Long gap esophageal atresia and esophageal replacement: moving toward a separation?

BACKGROUND/PURPOSE: Treatment of long gap esophageal atresia (EA) is still a major challenge. Gastric transposition and colon interposition are the 2 most popular choices for esophageal replacement, but there is general agreement that the child's own esophagus is the best. The aim of the study was to critically evaluate the feasibility and outcome of primary repair of long gap EA with or without tracheoesophageal fistula (TEF) by direct esophago-esophageal anastomosis as the only technique. METHODS: Seventy-one neonates with EA+/-TEF were considered. Nineteen cases were classified as long gap (> or =3 cm). All infants underwent either primary or shortly delayed repair. In the latter group, a gastrostomy was performed along with an x-ray evaluation of the gap a few days before surgery (mean age, 46.4 days). To avoid disruptive anastomotic force, all infants were kept paralyzed and mechanically ventilated for an additional 6 days after esophageal anastomosis. Before starting feeding, postoperative esophagogram was done on day 7. Endoscopy was done routinely, starting 1 month after surgery; pH monitoring was conventionally performed at 1 year of age or even earlier, should gastroesophageal reflux disease (GERD) be suspected. Follow-up ranged from 11 months to 7 years. RESULTS: In all 19 long gap EA infants an esophago-esophageal anastomosis was performed. Six of them (31%) required an anterior esophageal flap to bridge residual gap. Complications included minor anastomotic leak in 2 cases and anastomotic stricture (<5mm) in 12 (80%) cases, which were treated with an average of 5 dilatations (1 of which with resection of the stricture). GERD occurred in 8 cases (53.3%), of which, 3 required fundoplication. None of the patients had esophageal swallowing difficulties or persistent dysphagia. Two children experienced food aversion. Mean hospital stay was 66.2 (22 to 230) days. There were 4 deaths (very low birth weight, 1; associated anomalies, 1; and late sepsis, 2). CONCLUSIONS: Considering heat gap determination remains imprecise, it seems possible to conclude that in a well-established tertiary care level referral center: (1) long gap EA could be treated successfully with primary repair and anastomosis; (2) strictures and GER represent the most frequent postoperative problem, but additional procedures required seem "acceptable" to maintain the patient's own esophagus and avoid replacement; (3) esophageal substitution in long gap EA should be reserved for cases in which a previous attempt of esophageal reconstruction failed.

Severe congenital diaphragmatic hernia (CDH): a critical analysis of eight years' experience.

UNLABELLED: OBJECTIVES. 1) To define the best outcome of severe Congenital Diaphragmatic Hernia (CDH); 2) to critically evaluate deaths in order to identify possible criteria of exclusion from ECMO; and 3) to identify CDHs which could benefit from ECMO. MATERIALS AND METHODS: 63 severe CDHs, 35 (55.6 %) survivors and 28 (44.4 %) nonsurvivors, subdivided into 2 groups according to age at death: Group I dying at 12 < or = 24 hours, and Group II dying at > 24 hours after birth. The three groups were compared on the basis of prenatal diagnosis, polyhydramnios, gestational age, birth weight, pneumothorax, best values of postductal PaCO 2 and PaO 2, clinical and echocardiographic signs of persistent pulmonary hypertension, and severity of pulmonary hypoplasia (i.e., body weight to bilateral lung weight ratio at autopsy). RESULTS: PaCO 2, PaO 2 and degree of pulmonary hypoplasia were significantly worse in Group I compared to Group II and to survivors. PaCO 2 and PaO 2 in Group II did not differ significantly from those of survivors. CONCLUSIONS: In severe CDH it is possible: 1) to achieve a survival rate of 56 % without ECMO; 2) to identify a group of patients (Group I = 27 %) with severe pulmonary hypoplasia who would probably die even with ECMO; and 3) to identify a group of patients (Group II = 17 %) who might benefit from ECMO treatment.

Esophagogastric dissociation versus fundoplication: Which is best for severely neurologically impaired children?

PURPOSE: Neurologically impaired children (NIC) often have swallowing difficulties, severe gastroesophageal reflux, recurrent respiratory infections, and malnutrition. Bianchi proposed esophagogastric dissociation (EGD) as an alternative to fundoplication and gastrostomy. The authors compared these 2 approaches. METHODS: Twenty-nine consecutive symptomatic NIC refractory to medical therapy were enrolled in a prospective study and divided into 2 groups: A (n = 12), NIC who underwent fundoplication and gastrostomy; B (n = 14), NIC who underwent EGD. Three were excluded because of previous fundoplication. Anthropometric (percentage of the 50th percentile/age of healthy children) and biochemical parameters, respiratory infections per year, hospitalization (days per year), feeding time (minutes), and "quality of life" (parental psychological questionnaire, range 0 to 60), were analyzed (t test and Mann-Whitney test) preoperatively and 1 year postoperatively. Complications were recorded. RESULTS: Compared with group A, group B presented a statistically significant increase of all anthropometric and nearly all biochemical parameters with a statistical difference in terms of respiratory infections, hospital stay, feeding time, and psychological questionnaire. In group A, 2 bowel obstructions, 1 tight fundoplication, 1 dumping syndrome, and 3 failures of fundoplication occurred. Group B presented 1 anastomotic stricture, 1 paraesophageal hernia, and 1 bowel obstruction. CONCLUSIONS: Compared with fundoplication and gastrostomy, EGD offered better nutritional rehabilitation, reduction in respiratory infections, and improved quality of life. EGD can be rightfully chosen as a primary procedure.

[Anesthetics management in patients with epidermolysis bullosa]. / Condotta anestesiologica in un paziente affetto da epidermolisi bollosa.

The anesthetic management used in a 4-yr-old boy with dystrophic epidermolysis bullosa (DEB) submitted to long-lasting urological surgery is reported. Medical treatment of cutaneous bullae in various stages of healing was undertaken preoperatively. Other preoperative therapies were performed to treat sepsis and to improve poor nutritional state. Different measures were used during monitoring and inhalation induction and maintenance of anesthesia in order to prevent frictional trauma to the skin and oropharyngeal mucosa, leading to blistering. Anesthesia and surgery proceeded uneventfully, while surgical complications and sepsis occurred postoperatively. However the patient was discharged from intensive care unit to surgical ward on 35 postoperative days without evidence of oropharyngeal bullae and with a good improvement of his cutaneous condition. The conclusions are drawn that, even if the patient with DEB represents a challenge to the anesthesiologist, a long-lasting surgical intervention can be performed successfully. The main aspect in the anesthetic management of these patients is the detailed knowledge of all problems that anesthesiologist must face and solve to preserve skin and mucosa integrity, avoiding the risk of severe complications.

A prospective, randomized clinical comparison of sevoflurane and halothane in children.

BACKGROUND: The goal of the present multicenter investigation was to compare in a prospective and randomized study the induction, the maintenance and the recovery characteristics of halothane and sevoflurane when used in paediatric patients. METHODS: With the approval of the Ethical Committee and the parental written informed consent, 64 children aged 3-12 years, receiving general anaesthesia for urological, abdominal, and orthopaedic surgery, were studied. After oral flunitrazepam (0.05 mg kg-1), general anaesthesia was randomly induced by either sevoflurane (start: 1%, maximum: 7%, n = 32) or halothane (start: 0.5%, maximum: 4.5%, n = 32) and a 60% N2O in oxygen mixture until the loss of eyelash reflex (induction time). Then the trachea was intubated (if necessary, a muscle relaxant was administrated), and the concentrations of the anaesthetic vapours were adjusted in order to maintain cardiovascular stability until the end of surgery. The following times were recorded: time of extubation, time for having purposeful movements, time of eyes opening and readiness for discharge from the recovery area, as well as the occurrence of untoward events during either induction of, maintenance of, or recovery from anaesthesia. Before surgery and 24 hr after the procedure, blood was collected in order to measure serum creatinine and BUN. RESULTS: No differences in induction time, extubation time, side effects and postoperative renal function were observed between the two groups. Four patients in each group received muscle relaxants to perform intubation (p = NS). When compared to halothane group, children receiving sevoflurane had shorter times of showing purposeful movements (median: 9 min versus 15.5 min, p < 0.005), emergence from anaesthesia (median: 12 min versus 18 min, p < 0.05) and achieving readiness to be discharged (median: 18 min. versus 30 min, p < 0.005). Sevoflurane group also showed a more stable heart rate during the induction period than halothane one (p = 0.05). DISCUSSION: Sevoflurane is as effective as halothane in providing smooth and rapid induction of anaesthesia, while recovery is considerably faster and haemodynamic tolerance is better if compared to halothane; this suggests that sevoflurane could be an useful substitute for halothane in pediatric patients.

[Prevention of hemorrhagic cerebral injury in newborn and premature infants subjected to mechanical ventilation]. / La prevenzione del danno emorragico cerebrale nel neonato e nel prematuro sottoposti a ventilazione meccanica.

The newborn brain, and even more so the brain of the premature child, can be considered as an authentic target organ for numerous pathological conditions, some of which exist outside the central nervous system (changes involving primarily both respiratory function and cardiocirculatory function with serious repercussions at encephalic level). In the premature, this greater "vulnerability" is related to the reduced or absent capacity for self-regulation of the cerebral blood low (mechanism influenced negatively by hypoxia, hypercapnia and metabolic acidosis conditions) and the important role played by numerous factors in protecting newborns from haemorrhagic damage. Of these the most important are the state of prematurity, the presence of vascular, intravascular and extravascular changes, the effects exerted on cerebral haemodynamics by mechanical ventilation and by certain drugs employed in treatment. In mechanically ventilated newborns and premature, prevention of haemorrhagic damage (periendoventricular) is currently based on the application of clear-cut protocols of intensive and rehabilitative treatment. The following form part of these protocols: low damage ventilation techniques (high frequencies, low PJP, low MAP), curarisation (to avoid fluctuations in cerebral blood flow), neuroprotection (phenobarbital), the use of substances and drugs which, by exploiting different mechanisms, go to reduce the extent of the haemorrhage (vitamin E, indomethacin, ethamosylate, tranexamic acid).(ABSTRACT TRUNCATED AT 250 WORDS)