Surgical treatment of gastroesophageal reflux in children: a combined hospital study of 7467 patients.

OBJECTIVE: To review retrospectively the combined clinical experience with the surgical treatment of persistently symptomatic gastroesophageal reflux (SGER) in childhood from seven large children's surgery centers in the United States. DESIGN: During the past 20 years, 7467 children <18 years of age underwent antireflux operations for SGER at the seven participating hospitals. Fifty-six percent were neurologically normal (NN) and 44% were neurologically impaired (NI). The most frequent diagnostic studies were upper gastrointestinal series (68%), esophageal pH monitoring (54%), gastric emptying study (32%), and esophagoscopy (25%). The age at operation was under 12 months in 40% and 1 to 10 years in 48%. The type of fundoplication was Nissen (64%), Thal (34%), and Toupet (1.5%). A gastric emptying procedure was performed on 11.5% of NN patients and 40% of NI patients. Laparoscopic fundoplication was performed on 2.6% of patients. RESULTS: Good to excellent results were achieved in 95% of NN and 84.6% of NI patients. Major complications occurred in 4.2% of NN and 12.8% of NI patients. The most frequent complications were recurrent reflux attributable to wrap disruption (7.1%), respiratory (4.4%), gas bloat (3.6%), and intestinal obstruction (2.6%). Postoperative death occurred in 0.07% of NN and 0.8% of NI patients. Reoperation was performed in 3.6% of NN and 11.8% of NI patients. The results and complications were similar among the participating hospitals and did not seem related to the type of fundoplication used. CONCLUSION: The excellent results (94% cure) and low morbidity with gastroesophageal fundoplication with or without a gastric emptying procedure from a large combined hospital study indicate that operation should be used early for SGER in NN children and to facilitate enteral feedings and care in NI children.

Treatment of gastroesophageal reflux in children by Thal fundoplication.

The anterior fundoplication described by Thal has been used in treating gastroesophageal reflux surgically in 362 children at The Children's Mercy Hospital and at St. Luke's Hospital in Kansas City, Missouri, because medical therapy had failed or was inappropriate. Long-term results have been evaluated in regard to relief of reflux and relief of symptoms attributed to reflux. Of the 335 patients followed from 1 to 8 years, 90% had a satisfactory initial result. Five percent required reoperation for a recurrence of reflux due to failure of the fundoplication or development of a hiatus hernia. All recurrences developed with 5 months of the initial operation. Fifteen of 335 patients (4.5%) had persistent symptoms despite correction of the gastroesophageal reflux; in these patients, attributing the symptoms to reflux was incorrect. There were no deaths in this series of patients as a result of operation. The success rate of the Thal fundoplication in children compares favorably with that of the Nissen Fundoplication.

Care of infants with esophageal atresia, tracheoesophageal fistula, and associated anomalies.

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.

Gastroschisis complicated by intestinal atresia.

Gastroschisis complicated by intestinal atresia is a complex problem. Six cases are presented. A review of the literature and of our own cases shows a high mortality rate. Success or failure is related more to the pathology present than to any specific method of operative management. Resection and primary anastomosis is the favored method of treatment, as the intestine heals well in spite of its appearance. Primary closure of the abdominal wall musculature and skin is done whenever possible. A gastrostomy is used uniformly. Intravenous hyperalimentation is critical to survival of these babies and should be used early. The use of this therapeutic modality allows for the onset of gastrointestinal function spontaneously (often over prolonged periods of time) without nutritional deprivation. Intestinal atresias almost always are easy to identify in babies with gastroschisis. Extensive dissection and mobilization of this friable intestine is contraindicated in those babies in whom an atresia is not obvious but only suspected. In such cases the gastroschisis defect should be managed by whatever method is deemed appropriate and the baby observed while receiving intravenous nutritional support. If an atresia is present, it can be managed later in the baby's course by resection and primary anastomosis.

Minimally invasive repair of pectus excavatum: a single institution's experience.

BACKGROUND: The Nuss repair of pectus excavatum is a relatively new, minimally invasive surgical (MIS) alternative to the traditional open "Ravitch-type" operation. We have one of the larger single-center experiences to date, and we conducted this clinical study to evaluate our early experience, emphasizing initial outcome and technical modifications designed to minimize complications. METHODS: A retrospective chart review was performed on 112 patients who underwent 116 pectus excavatum repairs between January 1995 and January 2001. The Nuss procedure was performed in 80 patients, and open repair was performed in 32 patients. Information about demographics, deformity, operative course, complications, and early outcome was recorded. RESULTS: Operative duration was 143 minutes for the open group and 53 minutes for the Nuss MIS group (P <.001). Blood loss was 6 mL/kg for the open group and 0.5 mL/kg for the MIS group (P <.001). Postoperative hospitalization was 3.2 days for the open group versus 3.7 days for the MIS group (P<.05). CONCLUSIONS: The MIS pectus repair can be performed safely with minimal blood loss and reduced operative time. Short-term analysis of the quality of repair, including absence of preoperative symptoms, patient satisfaction, and cosmetic appearance are encouraging.

Tracheal agenesis.

Tracheal agenesis is a rare cause of respiratory distress in the neonatal period. Temporary survival depends on ventilation through the esophagus. Thirty-eight case reports of tracheal agenesis (including one from this institution) have appeared in the literature. In this paper, we present the case reports of our 2 patients and review the literature. Tracheal agenesis is associated with a wide variety of congenital anomalies, the most frequent being ventricular septal defect. A new classification encompassing seven types of tracheal agenesis is described.

Vascular anomalies and tracheoesophageal compression: a single institution's 25-year experience.

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 ligamentum, 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.

Gastroesophageal reflux.

GER is a major pediatric disease. The respiratory, nutritional, and inflammatory complications of this disease process, invisibly hidden in the gastroesophageal junction, have a profound effect on the quality and sometimes the very life of infants and children. The astute pediatrician and surgeon should always keep this disease process in mind when dealing with these problems of children. Having a high suspicion of GER is often necessary to find this hidden enemy. The Nissen fundoplication and the Thal fundoplication are equally successful in the treatment of GER in children. However, the complication rate with the Nissen fundoplication is higher than with the Thal. We feel that the Thal does an outstanding job of treating GER while maintaining normal gastroesophageal function in the developing child, and is therefore our operation of choice for GER.

The reimplanted ureter as a catheterizing stoma.

Some current trends in the management of neurogenic bladder have been toward utilization of the urinary bladder as a storage device with a continent catheterizing stoma. This study presents a procedure using the now standard Cohen ureteral reimplantation, followed some 6 weeks later by transureteroureterostomy and distal ureteral stomal formation, after the method of Mitrofanoff. This staged procedure appears to have allowed the recently reimplanted ureter to be divided and brought to the skin as a viable catheterizing stoma. We believe that this experimental study has demonstrated that the Cohen technique of ureteral reimplantation is a reasonable antecedent to the use of transureteroureterostomy and distal ureteral stoma creation for continent catheterization. Monfort has demonstrated this clinically in one patient, and we feel that this technique deserves more extensive clinical trials.

Bladder outlet obstruction after operation for ureterocele.

Two patients with ureterocele-induced bladder outlet obstruction are presented. In each instance, a broad-based diverticulum had developed in the floor of the bladder as a result of the muscular defect created by a simple ureterocele in one patient and an ectopic ureterocele in the other. This bladder diverticulum produced secondary obstruction of the bladder outlet during the act of voiding. Urinary diversion in one patient had been carried out because of bladder outlet obstruction and was being seriously considered in the other patient because of upper tract deterioration. After the correct diagnosis was established, reconstruction of the muscular defect eliminated the obstruction and reestablished satisfactory bladder function. Urinary diversion was thus prevented in one patient and undiversion accomplished in another, when the true obstructing nature of the bladder diverticulum was established and correct therapy instituted.

Gastric teratoma: the sixth reported case in a female infant.

Teratomas are derived from tissues of all three germ layers. Gastric teratomas are rare and represent 1% of all teratomas in boys. This report describes the sixth occurrence of gastric teratoma in a girl.

Ping-pong ball plombage for right postpneumonectomy syndrome in children.

BACKGROUND/PURPOSE: Right postpneumonectomy syndrome is a rare complication of pneumonectomy characterized by exertional dyspnea resulting from mediastinal shift into the empty hemithorax. Historically, this problem has been treated with thoracoplasty, muscle flap transposition, pericardial fixation, and plombage using a variety of materials. Significant postoperative complications have been reported with each of these methods. In an effort to avoid the problems known to be associated with other plombage materials, we have used sterilized ping-pong balls as plombage to treat the postpneumonectomy syndrome. METHODS: Two children with pneumonectomy syndrome were treated with ping-pong ball plombage. RESULTS: Symptoms improved immediately in both patients and postoperative chest x-rays in each showed a normal cardiac position. Neither child had a postoperative complication. Six-year follow-up chest films in both children have demonstrated some fluid filling of the ping-pong balls, but no clinical problems attributable to them have been noted. CONCLUSION: The authors conclude that ping-pong ball plombage appears to be a viable management option in children with right postpneumonectomy syndrome.

Early recognition and aggressive treatment of gastroesophageal reflux following repair of esophageal atresia.

Twelve patients with gastroesophageal reflux following repair of esophageal atresia are presented. Reflux produced recurrent stricture, failure to thrive, repeated pneumonitis, and in one patient, respiratory arrest and nearly death. Treatment consists of positional therapy or fundoplication operation both of which seem less successful in this combination of lesions than with GER and a normal esophagus. There was one death as a late postoperative complication of fundoplication.

Levator repair and posterior suspension for rectal prolapse.

An operative procedure for the management of rectal prolapse is described. It has the advantanges of simplicity, relative safety, short hospitalization, and lack of recurrence in the four patients presented.

Hepatic hemangioma: resection using hypothermic circulatory arrest in the newborn.

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.

Abbreviated esophageal pH monitoring as an indication for fundoplication in children.

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.

Congenital diaphragmatic hernia presenting due to gastrointestinal complications.

Congenital diaphragmatic hernia (CDH) presents beyond the first hours of life in 10% to 20% of cases. Presenting symptoms may be quite nonspecific, and are often gastrointestinal rather than respiratory in origin. We have recently had experience with five such cases, one in a newborn and four in older children. All presented with symptoms related to gastrointestinal complications of their diaphragmatic defect. In the newborn, gastric perforation had occurred, a complication of this anomaly not previously reported. The chest radiograph showed loops of bowel in the chest in all cases, allowing correct preoperative diagnoses. Urgent operative intervention was undertaken in each case with good results and no long-term morbidity. The risk of intestinal strangulation in the late-presenting CDH patient warrants emergent surgical management, which should be rewarded by uniform survival with few complications. Although preoperative stabilization may decrease the severity of pulmonary vasospasm in the newborn with respiratory failure, delay may increase the risk of bowel infarction in the older child presenting with gastrointestinal symptoms.

Esophageal duplication cyst with esophageal web and tracheoesophageal fistula.

The authors report the case of lower cervical/upper thoracic esophageal duplication associated with an obstructing esophageal web. This presented in the newborn period as an esophageal atresia. Initial resection of the web and closure of the fistula were performed. The duplication was excised electively at 2 months of age. Persistent symptomatic tracheomalacia required aortopexy, after which the child recovered uneventfully. There are only three other reports (four cases) in the literature of esophageal atresia (or variants) associated with esophageal duplication.

Postradiation renovascular hypertension.

Radiation injury to arteries can represent a significant complication of therapeutic irradiation, even when the dosage used has not been excessive as judged by approved protocols. Children in whom therapeutic abdominal irradiation has been used should be monitored indefinitely for the development of hypertension. The presence of hypertension in such children with normal blood urea nitrogen (BUN) and creatinine, and without proteinuria, should prompt investigation for a renovascular lesion. Standard bypass procedures are usually effective, although the long-term success may be compromised by continuing changes in affected vessels.

Esophageal atresia with double tracheoesophageal fistula.

Ninety cases of esophageal atresia (EA) with double tracheoesophageal fistula (TEF) from the literature and 4 new cases are examined. The incidence may be more common than is generally recognized with incidences up to 5.3% reported. The pre-, intra-, and postoperative diagnosis may be difficult, and half have been missed initially with almost half of these being first recognized at autopsy. During repair of EA and TEF the proximal esophagus should be mobilized looking for a proximal fistula. Many proximal TEF are missed at the initial operation and discovered in the postoperative period. The symptoms, diagnosis and treatment of the unrecognized proximal TEF are similar to that for isolated TEF. Results should be good since this problem occurs in large babies with fewer and less complex associated conditions.