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Purpose: The aim of this study was to determine the important factors in the development and subsequent treatment of postoperative paraesophageal hernia (PPEH)., Methods: A retrospective analysis was performed in 464 consecutive children (ages 3 days to 18 years) for PPEH after a primary antireflux operation performed at a Children's Hospital and University Hospital between 1985 and 1997. All operations included a crural repair, but the Nissen fundoplication was performed with (n = 162) and without (n = 70) plication of the esophagus to the crus at 3 points. Patients with and without PPEH were compared with respect to the type of antireflux operation, the patient's age at operation, and the preoperative and postoperative clinical courses. A preoperative corrected gastric emptying value was obtained from a radionuclide gastric emptying study in 289 patients. The treatment of PPEH also was examined., Results: The incidence of PPEH in our patients was 4.5% (21 of 464). Although there was a lower incidence of PPEH in patients with crural plication compared with patients without crural plication during Nissen fundoplication (5 of 162, 3% v 7 of 70, 10%; P = .035), 2 patients with crural plication had a postoperative esophageal leak. Patients with PPEH had a significantly increased prevalence of gagging before the initial antireflux operation compared with patients without PPEH (3 of 21, 14.3% v 7 of 443, 1.6%; P = .007). A higher prevalence of slow corrected gastric emptying preoperatively also was seen in patients with PPEH compared with patients without PPEH (8 of 15, 53% v 79 of 274, 29%; P = .046). The prevalences of central nervous system disease, young age (<6 months) at initial operation, and a particular type of antireflux operation were not higher in patients with PPEH. Nine patients with a small PPEH treated by simple observation alone subsequently had resolution of symptoms., Conclusions: Patients who have gagging or slow corrected gastric emptying before an antireflux operation are at higher risk for a postoperative paraesophageal hernia. Patients with a small postoperative paraesophageal hernia can be treated nonoperatively. Crural plication of the esophagus during Nissen fundoplication reduces the occurrence of postoperative paraesophageal hernia, but also may result in significant morbidity.

Hypothesis: Infants with rotational abnormalities of the midgut mesentery are at high risk for gastroesophageal reflux disease (GERD) and for sudden infant death (SID) from GERD., Design: A survey of the prevalence of GERD and the risk factor for SID from GERD in a case series of infants treated for congenital anomalies that include intestinal malrotation. Eighty-one (89%) of the infants studied for GERD had a mean follow-up of 23.2 months (median, 12 months)., Setting: Patients treated in 2 tertiary care centers consisting of a children's hospital and a university medical center., Patients: Two hundred eighty-six consecutive infants were treated for congenital anomalies from September 1, 1985, through May 31, 1998. The patients selected for study were 91 infants who had 18- to 24-hour esophageal pH monitoring performed and no prior operation on the stomach or esophagus. The studied infants had intestinal malrotation either alone (n = 55) or associated with a repaired abdominal wall defect (n = 23) or congenital diaphragmatic hernia (n = 13). Of the 91 infants, 34 were asymptomatic at the time of esophageal pH monitoring., Interventions: Eighteen- to 24-hour esophageal pH monitoring was used to determine the presence of GERD (abnormal pH score >2 hours postcibal) and the risk factor for SID from GERD (type I or III reflux pattern in combination with a prolonged mean duration of sleep reflux)., Main Outcome Measures: The prevalence of GERD and the risk factor for SID from GERD. The follow-up of GERD was reported as a combination of clinical outcome and subsequent extended esophageal pH monitoring., Results: Of the 91 infants studied, 80 (88%) had GERD and 26 (29%) had the risk factor for SID from GERD. Of 55 infants with intestinal malrotation alone, 52 (95%) had GERD, and 20 (36%) had the risk factor for SID from GERD. Although GERD was found in 19 (83%) of 23 patients with repaired abdominal wall defects, the prevalence of the risk factor for SID from GERD was significantly lower (13% [3 patients]; P = .03) than in patients with intestinal malrotation alone. The prevalence of GERD in infants with repaired congenital diaphragmatic hernia was significantly lower (69% [9/13]; P = .02) than in infants with intestinal malrotation alone but not for the prevalence of the risk factor for SID from GERD (23% [3/13]; P = .19). Both symptomatic and asymptomatic patients had similar prevalences of GERD (91% [52/57] vs. 82% [28/34], P = .17) and for the risk factor for SID from GERD (31% [18/57] vs. 24% [8/34]; P = .28). On follow-up, the prognosis for GERD in infants with intestinal malrotation was better in the infants who were asymptomatic than in those who were symptomatic at the initial extended esophageal pH monitoring., Conclusions: The prevalence of GERD in infants with intestinal malrotation is high, and the prevalence of the risk factor for SID from GERD is a significant concern. The prevalence of GERD is lower in infants with congenital diaphragmatic hernia. Infants with repaired abdominal wall defects have a lower prevalence of the risk factor for SID from GERD. We recommend careful evaluation and follow-up of infants with intestinal malrotation for problems, such as SID, from GERD.

Purpose: There is a paucity of quantitative and reproducible follow-up data on childhood operations for gastroesophageal reflux disease (GERD). With the development of minimally invasive techniques for antireflux operations in children, there is a need to quantitatively determine immediate outcomes for such operations performed by laparotomy for comparison., Methods: A retrospective review of 385 children (age range, 1 week to 15 years) who had a primary antireflux operation in a Children's or University Hospital performed by laparotomy between 1983 and 1997, and who also had an extended esophageal pH study performed within the first 12 postoperative weeks, was conducted. The operations performed included Nissen fundoplication (n = 135), Thal fundoplication (n = 195), and Boerema gastropexy (n = 55). An immediate postoperative failure of the operation to control GERD was defined as an abnormal esophageal pH score persisting up to the twelfth postoperative week., Results: Eleven patients (2.9%) were classified as having an immediate postoperative failure of their operation to control GERD. An additional three patients had an abnormal esophageal pH score 2 weeks postoperatively, which subsequently reverted to a normal esophageal pH score by 12 weeks. The immediate postoperative failure rate was 1.5% (2 of 135) for the Nissen fundoplication, 1.5% (3 of 195) for the Thal fundoplication, and 10.9% (6 of 55) for the Boerema gastropexy. A higher failure rate (five patients, 36%) was seen for the first 14 patients who underwent a Boerema gastropexy during the learning curve period for this operation before 1985, and by excluding these patients the failure rate was 2.4% (1 of 41) after 1985. There was no significantly increased probability of immediate postoperative failure in patients with central nervous system disorders, prematurity, repaired esophageal atresia, or gastric emptying abnormalities. Only 5 (36%) of the 14 children with persisting symptoms suggestive of GERD had immediate postoperative failure of their operation., Conclusions: Extended esophageal pH monitoring during the first 12 postoperative weeks is a helpful tool to assess the immediate outcome of antireflux operations in children because clinical symptoms alone may be unreliable. The immediate failure rate for an antireflux operation performed in children by laparotomy is very low and seems to be unaffected by comorbid factors.

Cytogenetic analysis performed on a 14-month-old boy with a primary retroperitoneal/paraspinal alveolar rhabdomyosarcoma showed the presence of a der(13)t(1;13)(q23;q32) resulting in partial trisomy of the 1q23-->qter region and loss of the 13q32-->qter region. The present case is discussed with reference to a similar case reported in the literature.

Vomiting is a very common symptom associated with gastroesophageal reflux disease (GERD) in infancy, although no longer the most common symptom seen in infants with GERD. A thorough evaluation of these patients with clinical history, physical examination, and sophisticated diagnostic procedures is necessary to identify that the vomiting is from GERD. Extended esophageal pH monitoring is the most reliable diagnostic procedure used to confirm the presence or absence of GERD, and provides additional information regarding prognosis and the relationship of the GERD to respiratory complaints. The latter information allows the clinician to determine the optimal type of antireflux therapy offered to the vomiting infant with GERD. Reevaluation of the vomiting infant with suspected recurrent GERD after antireflux operation should not differ from the evaluation performed in an untreated infant with vomiting from suspected GERD.

Although true vomiting is an unusual manifestation of pharyngeal or swallowing disorders in infants and children, the forceful regurgitation of feedings seen with respiratory distress in these children may be interpreted by the clinician as vomiting episodes. This scenerio usually is caused by structural abnormalities of the upper airway or swallowing discoordination placing food as an obstruction in the airway during feedings. A good clinical history and physical examination of affected children often will disclose an anatomic cause for regurgitated feedings, but swallowing disorders are more subtle and usually require a complete swallowing evaluation. The essential components in treating these problems in children include the removal of the airway obstruction or rehabilitation of feeding and swallowing. The approach to treatment is often multidisciplinary in nature and highly individualized.

This report summarizes a worldwide survey of achalasia in childhood. The data were obtained by circulating a questionnaire to pediatric surgeons throughout the world. Information was received regarding 175 patients, and the analysis is presented. Childhood achalasia appears to be more common in boys, and familial cases are rare. Regurgitation of food and dysphagia are the most frequent symptoms in affected children. Although 18% of patients had symptom onset during infancy, only 6% of the patients were identified as having achalasia during infancy. The survey suggests only a fair clinical response to transthoracic esophagocardiomyotomy and a good to excellent clinical response to transabdominal esophagocardiomyotomy. A cooperative effort between several institutions will permit the development of prospective treatment protocols for children with achalasia.

Parents caring for a child with gastroesophageal reflux are at risk for impaired home maintenance management because of the challenges they face such as the emotional impact, financial concerns, and difficulty maintaining the strict regime. Nursing interventions related to family education and support assist the families in becoming knowledgeable and compliant while fostering normal growth and development.

An understanding of gastroesophageal reflux disease in infants and children by the clinician requires a working knowledge of 18- to 24-hour esophageal pH monitoring and the motility disorders of the esophagus and stomach that may be associated with gastroesophageal reflux disease. The results of surgical therapy for childhood gastroesophageal reflux disease cannot be assessed accurately without this knowledge. Antireflux operations can be tailored to the child's situation, which includes a combination of clinical symptoms and findings on objective tests for reflux and associated alimentary-tract motility disorders. The presence of severe complications from gastroesophageal reflux disease in "asymptomatic" infants and children is a troublesome and not yet fully defined problem. Special areas include the documentation of gastroesophageal reflux disease as a cause of SIDS, the increased reporting of Barrett's esophagus and adenocarcinoma of the esophagus in childhood, and the effect of associated alimentary-tract motility disorders in children with CNS disease who have gastroesophageal reflux disease requiring surgical intervention.

The coexistence of biliary atresia and small bowel atresia has been described. However, attempts to correct the biliary atresia at the same time as small bowel atresia repair have not been detailed. In 1986, a 2,170-g female was delivered at 36 weeks' gestation. Postpartum, duodenal obstruction was suspected after oral feedings were not tolerated. At 37 hours after birth, laparotomy showed unsuspected biliary atresia in addition to intestinal malrotation, duodenal web, and type IIIb jejunal atresia with an apple-peel configuration of the upper jejunum and mesentery supplied by the superior mesenteric artery with normal small bowel length and normal ileal mesentery. A modified Roux-en-Y hepatic portoenterostomy was performed using the blind end of the apple-peel configuration of jejunum, with a side-to-side duodenojejunostomy 25 cm from the portoenterostomy. Microscopic examination of the fibrous extrahepatic biliary tissue showed a disarray of small bile ductules. The maximal ductal size at the portoenterostomy site was 600 microns. A liver biopsy specimen showed normal architecture with eosinophils infiltrating the portal triads and periductal areas. Postoperatively, she has maintained normal growth and development with no episodes of cholangitis. Liver biopsies at 1 year and 3 years were normal. At 4 years, total and conjugated bilirubin, serum proteins, liver enzymes, coagulation factors, and trace elements were normal. We conclude that simultaneous correction of biliary atresia and small bowel atresia can be accomplished in the neonate with excellent early results.

Continuous gastric pH monitoring offers the opportunity to evaluate gastric acid secretion in humans without the need for collection of gastric aspirates. However, gastric emptying may be an important variable for the accurate measurement of gastric acid secretion using continuous gastric pH monitoring. We conducted an in vitro study (phase I) to evaluate the effect of gastric emptying on gastric pH. The in vitro study used a laboratory model in which continuous pH monitoring of a standard formula (Nutramigen, 100 mL) in a reservoir was performed while varying both the reservoir emptying rate (10% to 80%) over 1 hour and the amount of added acid (2 to 15 mEq/h: in vivo equivalent of 6 to 45 mEq/m2 BSA/h). An in vivo study (phase II) was then performed in 28 infants less than 6 months of age who had gastroesophageal reflux (GER) documented by 18- to 24-hour esophageal pH monitoring. At the end of esophageal pH monitoring, the pH electrode was advanced into the stomach for measurement of gastric pH continuously for 2 hours after a feeding of Nutramigen formula (300 mL/m2 BSA). Each patient also had a radionuclide gastric emptying study performed with the same volume of Nutramigen formula. In the in vitro model, the reservoir pH (% time pH less than 4) was proportional to the reservoir emptying rate only when acid was added at less than 15 mEq/h (less than 45 mEq/m2 BSA/h). Reservoir pH was independent of reservoir emptying rate when acid was added at 15 mEq/h (45 mEq/m2 BSA/h).(ABSTRACT TRUNCATED AT 250 WORDS)

Gastroesophageal reflux (GER) has been known to occur in infants but was thought to be normal. As a result of increased recognition of GER and a clear documentation of GER with extended (18 to 24 hour) esophageal pH monitoring, several severe complications of GER in children have become apparent. An immature cardiorespiratory system is susceptible to some complications of GER such as apnea, choking, recurrent cough or wheezing, and recurrent aspiration pneumonia. Noncardiorespiratory complications include weight loss, esophagitis, anemia, irritability, posturing, malnutrition, and developmental delays. Nursing assessment contributes to a complete clinical picture and the subsequent treatment choice of the physician. To form an accurate assessment of the child with suspected GER, the nurse must be aware of the symptoms and complications of this condition and must precisely execute diagnostic studies, particularly extended esophageal pH monitoring. Nursing responsibilities also include providing a safe yet stimulating environment for the child, teaching parents to participate in the child's care, supporting parents through hospitalization, and preparing both the parents and child for discharge and follow-up care at home.

Gastroesophageal reflux (GER) has been known to occur in infants but was thought to be normal. As a result of increased recognition of GER and a clear documentation of GER with extended (18 to 24 hour) esophageal pH monitoring, several severe complications of GER in children have become apparent. An immature cardiorespiratory system is susceptible to some complications of GER such as apnea, choking, recurrent cough or wheezing, and recurrent aspiration pneumonia. Noncardiorespiratory complications include weight loss, esophagitis, anemia, irritability, posturing, malnutrition, and developmental delays. Nursing assessment contributes to a complete clinical picture and the subsequent treatment choice of the physician. To form an accurate assessment of the child with suspected GER, the nurse must be aware of the symptoms and complications of this condition and must precisely execute diagnostic studies, particularly extended esophageal pH monitoring. Nursing responsibilities also include providing a safe yet stimulating environment for the child, teaching parents to participate in the child's care, supporting parents through hospitalization, and preparing both the parents and child for discharge and follow-up care at home.

Gastroesophageal reflux (GER) has been a suspected cause of infant deaths and sudden infant death syndrome (SIDS). We examined our 10-year experience with 499 consecutive infants 6 months of age or less who had extended (18 to 24 hours) esophageal pH monitoring performed to evaluate for GER. The data extracted from the esophageal pH records included the pH score, the pattern of GER (type I, II, or III), and the mean duration of reflux during sleep (ZMD). All infants were followed to determine the occurrence and cause of death during the first year of life. Of the 19 deaths found in the series, three were classified as SIDS and two were in-hospital deaths caused by reflux-induced aspiration. All five of these infants who died had a prolonged ZMD (greater than 3.8 minutes) and received either basic medical (n = 4) or no (n = 1) antireflux therapy. Four infants also had the type I pattern of GER. There was a 9.1% (4/44; 95% confidence limits, 2.5% to 21.7%) incidence of reflux-related or SIDS deaths in infants with type I GER and a prolonged ZMD who were treated nonoperatively, compared with none (0/83, P = .03) in the same group of infants treated with antireflux surgery. The incidence of SIDS was higher in infants with type I GER and a prolonged ZMD who were treated nonoperatively (3/44, 6.8%) compared with all other infants treated nonoperatively (0/265, 0%; P = .003).(ABSTRACT TRUNCATED AT 250 WORDS)

The coexistence of gastroesophageal reflux (GER) and respiratory symptoms has been reported in children, but identifying a reliable indicator of reflux-induced respiratory symptoms has been difficult. The mean duration of reflux episodes during sleep (ZMD), extracted from a 18- to 24-hour esophageal pH recording, has been presented previously as a reliable correlate of respiratory symptoms caused by GER in infants. To reexamine the accuracy of the ZMD we report 519 consecutive children with respiratory symptoms (aged 1 week to 15 years) who were referred for documentation of GER by 18- to 24-hour esophageal pH monitoring. Follow-up information was obtained on 388 patients 1 to 115 months later (mean, 23 months). From the follow-up information, 259 children could be reliably classified; 128 as having reflux-related and 131 as having reflux-unrelated respiratory symptoms. None of the 131 children with reflux-unrelated respiratory symptoms had a prolonged ZMD (greater than 3.8 minutes). In comparison, 119 of the 128 children (93%) with reflux-related respiratory symptoms had a prolonged ZMD (P less than .001). The nine children who had a normal ZMD and improvement in their respiratory symptoms with the successful control of GER had advanced esophagitis (n = 4), central apnea (n = 3), or associated disorders in which respiratory symptoms improved with the avoidance of oral feedings (n = 2). In conclusion, the ZMD was confirmed to correlate directly with both the presence and absence of respiratory symptoms caused by GER in children.

An association between gastroesophageal reflux (GER) and central nervous system (CNS) disease has been suggested, but not defined clearly in children. We report on 613 children (ages 1 week to 16 years; mean, 16 months) studied at three institutions who were referred to the pediatric surgical service for documentation of GER by an abnormal pH score derived from 18- to 24-hour esophageal pH monitoring. Follow-up (range, 1 to 115 months; mean, 25 months) was obtained in 368 patients. One hundred thirty-two children had CNS disease documented prior to the evaluation for GER. In children older than 1 year, there was an increased incidence of GER (31/45, 69%) in those children with CNS disease compared with those without CNS disease (38/81, 47%; P = .014). On follow-up, only 4.6% (14/282) of children who were not diagnosed initially with CNS disease were found later to have overt CNS disease. There was no significant difference in the prevalence of newly diagnosed CNS disease in children with and without GER in long-term follow-up. In conclusion, in the population of children referred to the pediatric surgeon for evaluation of GER, children older than 1 year with CNS disease are at high risk to have associated GER documented by extended esophageal pH monitoring. Despite this association, those children with GER and no obvious CNS disease do not appear to be at increased risk to develop CNS disease.

The association between respiratory complications of gastroesophageal reflux (GER) and prematurity in infants has not been described completely. We studied 82 consecutive infants less than 6 months of age with major respiratory symptoms suspected to be caused by GER. Twenty-eight patients had bronchopulmonary dysplasia (BPD). Extended (18 to 24 hours) esophageal pH monitoring was used to document GER with a pH score. Respiratory complications were considered to be caused by GER if a prolonged mean duration of sleep reflux (ZMD) was found. Seventy-five of the 82 (91%) infants had documented GER, but only 45 (55%) had a prolonged ZMD. The incidence of GER was high regardless of the gestational age at birth, postconceptual age at time of study, and the presence of BPD. The incidence of a prolonged ZMD was higher in infants who were 34 to 39 weeks' gestation (10/12, 83%) than in infants who were less than 34 weeks' gestation at birth (15/37, 41%; P = .01). The incidence of a prolonged ZMD was lowest in infants 39 weeks or less postconceptual age at the time of study (4/14, 29%; P = .017). Most infants with BPD did not have a prolonged ZMD (12/28, 43%). However, 11 of the 12 (92%) infants with BPD and a prolonged ZMD showed dramatic improvement after effective antireflux therapy compared with 0 of 16 infants with BPD and normal ZMD (P less than .001).(ABSTRACT TRUNCATED AT 250 WORDS)

Persistent gastrointestinal symptoms are common postoperatively in children with intestinal malrotation. We investigated this problem in 14 children with intestinal malrotation who had a Ladd procedure (3 patients), gastroschisis repaired (6 patients), or omphalocele repaired (5 patients) between one month and 15 years prior to study. In 13 patients, gastric emptying was measured at 30 minutes (%GE30) and at 60 minutes (%GE60) following ingestion of 99m-Tc sulfur colloid in apple juice. We estimated the degree of gastric peristalsis using the %GE corrected for immediate postcibal gastroesophageal reflux (corrected %GE). Patients with vomiting exhibited slow gastric emptying compared to patients without vomiting (%GE30: 14.0 +/- 5.5 v 32.5 +/- 4.2, P less than .005). The slow gastric emptying was related to slow gastric peristalsis (corrected %GE30: 20.3 +/- 5.0 v 47.1 +/- 6.0, P less than .005). In all 5 patients with persistent bloating and diarrhea, gastric peristalsis was rapid at 30 minutes (corrected %GE30 = 56.7 +/- 4.2) and at 60 minutes (corrected %GE60 = 69.5 +/- 5.3). To assess the role of gastroesophageal reflux (GER) in persisting symptoms, all children had extended (18 to 24 hours) esophageal pH monitoring. Eleven (79%) of the 14 patients demonstrated GER by esophageal pH monitoring, including four of six patients without reflux symptoms. All ten children under two years of age demonstrated GER regardless of symptoms or congenital anatomic abnormality. In conclusion, GER is common in patients under two years of age with intestinal malrotation, but clinical symptoms seem related more to extreme variations in gastric peristalsis than to GER.

The association of gastroesophageal reflux (GER) and gastric emptying has been observed, but its importance is poorly understood. We compared 18- to 24-hour esophageal pH recordings with gastric emptying of radionuclide in apple juice in 109 children (2 weeks to 16 years old) referred for an evaluation of GER. A pH score and the pattern of GER (type I, II, and III) were obtained by esophageal pH monitoring. Gastric emptying was measured at 30 minutes (%GE30) and at 60 minutes (%GE60). The measured gastric emptying of apple juice was similar for the normal child and for those with GER regardless of pattern type. An inverse linear correlation between postcibal reflex and measured gastric emptying was demonstrated only by the eight asymptomatic children with a normal pH score (%GE30, r = -.9603, P less than .001; and %GE60, r = -.8263, P less than .05). If the contribution of postcibal reflux on gastric emptying was eliminated, then the effective gastric emptying was increased in many children with the type I (44%, P less than .05), type II (46%, P less than .05), or type III (60%, P less than .05) pattern of GER as compared with children with a normal reflux pattern (7%). In conclusion, measured gastric emptying of clear liquids is not necessarily decreased in most children with GER. Furthermore, many children with GER demonstrate an increased effective gastric emptying even though measured gastric emptying may not appear increased.

Extended (18 to 24 hour) esophageal pH monitoring establishes the diagnosis of gastroesophageal reflux (GER) utilizing a pH score, and relates respiratory symptoms to GER when the mean duration of sleep reflux (ZMD) is prolonged. A disadvantage of this method is the expense of overnight hospitalization. We performed extended esophageal pH recordings in 66 consecutive children (1 week to 15 years old) being evaluated for GER. Six portions of the 18- to 24-hour esophageal pH recording were compared to the complete record in an attempt to define the relative accuracy of abbreviated monitoring periods. The abbreviated monitoring periods included the 30 minutes after apple juice feedings (30 minutes AJ), the 2 hours after apple juice feedings (2 hours PC AJ), the 2 hours after milk-formula feedings (2 hours PC MF), the 8 hours with two feedings of apple juice (8 hours AJ), the 8 hours with two feedings of milk formula (8 hours MF), and the first 12 hours of recording (1st 12 hours). The accuracy relative to the 18- to 24-hour recording was poor for 30 minutes AJ, 2 hours PC AJ, and 2 hours PC MF periods (30% to 58%). An improved accuracy occurred during 8-hour AJ periods (29/31, 94%) in children without respiratory symptoms. Although the accuracy in patients with respiratory symptoms was best during 8-hour MF (31/35, 89%) and 1st 12-hour (33/35, 94%) periods, a high false-negative rate for the ZMD (31% to 41%) during abbreviated pH monitoring indicates that many patients with reflux-induced respiratory symptoms will be unrecognized.(ABSTRACT TRUNCATED AT 250 WORDS)

A serious consequence of antireflux surgery is postoperative small bowel obstruction in an infant who cannot speak and has been rendered unable to vomit. We reviewed the operative rate for small bowel obstruction following all antireflux operations (210 Nissen fundoplications, 16 Hill fundoplications, 12 modified Thal fundoplications, and 3 Boerema anterior gastropexies) performed on children at our institution between January 1977 and July 1984. Eighteen patients (17 Nissen fundoplications, one Hill fundoplication) were operated upon for small bowel obstruction within two years after the primary operation. The most consistent clinical findings in these children were abdominal distention and a decreased frequency of bowel movements. For operations performed between January 1982 and July 1984, reoperation for small bowel obstruction was needed in 6.1% (6/99) of children following Nissen fundoplication as compared to 0.9% (6/649), P less than 0.001) of children following other major laparotomies. A combination of our experience with that reported by others suggests an estimated incidence of postoperative adhesive small bowel obstruction of 5.5% (24/436) for Nissen fundoplication, 0.9% (3/347) for modified Thal fundoplication, and 0.8% (1/126) for Boerema anterior gastropexy. The performance of a Nissen fundoplication has led to a significant rate of reoperation for small bowel obstruction compared with other major laparotomies and antireflux operations performed in children.

Gastroesophageal reflux is common in infants and children and is associated with a broad spectrum of symptoms and disease. The majority of young patients with reflux can be managed nonoperatively because growth and time usually bring maturation of the antireflux mechanisms. We feel that operation is justified and indicated for the child with complications of reflux that are not reversed by a six-week medical trial, and children with stricture or infants with reflux-related apnea should have operation without delay. In our experience, analysis of the 24-hour esophageal pH tracing has proved most useful to correlate respiratory symptoms with reflux. The Nissen fundoplication is the most effective and the most reliable antireflux procedure, but it must be constructed loosely to minimize the side effects of dysphagia and gas bloating. Because the population at risk for reflux disease has approximately a 50 per cent incidence of significant associated medical problems, morbidity and mortality from some of these problems is inevitable. There should be little or no mortality associated with the antireflux operation itself.

While controversy continues over the exact pathophysiology of and optimal therapy for phlegmasia cerulea dolens, increasing evidence favors the role of elevated interstitial pressure in the generation of extremity ischemia. We report the use of intramuscular pressure measurements in the assessment of a patient suffering massive venous occlusion of one leg. Significant elevations in pressure were found in association with symptomatic ischemia. Successful thrombectomy restored blood flow, relieved symptoms, and produced an immediate decrease in intramuscular pressure. In this disorder, measurement of compartment pressures can be useful in assessing the severity of venous insufficiency and in monitoring the response to therapy.

The technique of chromosomal positioning of inheritable traits (C.P.I.T.) to project relative positions of two or more traits at more than one gene locus was employed to test the validity of the Fisher-Race model of Rh structure. The results of statistical analysis of two large samples of Rh phenotype distributions suggest that all models based on vertically related, linked alleles are invalid. On the basis of further analysis, Wiener's concept of the structure of the human Rh-Hr gene locus appears most valid among current hypotheses.

The effect of eating on childhood gastroesophageal reflux (GER) is unclear. Twenty-eight asymptomatic children and 28 children with symptoms of GER were fed apple juice or milk-formula and observed for 3 hr postcibal. Distal esophageal pH was monitored continuously during this interval and used to quantitate the frequency and duration of GER. A period of frequent GER occurred for up to 2 hr after apple juice feedings in asymptomatic children, whereas symptomatic patients had frequent GER for longer periods. Compared to apple juice feedings, milk-formula feedings resulted in a decreased esophageal acidity for the first 2 hr. However, the type of feeding did not affect GER seen in asymptomatic children more than 2 hr postcibal. The frequency and duration of postcibal GER were not reduced by the upright position in either group. Effective medical treatment of symptomatic children did not eliminate the frequent GER within 2 hr of apple juice feedings, whereas the Nissen fundoplication usually eliminated all GER. The absence of GER episodes following apple juice correlated with the inability of most children to burp or vomit following antireflux surgery. Therefore, frequent GER for up to 2 hr after clear liquid meals is probably physiologic in children. The effective control of vomiting by medical or surgical therapy correlated best with a decrease in GER more than 2 hr postcibal.

In children, Stamm tube gastrostomy can initiate gastroesophageal reflux (GER) or worsen preexisting GER. We identified a possible mechanism for this problem in 25 children with GER who had esophageal manometry performed in conjunction with an antireflux operation. Intraoperative lower esophageal high pressure zone (LEHPZ) pressure and length were recorded for a simulated gastrostomy in all patients prior to performing the antireflux operation. These same parameters were then recorded for a simulated (11 patients) or real gastrostomy (14 patients) following the antireflux procedure. The LEHPZ pressure decreased with simulated Stamm gastrostomy (7.8 +/- 1.1----6.6 +/- 1.1 mm Hg, NS: Normal = 11.2 +/- 0.9 mm Hg). This decrease was less significant than the decrease in LEHPZ length (1.1 +/- 0.1----0.8 +/- 0.1 cm, P less than .01: Normal = 1.3 +/- 0.1 cm). Following Boerema gastropexy, simulated gastrostomy produced a similar decrease in LEHPZ pressure (20.8 +/- 3.8----17.1 +/- 2.7 mm Hg, NS) and length (3.3 +/- 0.4----2.5 +/- 0.3 cm, P less than .025). The LEHPZ pressure and length were not decreased by real gastrostomy performed with modified Thal fundoplication or with Nissen fundoplication. Thus, a decrease in LEHPZ length may be one mechanism whereby GER is initiated or worsened by tube gastrostomy in children. Tube gastrostomy has a similar effect when performed with a Boerema gastropexy, but not when performed with a Nissen or modified Thal fundoplication.

Symptomatic infants displayed three patterns of gastroesophageal reflux after drinking apple juice (20 ml/kg or 300 ml/m2 of body surface area). The type I pattern occurred in patients who had continuous postcibal gastroesophageal reflux, large hiatal hernias and frequently required an antireflux operation. A functional motility disorder suggesting delayed gastric emptying appeared to be important in infants with discontinuous reflux (type II pattern). These infants had frequent gastroesophageal reflux for only 2 3/4 hours postcibally, antral-pylorospasm, increased low esophageal sphincter pressures, and a high incidence of pulmonary symptoms and non-specific watery diarrhea. The mixed (type III) pattern of gastroesophageal reflux occurred in a small number of infants and exhibited features of both type I and II patterns.

Gastroesophageal reflux (GER) can produce debilitating and even fatal disease in infants and children. GER is common in infancy, and most GER subsides with time or with postural and dietary therapy. Operation is justified only to control effectively by nonoperative methods. The clinical history and the patient's response to medical treatment remain the most important factors in our decision for or against operation. As in adults, esophagoscopy and esophageal biopsy are useful to document the presence of reflux in older children, but they seldom contribute to the decision for operation in infants. In our hands, gastric scintiscan has provided useful qualitative data on reflux-induced respiratory symptoms and quantitative data on gastric emptying. Esophageal pH monitoring is more quantitative for reflux evaluation and allows analysis and clinical correlations with reflux patterns. We have not used the reflux patterns to predict outcome or response to treatment in the individual patient. A prolonged average duration of reflux during sleep does appear to increase the probability that a patient with respiratory symptoms will respond to operative control of reflux. Our presently imprecise technique for patient selection, however, must not cause us to withhold operation from needy infants. For the infant who does not grow or who aspirates on a reflux board, or who requires prolonged hospitalization and for the preemie who aspirates at every extubation attempts or requires long-term nasojejunal feedings to prevent aspiration, we think antireflux surgery is appropriate, humane, and cost-effective. We see no excuse for persisting with ineffective management of a process that may result in stunting, chronic illness, persistent pain, esophageal scarring, or even respiratory death.

Intraoperative esophageal manometry has not been correlated with early postoperative extended esophageal pH monitoring (EEpHM) in children with gastroesophageal reflux. Twenty-seven children were studied with the following design: (1) abnormal preoperative EEpHM; (2) intraoperative measurement of lower esophageal high pressure zone (LEHPZ) pressure and length prior to and upon completion of an antireflux procedure; and (3) EEpHM seven to ten days postoperatively. Sixteen had a Nissen or modified Thal fundoplication and eleven a Boerema gastropexy. The postoperative EEpHM was normal in patients with fundoplication regardless of the increase in LEHPZ pressure (-4 to 36 mmHg) or length (0 to 2.5 cm). Four of the patients (36%) who had a gastropexy had abnormal EEpHM. The postoperative frequency of reflux was related inversely to the elevation of LEHPZ pressure (-3 to 39 mmHg), but not to the LEHPZ length (0 to 4.5 cm). Duration of reflux was independent of observed intraoperative manometric changes. In conclusion, early postoperative EEpHM in children having a gastropexy correlates with intraoperative increases in the LEHPZ pressure. There is no such correlation in children having a fundoplication procedure.

Regurgitation and aspiration of feedings is a significant problem in children with impaired oral intake fed via gastrostomy. Using extended (18-24 hour) esophageal pH monitoring to assess gastroesophageal reflux (GER), we studied prospectively 32 children (aged 2 to 16 years) referred for feeding gastrostomy. Twenty-five patients had repeat esophageal pH monitoring after surgery. Prior to surgery, GER was documented in 23 (72%) of the 32 children. Twenty-two of the 23 children with GER before surgery had an antireflux operation performed in conjunction with the feeding gastrostomy. Gastroesophageal reflux was clinically significant in the single failed antireflux operation and in the child with GER before surgery who only had a gastrostomy performed. All nine patients without GER only had gastrostomy performed. Six of these developed GER by pH monitoring after surgery, with significant vomiting in four. Of our 11 patients remaining at risk for GER after surgery, seven (64%) had persistent vomiting with gastrostomy feedings. Thus, 91% (29 of 32) of the children were potentially at risk for GER if a gastrostomy only was performed. We believe these data support the need for a "protective" antireflux operation in children referred for feeding gastrostomy.

The association of imperforate anus with bony sacral abnormalities and neurologic deficits is well recognized. These neurologic deficits have been considered static rather than progressive. However, recent experience indicates that some patients may develop progressive neurologic problems due to spinal cord lesions that are amenable to neurosurgical correction. To investigate the frequency of such lesions, routine myelography of imperforate anus patients with sacral anomalies was undertaken. The extraordinarily high incidence of unsuspected lesions known to cause progressive bowel, bladder, and musculoskeletal dysfunction is the basis of this interim report. Thirty percent of patients with anorectal abnormalities had sacral dysplasia. Of the nine patients undergoing myelography to date, eight have been abnormal. Six children had a tethered spinal cord, one had narrowing of the bony spinal canal and dural sac stenosis, and one an anterior meningocele. Spinal cord conditions that may cause deterioration of bowel, bladder, and extremity function should be defined and corrected before irreversible damage occurs. Because of the high incidence of spinal cord lesions detected in these patients with coexisting anorectal and sacral anomalies, routine screening for spinal cord lesions is recommended.

We studied gastroesophageal reflux (GER) in 25 children between 3 and 83 mo post-repair of esophageal atresia and distal tracheoesophageal fistula (EATEF). The incidence of GER was determined by 18-24 hr pH monitoring of the distal esophagus and gastroesophageal scintiscan following the ingestion of 99mTc sulfur colloid in apple juice. Gastric emptying was also assessed in 20 children. Only 17 of 25 (68%) children had significant GER by esophageal pH monitoring, and 13 of 20 (65%) had significant GER by gastroesophageal scintiscan. Significant GER was found in 10 of 12 (83%) patients wih recurrent vomiting, respiratory symptoms or severe esophagitis. Three of these 10 patients required an operation to control GER. Significant GER occurred in continuous, discontinuous and mixed patterns. The discontinuous pattern was seen in 11 of 17 (65%) children, and was associated with slow gastric emptying. The only factor during the repair of EATEF that subsequently was associated with a higher incidence of significant GER (88% vs. 59%) and slow gastric emptying (11.2 +/- 4.2% vs. 25.9 +/- 3.7% gastric emptying at 30 min, p less than 0.05) was excessive tension at the esophageal anastomosis. Many children with EATEF do not have significant GER, but in those with significant GER slow gastric emptying seems to be important.

We reviewed our seven-year experience in 63 children with an operation to control gastroesophageal reflux and respiratory symptoms. The age at operation, sex, major associated disorders, and control of vomiting in this group of children were compared with another group of 72 children without respiratory symptoms who also had an antireflux operation during the same period. Associated central nervous system, pharyngeal, or esophageal disorders were common in both groups. Vomiting was controlled in 96% of patients. Fifty-six of 61 (92%) children had at least partial relief of respiratory symptoms postoperatively. The complete relief of these symptoms was more likely in patients without major associated disorders (97% vs 59% P = 0.0009). Central nervous system disorders were present in most children with incomplete resolution of respiratory symptoms. It appears that a significant number of affected infants and children may have respiratory difficulties unrelated to the presence of GER.

Following antireflux surgery, children with persistent retching symptoms are presumably more likely to have delayed gastric emptying. We report 66 children between 2 weeks and 16 years of age who had an operation to control gastroesophageal reflux (GER). All patients had GER confirmed by 18- to 24-hour esophageal pH monitoring. Preoperative gastric emptying studies were performed in each patient with 99m-Tc sulfur colloid in apple juice. In addition to the percent gastric emptying (%GE), an effective gastric emptying was estimated by correcting the %GE for postcibal reflux (corrected %GE). Repeat 18- to 24-hour esophageal pH monitoring was performed postoperatively in all patients, and a repeat gastric emptying study was performed in 32 patients. After an average postoperative follow up of 6 months (range of 1 to 18 months), persistent retching was present in 12 (18%) patients. The retching was associated with dumping symptoms in six patients. Retching was seen in patients with a preoperative increase in effective gastric emptying (10/34, 29%, P less than .05) or a decrease in effective gastric emptying (2/15, 13%, NS), and not in patients with an effective gastric emptying within the control range (0/17, 0%). Postoperatively, retching with dumping symptoms was associated with an increased effective gastric emptying, and retching without dumping symptoms with a decreased effective gastric emptying. In conclusion, persistent retching followed anti-reflux surgery in children is related to extremes in effective gastric emptying. The preoperative measurement of corrected %GE identifies children at increased risk for this postoperative problem.