Craig T. Albanese

Anesthetic concerns for robot-assisted laparoscopy in an infant.

A 2-mo-old infant with biliary atresia was scheduled for laparoscopic Kasai with robot assistance. Before surgery, a practice trial maneuvering the cumbersome robotic equipment was performed to ensure rapid access to the patient in case of emergency. IV access, tracheal intubation, and arterial line placement followed inhaled anesthesia induction with sevoflurane. Robotic setup took 53 min and severely limited patient access. No adverse events occurred during the procedure requiring the removal of the robotic equipment, and the patient was discharged after a stable postoperative recovery. Advance preparation is required to maximize patient safety during robotic surgery.

Fetal liver position and perinatal outcome for congenital diaphragmatic hernia

Despite advances in postnatal care, patients born with a congenital diaphragmatic hernia (CDH) suffer substantial morbidity and mortality. The present study was undertaken to determine the prognostic influence of prenatally‐diagnosed liver herniation in the hemithorax in fetuses with CDH.

Thoracoscopic repair of esophageal atresia and tracheoesophageal fistula: a multi-institutional analysis.

Objectives:For the past 60 years, successful repair of esophageal atresia (EA) and distal tracheoesophageal fistula (TEF) has been performed via a thoracotomy. However, a number of reports have described adverse musculoskeletal sequelae following thoracotomy in infants and young children. Until now, only a few scattered case reports have detailed an individual surgeons success with thoracoscopic repair of EA/TEF. This multi-institutional review represents the largest experience describing the results with this approach. Methods:A cohort of international pediatric surgeons from centers that perform advanced laparoscopic and thoracoscopic operations in infants and children retrospectively reviewed their data on primary thoracoscopic repair in 104 newborns with EA/TEF. Newborns with EA without a distal TEF or those with an isolated TEF without EA were excluded. Results:In these 104 patients, the mean age at operation was 1.2 days (±1.1), the mean weight was 2.6 kg (±0.5), the mean operative time was 129.9 minutes (±55.5), the mean days of mechanical ventilation were 3.6 (±5.8), and the mean days of total hospitalization were 18.1 (±18.6). Twelve (11.5%) infants developed an early leak or stricture at the anastomosis and 33 (31.7%) required esophageal dilatation at least once. Five operations (4.8%) were converted to an open thoracotomy and one was staged due to a long gap between the 2 esophageal segments. Twenty-five newborns (24.0%) later required a laparoscopic fundoplication. A recurrent fistula between the esophagus and trachea developed in 2 infants (1.9%). A number of other operations were required in these patients, including imperforate anus repair in 10 patients (7 high, 3 low), aortopexy (7), laparoscopic duodenal atresia repair (4), and various major cardiac operations (5). Three patients died, one related to the EA/TEF on the 20th postoperative day. Conclusions:The thoracoscopic repair of EA/TEF represents a natural evolution in the operative correction of this complicated congenital anomaly and can be safely performed by experienced endoscopic surgeons. The results presented are comparable to previous reports of babies undergoing repair through a thoracotomy. Based on the associated musculoskeletal problems following thoracotomy, there will likely be long-term benefits for babies with this anomaly undergoing the thoracoscopic repair.

One-stage transanal Soave pullthrough for Hirschsprung disease: a multicenter experience with 141 children.

Background: The surgical management of Hirschsprungs disease (HD) has evolved from the original 3-stage approach to the recent introduction of minimal-access single-stage techniques. We reviewed the early results of the transanal Soave pullthrough from 6 of the original centers to use it. Methods: The clinical course of all children with HD undergoing a 1-stage transanal Soave pullthrough between 1995 and 2002 were reviewed. Children with a preliminary stoma or total colonic disease were excluded. Results: There were 141 patients. Mean time between diagnosis and surgery was 32 days, and mean age at surgery was 146 days. Sixty-six (47%) underwent surgery in the first month of life. Forty-seven (33%) had the pathologic transition zone documented laparoscopically or through a small umbilical incision before beginning the anal dissection. Mean blood loss was 16 mL, and no patients required transfusion. Mean time to full feeding was 36 hours, mean postoperative hospital stay was 3.4 days, and 87 patients (62%) required only acetaminophen for pain. Early postoperative complications included perianal excoriation (11%), enterocolitis (6%), and stricture (4%). One patient died of congenital cardiac disease. Mean follow-up was 20 months; 81% had normal bowel function for age, 18% had minor problems, and 1% had major problems. Two patients required a second operation (twisted pullthrough, and residual aganglionosis). One patient developed postoperative adhesive bowel obstruction. Conclusion: To date, this report represents the largest series of patients undergoing the 1-stage transanal Soave pullthrough. This approach is safe, permits early feeding, causes minimal pain, facilitates early discharge, and presents a low rate of complications.

Fetoscopic temporary tracheal occlusion for congenital diaphragmatic hernia: prelude to a randomized, controlled trial

OBJECTIVE As previously reported, high postnatal mortality seen in fetuses with congenital diaphragmatic hernia (CDH) with liver herniation and low lung-to-head ratio (LHR) appears to be improved in fetuses who undergo fetoscopic temporary tracheal occlusion (TO). To test whether further evolution of this technique produces results that justify a randomized controlled trial comparing prenatal intervention to postnatal care, the authors analyzed 11 additional cases and the cumulative experience with 19 cases. METHODS The authors analyzed retrospectively the outcome of 11 new and 8 previously reported cases of fetoscopic temporary tracheal occlusion. Various factors were studied including maternal morbidity, antenatal outcome, physiologic lung response, and neonatal course. RESULTS Temporary TO can be accomplished using 3 5-mm radially expanding uterine ports without hysterotomy. Obstetric morbidity included mild pulmonary edema in 6 cases, chorioamniotic separation and premature rupture of membranes in 12 patients, and preterm labor and delivery in all patients. Thirteen of 19 (68%) neonates survived for 90 days after delivery; one died in utero, and 5 died after birth. Late mortality included one death caused by sepsis and 2 by complications associated with tracheostomies. Morbidity from gastroesophageal reflux requiring Nissen fundoplication, tracheal injury requiring repair or tracheostomy, and recurrent hernias after diaphragmatic repair were characteristic in longterm survivors. CONCLUSIONS Fetoscopic temporary TO may improve outcome in poor-prognosis fetuses with CDH. However, complications related to tracheal dissection, premature delivery and late morbidity are significant. This experience has led to simpler techniques for fetoscopic tracheal occlusion and to an National Institutes of Health-sponsored randomized controlled trial comparing fetoscopic tracheal occlusion with optimal postnatal care.

Perineal one-stage pull-through for Hirschsprung's disease

PURPOSE The aim of this study was to present the strategy of a one-stage repair of Hirschsprungs Disease (HD) performed via a transanal approach. METHODS Ten consecutive neonates and one toddler underwent transanal repair for biopsy-proven HD. A rectosigmoid transition zone was suggested by contrast enema in all patients. The mean age at operation for the neonates was 4 days. A mucosal dissection was begun 0.5 cm proximal to the dentate line. Once the correct plane was established, up to 15 cm of bowel can be resected without ligating vessels or performing a transabdominal dissection. The proximal extent of dissection was delineated by the presence of ganglion cells seen on frozen section analysis. RESULTS The mean operating time was 105 minutes. There were no intraoperative or postoperative complications. All children had the presence of ganglion cells confirmed postoperatively on permanent sections. The mean hospital stay was 2 days. All children averaged three to six bowel movements per day without oral or enema therapy. CONCLUSIONS The perineal one-stage operative pull-through (POOP) procedure for Hirschsprungs disease is a quick and easy adaptation of a well-described technique of transanal mucosectomy. Long-term follow-up will be required to determine whether bowel function is better that that seen after traditional staged repairs.

Percutaneous gastrojejunostomy versus Nissen fundoplication for enteral feeding of the neurologically impaired child with gastroesophageal reflux

To determine the optimal method of providing enteral feeding to neurologically impaired children with gastroesophageal reflux, Nissen fundoplication with simultaneous gastrostomy tube placement (NGT) was compared with anterograde percutaneous gastrojejunostomy (APGJ), a nonsurgical procedure performed under fluoroscopic guidance. The records of 112 neurologically impaired children with gastroesophageal reflux were retrospectively reviewed; 68 had undergone NGT and 44 APGJ. Follow-up data were available for 45 NGT patients (mean age, 6.4 years) and 34 APGJ patients (mean age, 7.9 years). Mean follow-up was 1.8 years in the NGT group and 2.5 years in the APGJ group. Complications resulting from either procedure were classified either as major, which included treatment failures or morbidity resulting in prolonged hospitalization, or as minor, those requiring outpatient treatment only or not directly caused by the procedure. The NGT group had a significantly higher incidence of major complications in comparison with the APGJ group (33.3% vs 11.8%, p < 0.05). Ten patients (22.2%) in the NGT group required reoperation for complications; six required a second NGT for wrap hernia, failure, and continued gastroesophageal reflux. Two patients (5.9%) in the APGJ group required surgery for complications; one of these eventually required an NGT, and the other had an intussusception that necessitated a small-bowel resection. Minor complications were more common in the APGJ group than in the NGT group (44.1% vs 6.6%); the majority of complications were related to the jejunostomy tube. Premature replacement or reinsertion of the jejunostomy tube was necessary in 14 APGJ patients (32%). The mortality rate was 8.8% in the NGT group and 5.9% in the APGJ group (p = not significant). No death occurred within 30 days of either procedure. We conclude that APGJ is a safe alternative method for feeding the neurologically impaired child with gastroesophageal reflux.

Congenital diaphragmatic hernia without herniation of the liver: Does the lung-to-head ratio predict survival?

The purpose of the present study was to determine the ability of lung‐to‐head ratio to predict survival and need for extracorporeal membrane oxygenation support in fetuses with left congenital diaphragmatic hernia without herniation of the liver into the chest. The perinatal records of 20 fetuses with isolated left congenital diaphragmatic hernia without herniation of the left lobe of the liver into the chest were reviewed. Fetuses were stratified into two groups depending on lung‐to‐head ratio: those with a ratio of less than 1.4 (historically a poor prognosis group) and those with a ratio of greater than 1.4. The outcome of both groups was compared with chi‐square analysis. Eight of 11 fetuses with a lung‐to‐head ratio greater than 1.4 survived, whereas 8 of 9 fetuses with a ratio of less than 1.4 survived. No differences were noted in the need for extracorporeal membrane oxygenation support or survival between the two groups. Fetuses with a prenatally diagnosed left congenital diaphragmatic hernia without herniation of liver into the chest have a favorable prognosis even in the presence of a low lung‐to‐head ratio.

Maternal Fertility Is Not Affected by Fetal Surgery

The purpose of this report is to assess the impact of fetal surgery on future maternal fertility, subsequent pregnancy outcome, and the incidence of pregnancy complications. Retrospective data were collected on 70 mothers who underwent fetal surgery between April 1981 and June 1996. Indications for open hysterotomy fetal surgery included congenital diaphragmatic hernia (n = 44), congenital cystic adenomatoid malformation of the lung (n = 11), urinary obstruction (n = 9), sacrococcygeal teratoma (n = 4), heart block (n = 1), and acardiac-acephalic twin reduction (n = 1). The following data were obtained: number of pregnancy attempts, number of successful pregnancies, pregnancy outcome including obstetrical and neonatal complications, and infertility after fetal surgery. There were 45 respondents, of whom 35 attempted subsequent pregnancies. Thirty-two were successful, resulting in 31 livebirths. Two women had a strong prefetal surgery history of infertility, 1 has only attempted to conceive for 3 months. We report this experience because the effect of open fetal surgery on futrue fertility is such an important question for our patients and referring physicians. This analysis suggests that hysterotomy and open fetal surgery has a negligible impact on maternal fertility.

Doxycycline sclerotherapy as primary treatment of head and neck lymphatic malformations in children

PURPOSE The authors report their experience with doxycycline sclerotherapy as primary treatment of head and neck lymphatic malformations (LMs) in children. METHODS A retrospective chart review was used to collect data on 11 patients treated with doxycycline sclerotherapy for LMs of the head and neck at our institution since 2003. Radiographic imaging allowed classification of patient LM as macrocystic, microcystic, or mixed according to previously published guidelines. Only patients with macrocystic or mixed lesions were offered doxycycline sclerotherapy. Radiographic imaging and physical examination were used to determine efficacy of treatment. After each treatment, the clinical and radiographic response was characterized as excellent (> or = 95% decrease in lesion size), satisfactory (> or = 50% decrease in volume and asymptomatic), or poor (< 50% decrease in volume or symptomatic). RESULTS Eleven patients underwent a total of 23 sclerotherapies with an average of 2 treatments per patient (range, 1-4). All 7 patients with macrocystic lesions achieved complete clinical resolution with an average radiographic resolution of 93%. The 4 patients with mixed lesions achieved only partial clinical resolution and an average of 73% radiographic resolution. No patient experienced any adverse effects related to the treatment. At a median follow-up of 8 months, 2 patients (18%) experienced lesion recurrence in the setting of concomitant infection. CONCLUSION Doxycycline sclerotherapy is safe and effective as a primary treatment modality for macrocystic and mixed LMs of the head and neck in the pediatric population.