Andrea Conforti

Long term follow-up in high-risk congenital diaphragmatic hernia survivors: patching the diaphragm affects the outcome

BACKGROUND/PURPOSE The increased survival rate reached in infants with congenital diaphragmatic hernia (CDH) has shown a concomitant increase in late morbidity. A recent report from CDH Study Group showed that dimension of diaphragmatic defect is the only independent risk factor of mortality. However, the influence of defect size on late morbidity is still controversial. The aim of the study was to evaluate the influence of patch repair (proxy of diaphragmatic defects size) on midterm morbidity. METHODS All high-risk (prenatal diagnosis and/or respiratory symptoms within 6 hours of life) CDH survivors treated at our institution from 2004 to 2008 were followed up in a multidisciplinary outpatient clinic as part of a longitudinal prospective study. Auxological, gastroesophageal, pulmonary, and orthopedic evaluations were performed at 6, 12, and 24 months of age. Patient outcomes were compared with respect to +/- patch repair. RESULTS Of 70 survivors, 61 (87%) were enrolled and prospectively evaluated in follow-up. Poorer auxological outcome, increased rate of gastroesophageal reflux, and altered pulmonary function test were observed during follow-up. CONCLUSIONS Patch repair correlates with higher pulmonary, auxological, and gastroesophageal morbidity without increasing chest wall deformities at long-term follow-up.

Long-term outcome of home parenteral nutrition in patients with ultra-short bowel syndrome.

Objective: The patients with ultra-short bowel syndrome (U-SBS) have been considered potential candidates for a preemptive/rehabilitative intestinal transplantation owing to the high risk of death from the underlying disease. We hypothesized that children with U-SBS, in the absence of intestinal failure-associated liver disease (IFALD), could also have a good rate of survival on home parenteral nutrition (HPN). Methods: A prospective database from the “Bambino Gesù” Artificial Nutrition and Intestinal Failure Program was used to evaluate outcomes and morbidities of consecutive patients with ⩽10 cm of small bowel enrolled since 2000. Results: Eleven patients were identified with a median bowel length of 7.5 (3–9) cm. Eight patients developed IFALD, which reversed in 7 of them; the IFALD progressively worsened in 1 patient until death. One patient underwent isolated intestinal transplantation and 1 patient is no longer receiving parenteral nutrition (PN) and both are fully enterally fed. The other patients remained at least partially dependent on HPN. The number of days of inpatient care decreased in all of the patients except for the 1 who had repeated episodes of central line infections. Conclusions: The survival of patients with U-SBS receiving HPN was good. Although IFALD was frequent, it had been manageable in most of the patients, but in a single complex case, it led to death. The multidisciplinary management warranted to these patients to approach the school age, to grow, and to maintain the oral intake. Patients with U-SBS are rare, and to better understand their long-term survival, further studies, including more large patient populations, are required.

Symptomatic vocal cord paresis/paralysis in infants operated on for esophageal atresia and/or tracheo-esophageal fistula

OBJECTIVES To describe the prevalence and pathogenesis of symptomatic vocal cord paresis/paralysis (VCP) in patients treated for esophageal atresia (EA), tracheo-esophageal fistula (TEF) or both. STUDY DESIGN Retrospective study of all patients treated for EA/TEF in our center (1995 to 2009). Patients with and without symptomatic VCP were compared for gestational age, birth weight, associated anomalies, referrals, long-gap EA (> 3 cm or 3 vertebral bodies), cervical esophagostomy, anastomotic leakage, length of ventilation, and major cardiac surgery. Prevalence or median (IQR) is reported. RESULTS Of 174 patients, 7 (4%) had symptomatic VCP. Prevalence of referrals (5/7 versus 21/167; P = .0009), long gap (5/7 versus 41/167; P = .0146), previous cervical esophagostomy (5/7 versus 7/167; P < .0001), and anastomotic leakage (3/7 versus 10/167; P = .0097) was higher, and ventilation longer (8.5 days [7.0 to 15.5] versus 6.0 days (5.0 to 7.0); P = .0072) in patients with VCP. CONCLUSIONS In infants treated for EA/TEF, VCP should be ruled out in case of persistent respiratory morbidity or, when present, cautiously monitored. Surgical risk factors should be actively controlled. Further studies are needed to define the prevalence of acquired and congenital VCP in patients with EA/TEF.

A case of massive gastric necrosis in a young girl with Rett Syndrome

This is the unusual case of a 17-year-old girl affected by Rett Syndrome (RS) who suffered acute abdominal distension and constipation for a week. Laparotomy showed massive gastric dilatation, with total necrosis and perforation. Total gastrectomy and Y-Roux esophagojejunostomy were performed. We believe the clinical status was caused by the mechanism of air swallowing, present in our patient and typical in RS. In fact, as reported, massive air bloat may result in a decrease of the intramural blood flow with consequential ischemia of the gastric wall. We stress the importance of early detection of the gastroenterological symptoms in these patients, with timely positioning of nasogastric tube and gastrostomy, to prevent serious complications potentially life-threatening as massive gastric necrosis.

Adenomyomatosis of the Gallbladder in Childhood: Report of a Case and Review of the Literature

ABSTRACTAdenomyomatosis of the gallbladder is considered an acquired pathologic condition that affects adults, with only 2 cases previously described in childhood. We report on a 5-year-old boy with incidental detection of this lesion by ultrasonography. Pathologic and clinical assessments are discussed in addition to treatment decision-making, including current insights into the relation between adenomyomatosis and malignancy of the gallbladder. The liberal use of ultrasonography in children with abdominal pain may result in detection of an increasing number of asymptomatic patients. Caregivers should be aware of this condition and its therapeutic implications in the pediatric population.

Near infrared spectroscopy in newborns with surgical disease

NIRS has been used as a key device with the aim to evaluate the impact of surgery and anesthesia on cerebral and splanchnic oxygenation in neonatal population. The main applications has been in cardiac surgery, congenital diaphragmatic hernia and esophageal atresia. In this report we summarized the results published on the application of NIRS in neonatal surgery with particular respect to cerebral and splanchnic oxygenation, presenting also some future prospective.

Preoperative administration of Sudan III and successful treatment of persistent chylous ascites in a neonate

Congenital chylous ascites is a rare entity, and surgical treatment is confined to selected intractable cases. We report 2 cases of refractory congenital chylous ascites successfully treated with preoperative administration of lipophilic dye (Sudan III) followed by abdominal systematic surgical exploration, cauterization, and fibrin glue application.

Perioperative Complications of Esophageal Atresia.

Current survival rate of infants with esophageal atresia with/without tracheoesophageal fistula (EA/TEF) exceeds 90% and great interest is dedicated to the long-term outcomes and complications. However, perioperative complications represent a significant burden to the EA/TEF infants and a technical challenge for the surgeon. In this review, we will focus on the most frequent perioperative complications specific of EA/TEF to describe their predisposing factors, possible preventive measures, and treatment options.

Current Management of Congenital Pulmonary Airway Malformations: A “European Pediatric Surgeons' Association” Survey

Abstract Aim To define current management of congenital pulmonary airway malformation (CPAM). Methods A total of 181 European Pediatric Surgeons’ Association members (91% senior) from 48 countries completed an online questionnaire. Main Results Prenatal: 93% respondents work in centers with prenatal diagnosis facilities, and 27% in centers offering in utero surgery. Prenatal counseling is performed by 86% respondents, 22% of whom see >10 cases per year. Risk of single pre‐/postnatal complications is deemed low (<5%) by more than 60% of respondents. Eighty‐six percent respondents do not offer pregnancy termination for prenatally diagnosed CPAM. Fetal hydrops is the most frequent indication for termination (87%), followed by parental willingness (52%). Prenatal surgery is an option for 44% respondents, preferring thoracoamniotic shunt (82%). Postnatal: 75% respondents operate on asymptomatic patients, 18% before 6 months of age, 62% between 6 and 12 months of age, and 20% after 12 months of age. Risk of infection (86%), cancer (63%), and symptoms development (62%) are indications for surgery in asymptomatic CPAM. Sixty‐three percent prefer a thoracotomy. Lobectomy is the preferred procedure (58% respondents). Motivations against surgery include lesion <1 cm (64%), risk of postoperative complications (37%), and lack of evidence favoring surgery (27%). Seventeen percent respondents have seen at least one patient with CPAM with lung cancer, in 89% of the cases within the CPAM. Of all the respondents, 83% and 22% offered dedicated follow‐up and genetic screening, respectively. Conclusion Current pre‐ and postnatal management of CPAM lacks uniformity, particularly for surgical indication, timing, and approach. Efforts should be made toward standardization. Risk of CPAM‐associated cancer is not clear.

Guidelines of the Italian Society of Videosurgery in Infancy for the minimally invasive treatment of the esophageal atresia

Not available.