Background. Vascular rings are uncommon anomalies in which preferred strategies for diagnosis and treatment may vary among institutions. In this report, we offer a description of our approach and a review of our 25-year experience.Methods. A retrospective review was conducted of all pediatric patients with symptomatic tracheoesophageal compression secondary to anomalies of the aortic arch and great vessels diagnosed from 1974 to 2000.Results. Thirty-one patients (38%) of eighty-two patients (mean age, 1.7 years), were identified with double aortic arch, 22 patients (27%) with right arch left ligamenturn, and 20 patients (24%) with innominate artery compression. Our diagnostic approach emphasized barium esophagram, along with echocardiography. This regimen was found to be reliable for all cases except those with innominate artery compression for which bronchoscopy was preferred, and except those with pulmonary artery sling for which computed tomography or magnetic resonance imaging, in addition to bronchoscopy, were preferred. Left thoracotomy was the most common operative approach (70 of 82; 85%). Ten patients (12%) had associated heart anomalies, and 6 (7%) patients underwent repair. Complications occurred in 9 (11%) patients and led to death in 3 (4%) patients.Conclusions. In our practice, barium swallow and echocardiography are sufficient in diagnosing and planning the operative strategy in the majority of cases, with notable exceptions. Definitive intraoperative delineation of arch anatomy minimizes the risk of misdiagnosis or inadequate treatment. (C) 2001 by The Society of Thoracic Surgeons.

Gastroesophageal reflux (GER) often develops in children who have undergone prior repair of esophageal atresia/tracheoesophageal fistula (EA/TEF). Fundoplication is necessary in many of these children. The complete wrap (Nissen) fundoplication is often used in this setting. However, poor results have been noted, with a mean failure rate of 30% reported in four recent studies. A partial wrap fundoplication for GER associated with EA/TEF is theoretically attractive, because the poor esophageal motility and diminished acid clearance (already physiologically present) is exacerbated by a complete wrap fundoplication. The authors reviewed their extensive experience with partial wrap (Thal) fundoplication in EA/TEF to determine if the failure rate was better than that reported for the Nissen fundoplication. In the past 18 years, the authors performed 1,467 fundoplication procedures. During the same period, 143 children underwent repair of EA/TEF. Fifty-nine children underwent fundoplication after a previous EA/TEF repair. Most of the fundoplications (58 of 59, 98%) were Thal procedures. Defining failure strictly as a need for reoperation, the failure rate in our series was 15% (9 of 59 children). Compared with the failure rate in the 1,408 non-EA/TEF patients (61 of 1408, 4.3%), results were significantly worse for the EA/TEF group (P > .001). The failure rate of Thal fundoplication performed for GER in the EA/TEF population is substantially higher than the non-EA/TEF patients. The same factors responsible for the development of reflux in these children (poor acid clearance, altered motility, esophageal shortening) may contribute to the higher failure rate. Although partial wrap fundoplication frequently failed (15%), the results were still substantially better than those reported for Nissen fundoplication in these children (30% failure rate).

A persistent left superior vena cava (PLSVC) is the most common anomaly of the major veins. However, an absent right superior vena cava and PLSVC is much less common; fewer than 100 cases have been reported in the world literature in patients with situs solitus. This rare variation was encountered in a newborn requiring extracorporeal membrane oxygenation (ECMO) for meconium aspiration and interfered with venous cannulation. The anomaly was not suspected before an unusual cannula position was observed on the chest radiograph, and it was confirmed by bedside venography. Adequate venous cannulation was obtained through the anomalous vessel using a flexible catheter, allowing for an uneventful ECMO course. The epidemiology and embryology of this incidental anomaly are reviewed, and the methods used to circumvent the difficulties it presented in this case are described.

Intraesophageal pH recordings from 40 patients with abnormal studies who underwent fundoplication during a 1-year period were evaluated. Scores were recalculated for the first 12, 16, and 20 hours, respectively. Five of the recordings were for less than 24 hours, but were grossly abnormal and mathematically could not have normalized at 24 hours. The percentage of studies which were normal at 12, 16, and 20 hours were 20%, 15%, and 0%, respectively. One fifth of the patients would have been denied a fundoplication based on 12-hour results. In addition, 75 consecutive pH recordings were evaluated prospectively. Total scores were determined for the first 12, 16, and 20 hours with accuracies of 85%, 93%, and 92%, respectively. The sensitivity of 12-hour monitoring was only 78%. All abbreviated studies had a low rate of false positives; however, any false positives may be significant when used to determine the need for an operation. Abbreviated studies were associated with high false-negative rates (up to 52% for the 12-hour studies) for the calculated mean duration of sleep reflux, a parameter that has been found to be useful in identifying patients with respiratory symptoms secondary to gastroesophageal reflux. The use of abbreviated pH monitoring is discouraged.

Anterior cricoid resection is an effective procedure to relieve subglottic stenosis. This is well documented in adults, although reports of the procedure in growing airways are limited. Over an 11-year period, seven pediatric patients underwent anterior cricoid resection for recalcitrant subglottic stenosis. In four patients, the stricture was secondary to prolonged intubation, one developed subglottic stenosis following a high placement of tracheostomy for epiglottitis and another had congenital subglottic stenosis. One child had subglottic stenosis combined with laryngotracheoesophageal cleft and more distally located tracheoesophageal fistula. All patients had failed to respond to previous treatment: dilatations (3 to 20), steroid injection (3 patients), and Evan's tracheoplasty (2 patients). All patients had an excellent result from anterior cricoid resection. The median age of children undergoing anterior cricoid resection was 3 years. There was no mortality. Tracheostomy decannulation was accomplished within 12 weeks following operation in all patients. It was necessary to remove a tracheal granuloma in one patient. Anterior cricoid wedge resection leaving the posterior portion of the cricoid in place is done to avoid recurrent nerve injury. It is a relatively simple and effective procedure. There has been minimal morbidity and no mortality. Follow-up from 1 to 11 years shows no recurrence of stenosis. There has been normal laryngeal and airway growth.

Using a posterior repair and rectal suspension procedure for those patients who need surgical treatment of rectal prolapse, we have treated 46 patients over a period of 17 years at Children's Mercy Hospital in Kansas City, MO. One patient with caudal dysgenesis died of multiple congenital anomalies following two unsuccessful attempts at posterior repair and suspension. Four patients developed a recurrence afterwards, which was found to be due to sigmoid intussusception and, presumably, had played a major part in their original prolapse. Two of these required resection, one from the transanal approach and one from the transabdominal approach. One resolved spontaneously and another is as yet unresolved. Three patients had minor mucosal prolapse that was transient and two patients had extrusion of silk sutures but continued to have a very satisfactory result. Overall, 42 patients had satisfactory resolution of their rectal prolapse. Three of the four patients who had unsatisfactory results had associated anomalies that contributed to their poor outcome.

Hepatic hemangioma (hemangioendothelioma) is a benign vascular tumor with significant morbidity and mortality. Two newborn infants underwent partial hepatectomy for treatment of massive hepatic hemangioma, one with atrial septal defect and secondary congestive heart failure, and the other with symptomatic marked hepatomegaly. Cardiopulmonary bypass with induced deep hypothermic circulatory arrest was used in both patients in order to facilitate resection and prevent sudden massive blood loss. One infant also had closure of an atrial septal defect in addition to the partial hepatectomy. Both infants survived without complication and showed complete resolution of preoperative symptoms. This technique coupled with the use of fibrin glue is of value in infants with large hepatic hemangiomata and cardiac decompensation in the neonatal period.

There are conflicting views on the pathogenesis of the intestinal malfunction seen in infants with gastroschisis. It has been variously ascribed to abnormalities of ganglion cells and smooth muscle elements, intestinal ischemia, and the "peel" which invests the serosa of the intestine. Review of the clinical and experimental literature showed only limited information on the histology of the eviscerated human intestine. In order to add to this data base, and to further investigate the pathogenesis of the intestinal malfunction from a histologic standpoint, we reviewed surgical and autopsy material from our experience with 105 neonates with gastroschisis. Ten specimens were satisfactory for evaluation from a standpoint of tissue integrity. The specific mural components of mucosa, submucosa, muscularis, and ganglion cells were examined and found to be either normal, or to show nonspecific abnormalities that varied from case to case, and were related mostly to intestinal infarction due to compromise of the gut at the site of the gastroschisis defect. In six patients, this progressed to atresia formation. The most consistent abnormalities were found in the serosal layer with its peel. Using special stains, the peel was found to be composed largely of fibrin and collagen. Based on this study, we feel that edema and ischemic changes, though often present, are much less prominent than the peel, as the leading histologic abnormality of the intestine of gastroschisis. Squamous epithelial cells were seen in the peel in four cases, suggesting that the peel had been "appliqued" onto the serosa of the herniated fetal gut.

A newborn infant with intestinal obstruction resulting from intussusception associated with neonatal small left colon syndrome is described. Differences between neonatal and infantile intussusception are detailed. Chronic loose intussusception in utero is proposed as one cause of the neonatal small left colon syndrome. Consideration should be given to doing a rectal biopsy and a sweat chloride test to differentiate neonatal small left colon syndrome from Hirschsprung's disease and cystic fibrosis, respectively, when the clinical diagnosis is not readily apparent.

It is evident from studies of boys who suffered a surgical catastrophe at a young age and were then assigned a female sex role that cultural and environmental influence are a potent determinant of a child's gender identity. It is imperative that parents have their child's sex assignment firmly fixed in their minds as early as possible. Early surgical correction of a child with ambiguous genitalia to conform to the sex of assignment will serve greatly to reinforce appropriate behavior in the parent. Such surgical intervention for diagnostic and reconstructive purposes is both desirable and safe in the first weeks of life.

One hundred consecutive patients with esophageal atresia or tracheoesophageal fistula, or both, were treated at The Children's Mercy Hospital during the past 14 years. Each patient was evaluated and a plan for therapy was formulated depending on the type and urgency of concomitant disease. Healthy patients were treated by primary repair. Those with respiratory disease were treated before repair by gastrostomy, upper pouch suction, and antibiotics. In the small premature infant and the patient with major associated anomalies, repair of the esophageal atresia and tracheoesophageal fistula was postponed by gastrostomy, upper pouch suction, and parenteral nutrition while the concomitant disease was treated. Fifteen patients with life-threatening associated anomalies required operation before esophageal repair and 13 of them survived. During the initial hospitalization two deaths related to esophageal atresia occurred, whereas three prerepair and two late (2 1/2 and 3 months) postrepair deaths related to associated anomalies occurred. Thus, 93 of the 100 patients left the hospital alive. The increased survival is due not only to improved care of esophageal atresia and tracheoesophageal fistula, but also to a more aggressive approach to the treatment of the serious associated anomalies.

When corrective surgery for tetralogy of Fallot was accomplished through the use of a transannular GORE-TEX patch with a pericardial unicusp, the right-ventricular end-diastolic volumes of all patients studied within a year of the surgery were within the normal ranges because of decreased pulmonary valve regurgitation. The right-ventricular ejection fraction was also only slightly depressed, indicating preservation of right-ventricular function. All patients were noted to maintain normal stroke volumes and normal systolic indices. In contrast, the patients who had transannular patches placed without unicusps showed significantly elevated right-ventricular end-diastolic volumes and lower right-ventricular ejection fractions. These resulted from markedly dilated right-ventricular outflow regions in conjunction with enlarged right-ventricular chambers, which manifested as large dyskinetic areas in the anterior right-ventricular walls.

Two patients were observed who had transient quadriceps paresis following local inguinal block for postoperative pain control following inguinal herniorrhaphy.

Uretero pelvic junction (UPJ) obstruction is a common cause of hydronephrosis in infants. The frequent use of fetal ultrasound is allowing early (prenatal) diagnosis of numerous uropathies previously delayed until the child either became symptomatic or had a palpable mass. Newborn with severe obstruction often has marked improvement following correction, therefore, early diagnosis and operation are important. From 1993 to 2002, 21 patients were operated on for severe UPJ obstruction who were diagnosed before 6 weeks of age. Only 10 patients (63%) had antenatal ultrasonographic diagnosis, the remaining were diagnosed by postnatal ultrasound and IVP or radionuclide scan for palpable renal enlargement or for associated anomalies. Eighteen of them had unilateral and three had bilateral obstruction. Twenty-four pyeloplasties were done; all pyeloplasties were dismembered with tailoring of the dilatated renal pelvis. Postoperative renal function was followed with laboratory blood test, urine test or radionuclide scan or IVP. Postoperative complications included urinary tract infection in three patients and postoperative stenosis in one patient were seen. No mortality occurred on infants in unilateral but one occurred in bilateral obstruction. Also, there was one unrelated late death. We report documented functional improvement with minimal complications in unilateral or bilateral pyeloplasty in newborns with UPJ obstruction. We recommend that if the initial scan shows substantially reduced function in the obstructed kidney, a pyeloplasty (rather than nephrectomy) generally should be performed, because the newborn kidney has tremendous capacity for improvement in renal function following decompression.

The technique of anterior fundoplication for gastroesophageal reflux originally described by Alan Thal has been used in our institution in 605 patients. The description of the technique is the purpose of this paper. A brief description of the results in these 605 patients are also presented.

Neonatal sepsis due to group B beta-hemolytic Streptococcus (GBS) is reported to occur in about 1 out of 330 live births. Right-sided Bochdalek hernia (RBH) occurs in about 1 of 20,000 live births. The combination of group B streptococcal sepsis and delayed appearance of a right Bochdalek hernia is an infrequently reported phenomenon--18 patients have been previously reported in the English literature. We add four patients from our own experience to these previous reports. Since approximately 10% to 15% of the newborn population are exposed to group B Streptococcus we suspect that the inadequate diaphragmatic motion on the side of the Bochdalek hernia predisposes the child to development of septicemia and/or pneumonitis. Once the etiology has been established and appropriate antibiotic therapy instituted, progressive improvement in the patient's course should be seen. This is in contrast to a very significant mortality rate in many of the patients having early onset GBS. Any child, therefore, surviving early onset GBS only to deteriorate again, should be suspected of having an associated right Bochdalek hernia, and diagnostic steps should be taken to evaluate the integrity of the right diaphragm.

The medical literature has been deluged with articles on the relation between gastro-esophageal reflux (GER) and asthma. In an effort to piece together the complex puzzle, investigators from all disciplines have gathered their patients with wheezing and heartburn and studied the epidemiology, the possible cause or effect mechanisms and the therapeutic response to GER treatment. Indeed, since humans first began to hunker down and work together to discuss interesting observations, the world has begun to breathe easier. Epidemiological evidence for a GER/asthma association suggests that about three-fourths of asthmatics, independent of the use of bronchodilators, have acid GER, increased frequency of reflux episodes, or heartburn; and 40% have reflux esophagitis. Physiological studies suggest that 2 separate mechanisms are involved in the GER/asthma relationship: (1) a vagally mediated pathway and (2) microaspiration. In any given patient, however, there is no acceptable diagnostic method available to confirm the presence or absence of GER-induced asthma. Clinical trials, using antireflux medical therapy and antireflux surgery have begun to provide some clues about GER-related pulmonary symptoms. The trials of medical therapy using acid suppressing drugs (e.g. histamine-2 receptor antagonists) have ranged from no benefit to modest improvement of only nocturnal asthma symptoms. Studies with proton-pump inhibitors are underway. In uncontrolled surgical studies, antireflux surgery has resulted in partial or complete remission of asthma symptoms in a large proportion of patients. Despite the uncontrolled nature of these studies, many patients have had dramatic subjective improvement in pulmonary symptoms. It appears for now that clinical trials are the only available means to assess whether medical or surgical treatment of GER in patients with both GER and asthma improves the symptoms of asthma and decreases the need for pulmonary medications. One conclusion is certain: We no longer can ignore the important co-existent nature of these 2 afflictions.