Maria Rita Di Pace

Evaluation of esophageal motility and reflux in children treated for esophageal atresia with the use of combined multichannel intraluminal impedance and pH monitoring

BACKGROUND Gastroesophageal reflux (GER) and dysmotility are frequent in patients treated for esophageal atresia (EA). This aim of this study is to evaluate GER and dysmotility in young EA patients using pH-multichannel intraluminal impedance (pH-MII). METHODS Fifteen patients with a mean age of 7.5 years (group 1) have been studied and compared with 15 children without congenital malformation, submitted to pH-MII for suspected GER (group 2). These latter patients serve as a control group of healthy subjects. The following impedance reflux and motility parameters have been studied on 10 standardized swallows: number of reflux episodes, mean acid clearing time, median bolus clearing time, bolus presence time, total bolus transit time, segmental transit time, and total propagation velocity. RESULTS In the group of EA patients, mean acid clearing time and median bolus clearing time were pathological. In the control group, all reflux parameters were normal. Patients with EA had significantly longer median bolus presence time at each measuring site, median total bolus transit time, and median segmental transit time and slower total propagation velocity (P < .001). CONCLUSIONS pH-multichannel intraluminal impedance evaluates both GER and motility patterns. Our report studies impedance parameters of esophageal motility in healthy children and in EA patients using only pH-MII.

Gastroesophageal Reflux in Young Children Treated for Esophageal Atresia: Evaluation With pH-Multichannel Intraluminal Impedance

Objectives:Gastroesophageal reflux (GER) and dismotility occur frequently after repair of esophageal atresia (EA). GER-associated complications can manifest either early or later; then precocious diagnosis and treatment are essential. The aim of the study was to evaluate characteristics of GER and esophageal clearance in children treated for EA with distal tracheoesophageal fistula, using pH-multichannel intraluminal impedance (pH-MII). Patients and Methods:Twenty-two children (ages 3–40 months) treated for EA at birth, and 20 normal children of similar age with suspected GER disease were included in the study. Impedance parameters were analyzed according to age and symptoms. Results:Reflux events were mainly nonacidic. About bolus exposure index, mean acid clearing time (MACT), and mean bolus clearing time (MBCT), a significant difference was found between 2 groups: the median MACT and MBCT were longer, with values of 281 and 39 seconds, respectively, in the EA group and 110 and 15 seconds in the control group (P < 0.0005). Conclusions:Our data show that in young patients the majority of refluxes are not acid. This implies that the incidence of GER may be underestimated if pH-metry is used. The pH-MII is an ideal test in children because it studies both GER with its characteristics and motility pattern. The quality of reflux does not seem to influence the onset of symptoms that are related to an impaired esophageal clearance. Hence, this technique could be useful to study patients treated for EA, avoiding the onset of complications.

Esophageal atresia in newborns: a wide spectrum from the isolated forms to a full VACTERL phenotype?

BackgroundVATER association was first described in 1972 by Quan and Smith as an acronym which identifies a non-random co-occurrence of Vertebral anomalies, Anal atresia, Tracheoesophageal fistula and/or Esophageal atresia, Radial dysplasia. It is even possible to find out Cardiovascular, Renal and Limb anomalies and the acronym VACTERL was adopted, also, embodying Vascular, as single umbilical artery, and external genitalia anomalies.MethodsData on patients with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) admitted in the Neonatal Intensive Care Unit (NICU) between January 2003 and January 2013 were evaluated for the contingent occurrence of typical VACTERL anomalies (VACTERL-type) and non tipical VACTERL anomalies (non-VACTERL-type). The inclusion criterion was the presence of EA with or without TEF plus two or more of the following additional malformations: vertebral defects, anal atresia, cardiovascular defects, renal anomalies and lower limb deformities, like radial dysplasia.ResultsAmong 52 patients with EA/TEF, 20 (38,4%) had isolated EA and 7 (21,8%) had a recognized etiology such a syndrome and therefore were excluded. Among 32 infants with EA and associated malformations, 15 (46,8%) had VACTERL association. The most common anomalies were congenital heart defects (73,3%), followed by vertebral anomalies (66,6%). Many patients also had additional non-VACTERL-type defects. Single umbilical artery was the most common one followed by nervous system abnormalities and anomalies of toes. Between the groups of infants with VACTERL type and non-VACTERL-type anomalies, there are several overlapping data regarding both the tipically described spectrum and the most frequently reported non-VACTERL-type malformations. Thus, it is possible to differentiate infants with a full phenotype (VACTERL full phenotype) and patients that do not meet all the criteria mentioned above, but with some homologies with the first group (VACTERL partial phenotype).ConclusionThe high frequency of non-VACTERL-type anomalies encountered in full and partial phenotype patients would suggest the need for an extension of the clinical criteria for the diagnosis of VACTERL association and also for pre- and post-operative management and follow-up in the short and long term.

Evaluation of esophageal motility and reflux in children treated for congenital diaphragmatic hernia with the use of combined multichannel intraluminal impedance and pH monitoring.

BACKGROUND Gastroesophageal reflux (GER) is frequently observed in children treated for congenital diaphragmatic hernia (CDH) at birth, as well as esophageal dysmotility, that has been hypothesized to be caused by innervatory anomalies. The aim of this study is to evaluate GER and dysmotility in young patients with CDH using pH-multichannel intraluminal impedance. METHODS Thirty children (17 boys and 13 girls) who underwent repair for CDH between 2002 and 2007 with a median age of 5.2 years (range, 3-10 years) were included in the study. All patients were operated on with a subcostal laparotomy incision and had a left-sided diaphragmatic defect. The defect repair required an artificial patch (Goretex, Gore Medical, Flagstaff, AZ) in 8 patients (27%) because of its size. We described impedance reflux parameters and some specific motility parameters studied on 10 standardized swallows. RESULTS The incidence of GER was 86%. Reflux was mainly nonacidic, postprandial, and short-term and reached only the distal esophagus. Esophageal dysmotility was observed only in the distal esophagus. CONCLUSIONS With the use of pH-multichannel intraluminal impedance, both GER and esophageal motility in patients with congenital malformations can be analyzed. In patients with CDH, impaired motility seems to involve only the distal esophagus. In this group, the specific pattern of reflux is probably caused by the involvement of gastroesophageal junction, without significant intrinsic innervation abnormalities as observed in patients with esophageal atresia.

Unilateral multicystic dysplastic kidney in infants exposed to antiepileptic drugs during pregnancy

Prenatal exposure to antiepileptic drugs (AEDs) increases the risk of major congenital malformations (MCM) in the fetus. AED-related abnormalities include heart and neural tube defects, cleft palate, and urogenital abnormalities. Among the various congenital anomalies of the kidney and urinary tract (CAKUT), multicystic dysplastic kidney (MCDK) disease is one of the most severe expressions. Although prenatal ultrasound (US) examination has increased the prenatal diagnosis of MCDK, the pathogenesis is still unclear. We report on four cases of MCDK in infants of epileptic women treated with AEDs during pregnancy. From October 2003 to June 2006, we observed four infants with unilateral MCDK born to epileptic women. Three patients were considered to have typical features of multicystic dysplastic kidney, and one infant was operated because of a cystic pelvic mass in the absence of a kidney in the left flank. The macroscopic appearance of this mass showed an ectopic multicystic kidney confirmed by histological findings. All patients have been studied by US scans, voiding cystourethrogram (VCUG), and radionuclide screening isotope imaging. The prenatal exposure to AEDs increases the risk of major congenital malformations from the background risk of 1–2% to 4–9%. AEDs may determine a defect in apoptosis regulation that could lead to abnormal nephrogenesis, causing MCDK. Carbamazepine (CBZ) and phenobarbital (PHB) during pregnancy should be used at the lowest dosage compatible with maternal disease. The reduction, or even suspension, of drug dosage should be achieved from the periconceptional period to the first 8 weeks of gestation to avoid any interference with organogenesis.

The role of Doppler ultrasonography in determining the proper surgical approach to the management of varicocele in children and adolescents.

In a study from Italy, colour‐Doppler ultrasonography was a reliable diagnostic tool in the preoperative assessment of patients with varicocele. The authors also found that it helped to distinguish those who could be treated laparoscopically from those who should be treated by microsurgical subinguinal ligature.

Ileocecal duplication cysts: Is the loss of the valve always necessary?

BACKGROUND Ileocecal (IC) duplication cysts are enteric duplications located at the IC junction, not clearly identified in all the published series. The reported treatment is IC resection and ileocolic anastomosis. It is well known that the loss of the IC valve has several adverse effects. This study is aimed at demonstrating that cyst removal together with the common ileal wall and following enterorrhaphy is possible, safe, and effective in preserving the IC region. METHODS Medical records of 3 patients who underwent surgery for IC duplication between 2003 and 2013 were retrospectively reviewed evaluating follow-up results. RESULTS All patients had an antenatal diagnosis of intraabdominal cystic mass. In two cases associated malformations were reported. The lesions presented at newborn age with intermittent small bowel obstruction and required removal. No patients underwent IC resection. The diagnosis of duplication cyst was confirmed by histo-pathologic examination. The postoperative course was uneventful, even in the long-term follow-up. CONCLUSIONS Our conservative approach is a simple and safe technique, effective in avoiding the loss of the IC valve in children with duplication at the IC junction.

A simple technique of oblique anastomosis can prevent stricture formation in primary repair of esophageal atresia

BACKGROUND Anastomotic stricture is an important problem after esophageal atresia (EA) repair. This study evaluates a technique of oblique esophageal anastomosis without use of a flap in order to prevent stricture formation. METHODS Medical records of 16 patients (14 with EA type III and 2 with EA type IV Ladd-Gross classification) who underwent primary repair of EA at birth without anastomotic tension were reviewed, evaluating long-term follow-up results. All patients were studied with esophageal contrast study, pH-multichannel intraluminal impedance, and endoscopy. The incidence of complications and their management were analysed. RESULTS Contrast esophagogram and esophagoscopy always showed regular patency of the suture line. CONCLUSIONS Our technique of oblique anastomosis is simple, safe, and effective in preventing stricture formation even in the long-term follow-up.

One-trocar, video-assisted stripping technique for use in the treatment of large ovarian cysts in infants.

BACKGROUND Management of ovarian cysts in infants is controversial; it can be conservative or surgical, and the management is determined by the cysts size and sonographic features. METHODS A surgical approach using a 10-mm umbilically placed operative laparoscope was taken in 3 female infants with antenatally diagnosed large, simple ovarian cysts. The contents of the cysts were partially aspirated and the cyst walls were stripped off the remaining ovarian parenchyma. No intraoperative or postoperative complications were recorded. CONCLUSIONS The one-trocar video-assisted stripping technique for large ovarian cysts in infants appears to be an ovarian-tissue-preserving procedure, and it sidesteps the disadvantages of large scars and formation of adhesions.