foetal complications and premature delivery. CONCLUSIONS: Prenatal diagnosis selects patients with complete CDO and hydramnios. These pregnancies present a high incidence of maternal-foetal complications, which may require an emergency Caesarean section, and are frequently associated with premature delivery. These aspects must be considered in prenatal counselling. In utero transfer to a tertiary centre for delivery and appropriate perinatal care should be recommended.

The fate of prenatally diagnosed primary nonrefluxing megaureter: do we have reliable predictors for spontaneous resolution?

OBJECTIVES: To compare predictive values of current morphologic parameters with congenital renal damage associated with severe megaureter. METHODS: A retrospective analysis was performed using records of 37 patients (50 megaureters) referred before birth for a primary megaureter. Mean follow-up was 26 months (range, 1 to 8 years). RESULTS: Dilatation resolved spontaneously in 46 of 50 ureters. Only 4 of 37 patients required surgery (10.8%) after a mean follow-up of 58 months (range, 32 to 80 months). Average time to resolution was 24 months (range, 1 to 82 months) and was independent from sex, side, and bilaterality. A weak correlation was found with initial anteroposterior pelvic diameter, ureteral diameter, and separate function at renogram. A significant correlation (P <0.02) was found between megaureter type and time elapsed to spontaneous resolution. As far as differential function was concerned, mean values were significantly lower among type III megaureters, which had the lowest rate of resolution. CONCLUSIONS: The fate of severe megaureter seems strongly influenced by congenital renal damage secondary to a developmental abnormality of the ureteric bud. A poor resolution rate has to be expected in these cases; surgery must be reserved for symptomatic cases but has no influence on pre-existing renal damage.

Tracheomalacia in esophageal atresia. Usefulness of preoperative imaging evaluation for tailored surgical correction.

Tracheomalacia (TM) associated with esophageal atresia (OA) generally is located in the thoracic segment of the trachea, and the close anatomical relationships it has with the aortic arch and the other mediastinal structures play a remarkable role in the dynamic obstruction of the airways. Only correct preoperative imaging studies permit a precise anatomic description of TM, reducing the risk of incomplete treatment owing to the persistence of other undervalued problems. This report describes the usefulness of preoperative imaging by dynamic fiberoptic bronchoscopy and spiral multilayer computed tomography with 3-dimensional reconstruction in 7 symptomatic children with segmentary TM associated with esophageal atresia and treated surgically "case by case" based on dynamic fiberoptic bronchoscopy and computed tomography data.

Tracheomalacia in oesophageal atresia: morphological considerations by endoscopic and CT study.

OBJECTIVE: A Tracheomalacia complicates 11-33% of cases of Oesophageal Atresia with distal Tracheo-Oesophageal Fistula. The lesion generally involves only the thoracic segment of the trachea, and it has close anatomical relationships with the mediastinal structures, specially with the aortic arch. We therefore tried to define the most important morphotypes of tracheobronchial malacia by using dynamic fiberoptic bronchoscopy (DFB) and spiral multilayer computed tomography (CT). METHODS: Between 1999 and 2003 we studied 40 children from two different institutions who had been operated on at birth for oesophageal atresia. All patients were been submitted to DFB, and the positive cases underwent examination by CT with an iodinated contrast medium. CT angiographic images of great vessels and multiplanar and three-dimensional images of the airways (virtual broncoscopy and broncography) were obtained for morphological evaluation. RESULTS: Twenty-five patients (62%) tested positive for malacia using DBF and all were also confirmed by CT study. In 11 cases (46%), the malacia was located at the thoracic section of the trachea, which was occluded by compression of the aorto-innominate complex. A simple intrinsic tracheomalacia without any vascular compression was present in eight cases (33%), while in five cases (21%), the malacia was complex. CONCLUSIONS: A correct morphological analysis of the malformed segment permitted 'tailored surgery' for each individual patient, allowing us to take account of the type of malacia, its length, and the compressive action exercised by the mediastinal great vessels.

Mortality and long term morbidity in esophageal atresia: the reduced impact of low birth weight and maturity on surgical outcome.

Progress in perinatal and postoperative techniques has reduced the prognostic role of traditional risk factors in esophageal atresia (EA). This paper reports on 75 cases of esophageal anomalies observed between 1992 and 2002 and followed after surgery from a minimum of six months to a maximum of ten years (mean five years). The impact on survival of birth weight, week of delivery, associated anomalies and need of ventilatory support at birth are discussed. Twenty-four patients were born before 37 weeks of gestation, 18 weighed less than 2000 g.; major anomalies affected 11 neonates, 23 cases required mechanical ventilation at birth. Seventy-four patients were operated on with a 90.6% survival rate; no deaths were related to surgical treatment. Three cases required reoperation for postoperative complications. Birth weight and week of delivery did not seem to influence outcome; this is affected by severe associated cardiovascular anomalies and the need of ventilation at birth. Follow up at 24 months on 51 patients, revealed respiratory problems in 12 cases and severe gastro-esophageal reflux in 16. This affected quality of life of EA patients and required long term medical attention; improvement with growth was observed. No correlation between perinatal conditions and late sequelae could be demonstrated in our series.

Giant tracheocele following primary tracheostomy closure in a 3 year old child.

Tracheocele is a rare pathology, especially complication of tracheal surgery. We present a case of a 3 year old pediatric patient with secondary tracheocele arising after simple surgical closure of tracheostomy. It appeared 2 months after intervention that cervical mass swelling during respiratory acts. Diagnosis was made by TC scan and by dynamic flexible bronchoscope which revealed a substomal segmental tracheomalacia. Surgical procedure consisted of simple excision of tracheocele associated with enhancing tracheomalacic segment by free graft of costal cartilage as Cotton procedure. Follow up at 14 months demonstrated a patent of cervical trachea without any sign of malacia.

Reflux strictures of the oesophagus in children: personal experience with preoperative dilatation followed by anterior funduplication.

INTRODUCTION: Oesophageal surgery for reflux stricture is as challenging in adults as in the paediatric age group. Several management protocols, both medical and surgical, are currently proposed, such as bougienage, funduplication without dilatation, funduplication with pre- and postoperative dilatation, resection and interposition, and pharmacological therapy. However, reported results are not univocal. The aim of this work is to demonstrate that preoperative treatment with H2-antagonist combined with oesophageal dilatation and followed by anterior funduplication (Boix-Ochoa procedure with elongation of intraabdominal segment of the oesophagus) is a long-term, effective treatment for reflux stricture in children. It provides a tension free repair and an adequate protection to reflux, thus, preventing recurrences. MATERIALS AND METHODS: In the last five years we observed oesophageal stenosis in 10 out of 49 children, operated for gastroesophageal reflux (mean age 62.9 months, range 12-156 months). All children underwent treatment with H2-antagonist (Ranitidine) and prokinetic agent (Cisapride), followed by oesophageal dilatations (mean 2.8, range 2-4 cycles) with Savary-Gillard dilators. An open anti-reflux procedure was performed (9 Boix-Ochoa and 1 Nissen) on children where a 9 mm endoscope passed easily through the oesophageal lumen. The pre and postoperative evaluation of all patients included symptoms assessment, esophagogram and endoscopy. RESULTS: Results were satisfactory in 9 patients. Only one patient where a Nissen wrap was performed, incomplete relaxation was documented radiologically. The patient required several dilatations for residual dysphagia before reaching a symptom free status. All other patients had an average follow-up of 38 months (range, 5 months to 5 years) with relief from dysphagia and no recurrence of stricture. Radiological controls showed good oesophageal lumens, with normally positioned neocardias, opening regularly during barium passage with no sign of reflux. Multiple biopsies from endoscopic controls confirmed complete relief from oesophageal stricture but persistence of Barrett's mucosa. CONCLUSIONS: Our treatment of choice for reflux stricture is preoperative pharmacological therapy followed by series of dilatation with Savary-Gillard dilators till oesophagus is adequately dilated. Antireflux surgery is mandatory when a stricture is observed. We prefer a Boix-Ochoa funduplication with extensive transhiatal mobilization of thoracic oesophagus. This results in a "tension free" fundoplication even when brachioesophagus is present. The procedure appears to be physiological for pediatric patients and in our hands was free from recurrences.

Posterior urethral valves and mirror image anomalies in monozygotic twins.

Monozygotic (MZ) twins with both posterior urethral valves (PUV) and additional mirror image malformations are described. This association suggests that an early embryonic event may lead to MZ twinning, PUV, and mirror image anomalies.