Endoscopic evaluation of the esophagus in infants and children immediately following intraoperative use of transesophageal echocardiography.

OBJECTIVE: Intraoperative transesophageal echocardiography (TEE) has evolved as an essential technique for use during pediatric cardiac surgery; however, few studies have evaluated the safety of TEE in children. This series reports endoscopic examination of the esophagus following intraoperative TEE in pediatric patients. METHODS: Fifty children undergoing congenital heart surgery underwent flexible esophagoscopy that was performed after completion of their heart surgery and after the removal of the transesophageal echo probe. The patients' ages ranged from 4 days to 10 years old, and their weight ranged from 3.0 to 39.8 kg, with a mean weight of 12.6 kg. RESULTS: Thirty-two of 50 patients (64%) had abnormal results shown on esophageal examinations; this occurred more frequently in the subset of patients weighing < 9 kg. No long-term feeding or swallowing difficulties were noted in any of the 48 patients who survived. CONCLUSIONS: Intraoperative TEE in infants and children frequently caused mild mucosal injury. Care must be exercised in the insertion and manipulation of the probes.

Detrimental effects of standard medical therapy in congenital diaphragmatic hernia.

OBJECTIVE: To evaluate the impact of a nonstandard ventilation strategy on survival in congenital diaphragmatic hernia (CDH). BACKGROUND: Despite recent advances, including nitric oxide, CDH remains an unsolved problem with a mortality rate of 35% to 50%. Hyperventilation and alkalization remain common therapies. METHODS: In 1992, the authors prospectively abandoned hyperventilation and alkalization. Patients are lightly sedated and ventilated with the lowest pressure providing adequate chest movement, and the rate is set to patient comfort. Nitric oxide and extracorporeal membrane oxygenation (ECMO) are reserved for life-threatening instability. Surgical repair is delayed 1 to 5 days. Sixty consecutive patients are compared with 29 previous patients treated with hyperventilation and alkalization, 13 before and 16 after the availability of ECMO. RESULTS: Overall, 47 of 60 patients (78%) in study era 3 survived compared with 2 of 13 (15%) in the hyperventilation era and 7 of 16 (44%) in the hyperventilation/ECMO era (p < 0.0001). The disease severity and the incidence of associated anomalies did not differ between groups. To compare management strategies, patients who had treatment withheld because of lethal associated conditions were then removed from analysis. Peak inspiratory pressure and arterial pH were lower (p < 0.0001) and Paco2 was higher (p < 0.05) in era 3 than in the previous eras. The rate of pneumothorax (1.9%) decreased (p < 0.0001). In era 3, survival was 47 of 53 (89%) treated patients, and 23 of 25 inborn patients with isolated CDH survived (92%). CONCLUSIONS: Nonstandard ventilatory support of patients with CDH has led to significantly improved survival rates. This study sets a survival benchmark and strongly suggests the negative effects of hyperventilation and alkalization.

Congenital diaphragmatic hernia survival and use of extracorporeal life support at selected level III nurseries with multimodality support.

BACKGROUND: Congenital diaphragmatic hernia (CDH) has been cited to have a mortality rate of 50%. There have been multiple studies at individual institutions demonstrating potential benefits from various strategies including extracorporeal life support (ECLS), delayed repair, and lower levels of ventilator support. There has been no multicenter survey of institutions offering these modalities to describe the current use of ECLS and survival of these infants. In addition, the relationship between the number of patients with CDH managed at an individual institution and outcome has not been evaluated. METHODS: We queried 16 level III neonatal intensive care centers on the use of ECLS and survival of infants with CDH who were treated during 2 consecutive years (1993 to 1995). Data are presented as mean +/- SEM, median, and range. RESULTS: Data were collected on 411 patients. Of these, 71% +/- 8% were outborn and 8% +/- 3% were considered nonviable. Overall survival of CDH infants was 69% +/- 4% (range, 39% to 95%). The survival rate of infants on ECLS was 55% +/- 4%, whereas survival of infants not requiring ECLS was significantly increased at 81% +/- 5% (p = 0.005). The mean rate of ECLS use was 46% +/- 2%. There was no correlation between the number of cases per year at an individual institution and overall survival, ECLS survival, or ECLS use (r = 0.341, 0.305, and 0.287, respectively). There was also no correlation between case volume at an individual institution and ECLS survival (r = 0.271). CONCLUSIONS: The current survival rate and rate of ECLS use in infants with CDH at level III neonatal intensive care units in the United States are 69% +/- 4% and 46% +/- 2%, respectively. There is no correlation between the yearly individual center experience with managing CDH and rate of ECLS use or outcome.

Congenital diaphragmatic hernia. Epidemiology and outcome.

Congenital diaphragmatic hernia is a relatively common birth defect. It affects about 1114 babies a year in the United States. Reported survival averages 60% but may be significantly lower. We do not understand the etiology of CDH. Its association with other anomalies and several distinct patterns of presentation suggest that more than one cause may exist. There is a high degree of variability in both treatment and outcomes, but no data exist to allow a rigorous comparison of the efficacy of various treatment strategies. Stratification of patients into more homogeneous groups will be a necessary prerequisite for the design of meaningful comparative trials. The incidence of the lesion prevents any single institution from accruing sufficient patients to conduct such a trial. An ad hoc multicenter study group (the Congenital Diaphragmatic Hernia Study Group) has been formed for this purpose. This organization has begun collecting data with an initial goal of developing a stratification scheme. Prospective data collection should allow verification of several of the estimates made in this article. Current data make it clear that CDH represents a major cause of perinatal morbidity and mortality.

Extradural myxopapillary ependymoma: report of two cases and review of the literature.

The cauda equina is the most frequent location for ependymomas, particularly the myxopapillary variant, which generally arises from the filum terminale. These tumors have a characteristic histopathologic pattern and are usually easily recognized. The occurrence of these tumors in an extradural, sacrococcygeal, or subcutaneous location may prove challenging, particularly in the absence of any obvious central nervous system connection. We describe two such extradural cases, one with multiple regional and distant metastases and the other with multiple recurrences. The origin of these tumors from subcutaneous sacrococcygeal ependymal rests is postulated on the basis of earlier reports. Clinical and histopathological features are described and a review of the literature is presented.

Surgical conditions of the neonatal intestinal tract.

Many different pathophysiologic conditions can affect the neonatal intestinal tract and ultimately require surgical intervention. The symptomatology is primarily that of bowel dysfunction and obstruction. A thorough understanding of these disease processes is necessary of the managing physician. With this knowledge, a systematic course of resuscitation, evaluation, and treatment can be instituted, usually with predictably gratifying results for patient, family, and physician alike.

Unilateral pulmonary agenesis, esophageal atresia, and distal tracheoesophageal fistula.

A small-for-gestational-age, premature infant with the combination of unilateral pulmonary agenesis, esophageal atresia, and distal tracheoesophageal fistula was treated successfully by early gastrostomy and delayed fistula division with esophagoesophagostomy. Only 2 other successfully treated cases have been reported previously. Both were full-term infants treated with early division of the tracheoesophageal fistula and esophagoesophagostomy. Gestational age, size, and associated medical problems need to be considered when planning operative therapy for these babies.

Posterior hepatodiaphragmatic interposition of the colon complicated by appendicitis.

Posterior hepatodiaphragmatic interposition is a rare malposition of the cecum that persists over time but is usually of little or no clinical importance. However, when inflammation occurs in the appendix, the symptoms and signs are those of subphrenic disease rather than abdominal disease and may be misleading to the attending physicians. Radiologists should report the presence of anterior and posterior hepatodiaphragmatic interposition and should differentiate between the two.

Pediatric liver cysts and abscesses.

Cystic liver lesions in children pose diagnostic and therapeutic dilemmas. When discovered during evaluation of abdominal distension, the likely cause is benign hepatic cyst. Surgical intervention aimed at resection is highly curative. Echinococcal disease, usually distinguishable from nonparasitic cystic disease, is rare in children in the United States. Therapy is aimed at sterilization and complete excision of hydatid tissue. Cystic liver lesions in septic children represent liver abscesses, pyogenic or amebic, which are frequently fatal if not diagnosed early and treated appropriately. Ultrasonography and computed tomography have simplified the diagnosis and treatment of these diseases, but a high index of suspicion is required because the signs and symptoms of liver abscess in children are nonspecific.

Wilmer fundus camera stimulator.

The Wilmer fundus camera stimulator has been designed to allow testing of visual function at precise retinal locations under direct visualization. We use a modified fundus camera with a deep red source and IR vidicon to allow for both low levels of illumination and visualization of the fundus. The stimulus location is plotted directly on a real-time digitized image of the fundus using computer-generated graphics, and fixation can be monitored as testing proceeds. The fundus image with stimulus location can be stored on floppy disk for later retrieval and analysis. Both static and kinetic perimetry can be performed. This paper describes the fundus camera stimulator in detail, compares it to existing types of fundus perimeters, and provides clinical examples of its use.