PAPER. Esophageal ph Monitoring Abnormalities and Gastroesophageal Reflux Disease in Infants With Intestinal Malrotation

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1 PAPER Esophageal ph Monitoring Abnormalities and Gastroesophageal Reflux Disease in Infants With Intestinal Malrotation Stephen G. Jolley, MD; Mary L. Lorenz, RN, BSN; Margo Hendrickson, MD; J. Parker Kurlinski, MD Hypothesis: Infants with rotational abnormalities of the midgut mesentery are at high risk for gastroesophageal reflux disease () and for sudden infant death (SID) from. Design: A survey of the prevalence of and the risk factor for SID from in a case series of infants treated for congenital anomalies that include intestinal malrotation. Eighty-one (89%) of the infants studied for 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, 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 (abnormal ph score 2 hours postcibal) and the risk factor for SID from (type I or III reflux pattern in combination with a prolonged mean duration of sleep reflux). Main Outcome Measures: The prevalence of and the risk factor for SID from. The follow-up of was reported as a combination of clinical outcome and subsequent extended esophageal ph monitoring. Results: Of the 91 infants studied, 80 (88%) had and 26 (29%) had the risk factor for SID from. Of 55 infants with intestinal malrotation alone, 52 (95%) had, and 20 (36%) had the risk factor for SID from. Although was found in 19 (83%) of 23 patients with repaired abdominal wall defects, the prevalence of the risk factor for SID from was significantly lower (13% [3 patients]; P =.03) than in patients with intestinal malrotation alone. The prevalence of 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 (23% [3/13]; P =.19). Both symptomatic and asymptomatic patients had similar prevalences of (91% [52/57] vs 82% [28/34]; P =.17) and for the risk factor for SID from (31% [18/57] vs 24% [8/34]; P =.28). On followup, the prognosis for 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 in infants with intestinal malrotation is high, and the prevalence of the risk factor for SID from is a significant concern. The prevalence of 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. We recommend careful evaluation and follow-up of infants with intestinal malrotation for problems, such as SID, from. Arch Surg. 1999;134: From the Departments of Surgery (Drs Jolley and Hendrickson and Ms Lorenz) and Pediatrics (Dr Kurlinski), University of Nevada School of Medicine, Sunrise Children s Hospital, and the University Medical Center of Southern Nevada, Las Vegas. ROTATIONAL abnormalities of the midgut mesentery are a ubiquitous congenital anomaly. Intestinal malrotation is found either alone or in combination with other congenital anomalies such as abdominal wall defects or congenital diaphragmatic hernia. The lack of normal mesenteric fixation leads most often to acute or chronic symptoms from intermittent intestinal obstruction as a consequence of midgut volvulus or internal herniation. These symptoms referable to obstruction are usually relieved successfully by the Ladd operative procedure with or without suture fixation of the midgut mesentery. The Ladd operation appears to be less commonly needed in infants with abdominal wall defects or congenital diaphragmatic her- 747

2 PATIENTS AND METHODS PATIENT POPULATION From September 1, 1985, to May 31, 1998, a total of 286 consecutive infants with rotational abnormalities of the midgut mesentery were treated at Sunrise Children s Hospital and the University Medical Center of Southern Nevada, Las Vegas. Ninety-one of these infants were selected for this report because they had no prior operation on the stomach or esophagus and underwent extended esophageal ph monitoring. Informed written consent for the extended esophageal ph studies was obtained from the patients parents or legal guardian. Of the patients studied, 55 had intestinal malrotation without an abdominal wall defect or congenital diaphragmatic hernia, 23 had a repaired abdominal wall defect (omphalocele [n = 5] and gastroschisis [n = 18]), and 13 were survivors of a repaired congenital Bochdalek diaphragmatic hernia. Fifty-nine infants (65%) had additional conditions, shown in Table 1. The ages at the time of initial extended esophageal ph monitoring ranged from 2 weeks to 9 months (mean, 2.3 months; median, 2 months). There were 59 boys and 32 girls. Fifty-seven patients (63%) had symptoms suggestive of. These included repeated nonbilious emesis (n = 36), growth retardation (n = 15), recurrent respiratory tract symptoms (n = 25), and irritability (n = 2). Eighteen infants had more than 1 symptom. Thirtyfour infants had no symptoms to suggest and were studied because of a perceived increased risk for SID from. 2 Of the 42 symptomatic infants studied with intestinal malrotation alone, 19 were found to have unsuspected and untreated intestinal malrotation at the time of an antireflux operation after had been identified by extended esophageal ph monitoring. Although all symptomatic infants had a barium esophagram and upper gastrointestinal tract series before esophageal ph monitoring, intestinal malrotation was not detected in the 19 infants with intestinal malrotation found at the time of an antireflux operation. In the remaining 23 symptomatic infants, the Ladd operation without intestinal suture fixation was performed before extended esophageal ph monitoring. EXTENDED ESOPHAGEAL ph MONITORING Intraesophageal ph monitoring was performed in the distal esophagus for 18 to 24 hours and was evaluated according to a method reported previously. 5 The esophageal ph score more than 2 hours after a meal was used to determine the presence or absence of. A score greater than 64 was thought to be abnormal and indicative of. In patients with, the type I, II, or III pattern of reflux was also determined from the esophageal ph recording. These reflux patterns occur following feedings of apple juice (300 ml/m 2 of body surface area) and appear as continuous reflux episodes (type I), discontinuous episodes of reflux (type II), or a mixture of continuous and discontinuous occurrences of reflux episodes (type III). 6 The mean duration of acid reflux episodes during sleep more than 2 hours after a feeding (ZMD) was determined. A ZMD was either prolonged ( 3.8 minutes) or normal ( 3.8 minutes). The risk factor in the esophageal ph recording that has been associated with SID from is the coexistence of a type I or III reflux pattern and a prolonged ZMD. 9 PATIENT TREATMENT AND FOLLOW-UP All patients with documented by extended esophageal ph monitoring were offered treatment of their. Patients with symptoms were offered aggressive treatment with medical antireflux therapy (postural therapy, thickened feedings, and a combination of prokinetic and histamine 2-receptor blockade medications). An antireflux operation was offered if medical therapy for 6 weeks did not control symptoms or if symptoms were lifethreatening. In patients with a prolonged ZMD, medical therapy also included home cardiac and apnea monitoring. Infants with no symptoms of were offered postural therapy as long as they remained asymptomatic. Home cardiac and apnea monitoring was recommended in the treatment of asymptomatic infants with a prolonged ZMD. Follow-up information was available in 64 (89%) of the 72 patients in whom intestinal malrotation was diagnosed before extended esophageal ph monitoring, and in 17 (89%) of the 19 patients in whom intestinal malrotation was diagnosed at an antireflux operation (Table 2). STATISTICAL ANALYSIS The prevalence of and the risk factor for SID from were determined. Patient groups were compared using the Fisher exact test. Differences between patient groups were considered to be significant if P.05. nia. 1 Some chronic symptoms in infants following the Ladd operation for intestinal malrotation have been attributed to gastric emptying abnormalities, 2 but others have been associated with gastroesophageal reflux disease (). 3,4 Infants with intestinal malrotation have been thought to be at risk for, including sudden infant death (SID) from. 2 When performed properly, extended (18- to 24- hour) esophageal ph monitoring has become a useful tool in the identification of in children. An abnormal ph score more than 2 hours after a meal is used to identify. 5 Three patterns of types I, II, and III also can be detected with esophageal ph monitoring and correlate with the course and prognosis of treated childhood. 6,7 The mean duration of acid reflux episodes during sleep (ZMD) correlates with the presence (prolonged ZMD) or absence (normal ZMD) of refluxinduced respiratory symptoms. 8 A prolonged ZMD in combination with a type I or III reflux pattern is associated with the highest risk for SID from untreated, even in asymptomatic infants. 9 In the present study, we describe a large group of infants with intestinal malrotation who were studied with extended esophageal ph monitoring in an attempt to determine the prevalence of and the risk for SID from. RESULTS Of the 91 infants studied with extended esophageal ph monitoring, 80 (88%) had and 26 (29%) had the risk factor for SID from. The prevalence of 748

3 Table 1. Ages and Associated Conditions in 59 Infants With Intestinal Malrotation Studied for Gastroesophageal Reflux Disease With Extended Esophageal ph Monitoring* Associated Condition No. of Infants With Intestinal Malrotation Alone With Repaired Abdominal Wall Defect With Repaired Congenital Diaphragmatic Hernia and the risk factor for SID from were not higher in patients with additional conditions (88% [52/59] and 29% [17/59], respectively) than in those without additional conditions (88% [28/32] and 28% [9/32], respectively). Infants with symptoms were not more likely than asymptomatic infants to have a significantly higher prevalence of (91% [52/57] vs 82% [28/34]; P =.17) or the risk factor for SID from (31% [18/57] vs 24% [8/34]; P =.28). INTESTINAL MALROTATION WITHOUT ABDOMINAL WALL OR DIAPHRAGMATIC DEFECT Total Age, range, mo Mean (median) 2.6 (2) 2.2 (2) 1.1 (1) 2.3 (2) Associated conditions CNS disease Congenital heart disease Prematurity Jejunoileal atresia Anorectal atresia Cystic fibrosis Hiatal hernia Laryngomalacia Cleft palate Bronchopulmonary dysplasia alone DiGeorge syndrome Meconium aspiration Pulmonary sequestration 1 Condition *CNS indicates central nervous system. Table 2. Length of Follow-up in the Patient Groups With Intestinal Malrotation Follow-up, mo Group (No. of Patients) Range Mean Median Intestinal malrotation alone (48) Diagnosis before ph study (31) Diagnosis after ph study (17) Repaired abdominal wall defect (21) Repaired diaphragmatic hernia (12) All patients (81) Fifty-two (94%) of the 55 infants with isolated intestinal malrotation had. Twenty (36%) had the risk factor for SID from. The prevalence in patients with extended esophageal ph monitoring performed after intestinal malrotation was corrected was 92% (33/36) for and 33% (12/36) for the risk factor for SID from. The presence or absence of symptoms did not significantly affect the prevalence of (96% [22 of 23 patients] vs 85% [11 of 13 patients]; P =.29) or the prevalence of the risk factor for SID from (38% [8 of 23 patients] vs 31% [4 of 13 patients]; P =.55). Among the infants with and isolated intestinal malrotation treated before extended esophageal ph monitoring, the prevalence of each reflux pattern was 70% (23/ 33) for type I, 15% (5/33) for type II, and 15% (5/33) for type III. This did not differ significantly from the prevalence of reflux patterns in the 19 patients who had intestinal malrotation diagnosed at the time of antireflux operation and after had been documented by extended esophageal ph monitoring (type I, 63% [12/19]; type II, 16% [3/19]; and type III, 21% [14/19]). REPAIRED ABDOMINAL WALL DEFECTS Nineteen (83%) of the 23 infants with a repaired abdominal wall defect were found to have, and only 3 infants (13%) were found to have the risk factor for SID from. The prevalence of the risk factor for SID from was significantly lower (P =.03) in these infants than in infants with isolated intestinal malrotation. Even though the risk factor for SID from was less prevalent, the prevalence of a prolonged ZMD was 39% (9/23). For symptomatic and asymptomatic patients with repaired abdominal wall defects, the prevalences of (75% [6/8] vs 87% [13/15]; P =.90) and the risk factor for SID from (0% [0/8] vs 20% [3/15]; P =.25) were not significantly different. In the patients with a repaired abdominal wall defect and, the prevalence of each reflux pattern was 47% (9/19) for type I, 42% (8/19) for type II, and 11% (2/19) for type III. REPAIRED CONGENITAL DIAPHRAGMATIC HERNIA The prevalence of in infants with repaired congenital diaphragmatic hernia was 69% (9/13). This prevalence was significantly lower (P =.02) than that in infants with isolated intestinal malrotation. The prevalence of the risk factor for SID from in these patients was 23% (3/13). Symptomatic and asymptomatic patients with repaired congenital diaphragmatic hernia had similar prevalences for (71% [5/7] vs 67% [4/6]; P =.78) and the risk factor for SID from (29% [2/7] vs 17% [1/6]; P =.56). In patients with repaired diaphragmatic hernia and, the prevalence of the type I reflux pattern was 89% (8/9) and of the type II reflux pattern was 11% (1/9). No patients were seen with the type III pattern of. FOLLOW-UP IN ALL PATIENTS WITH INTESTINAL MALROTATION Even though the presence or absence of symptoms did not have a significant bearing on the prevalence of or the risk factor for SID from, such was not the 749

4 ph Study (n = 4) No Antireflux Operation (n = 4) ph Study (n = 4) No Antireflux Operation (n = 4) Asymptomatic (n = 2) Died of Bronchopulmonary Dysplasia (n = 2) Type I Reflux Pattern (n = 21) Antireflux Operation (n = 11) No Antireflux Operation (n = 10) Persistent Symptoms (n = 2) Asymptomatic (n = 8) Asymptomatic (n = 4) Type I Reflux Pattern (n = 17) No Antireflux Operation (n = 17) Symptoms (n = 2) Asymptomatic (n = 15) Died of Candida Sepsis Medical Therapy ph Study, No (n = 4) ph Study, Yes (n = 4) ph Study, Yes ph Study, No ph Study, No (n = 8) ph Study, Yes (n = 7) (n = 0) (n = 4) Medical Therapy (n = 3) (n = 4) Type II Reflux Pattern (n = 6) No Antireflux Operation (n = 6) Persistent Symptoms Asymptomatic (n = 5) Type II Reflux Pattern (n = 5) No Antireflux Operation (n = 5) Symptoms (n = 0) Asymptomatic (n = 5) Medical Therapy ph Study, No (n = 3) ph Study, Yes (n = 2) ph Study, No (n = 4) ph Study, Yes Type III Reflux Pattern (n = 4) Antireflux Operation (n = 2) No Antireflux Operation (n = 2) Persistent Symptoms (n = 0) Asymptomatic (n = 2) ph Study, No (n = 2) Figure 1. Follow-up information in 35 infants with intestinal malrotation diagnosed before extended esophageal ph monitoring (ph study) and with symptoms suggestive of gastroesophageal reflux disease () at the time of initial esophageal ph monitoring. The subsequent clinical course and follow-up extended esophageal ph monitoring are presented according to the findings on initial monitoring (normal or type I, type II, or type III reflux pattern). All patients with received medical antireflux therapy until the resolved or an antireflux operation was performed. Type III Reflux Pattern (n = 3) No Antireflux Operation (n = 3) Symptoms (n = 0) Asymptomatic (n = 3) case for the subsequent clinical course in the 81 patients observed. Two of the 4 patients with symptoms suggestive of and normal results on extended esophageal ph monitoring died. Symptoms and the cause of death were directly attributable to cor pulmonale from severe bronchopulmonary dysplasia. The remaining 2 symptomatic patients with normal results of extended esophageal ph monitoring subsequently became asymptomatic without treatment of. In contrast, all 4 asymptomatic patients with normal results of extended esophageal ph monitoring remained asymptomatic without any treatment of. Thirteen (42%) of the 31 symptomatic infants observed with detected after the diagnosis of intestinal malrotation required an antireflux operation to control (7 Nissen fundoplications and 6 Thal fundoplications). None of the 25 asymptomatic infants with required an antireflux operation, however, even though in some, symptoms from subsequently developed (P.001). The subsequent course of patients with treated nonoperatively is summarized in Figure 1 for symptomatic patients and in Figure 2 for asymptomatic patients. Seventeen of the patients with treated nonoperatively had extended esophageal ph monitoring repeated after 1 year of age. The spontaneous resolution of with time was more likely in patients who were asymptomatic at the time of initial extended esophageal ph monitoring. None of the 25 patients observed with the risk factor for SID from and treated for died of or of the sudden infant death syndrome (SIDS). There were 5 unrelated deaths in infants with. Four infants died of congenital heart disease beyond the postoperative period for their antireflux operation, and 1 infant died of Candida albicans sepsis during medical treatment of both the sepsis and. COMMENT ph Study, No ph Study, Yes (n = 2) Figure 2. Follow-up information in 29 infants with intestinal malrotation diagnosed before extended esophageal ph monitoring (ph study) who had no symptoms suggestive of gastroesophageal reflux disease () at the time of initial esophageal ph monitoring. The subsequent clinical course and follow-up extended esophageal ph monitoring are presented according to the findings on initial monitoring (normal or type I, type II, or type III reflux pattern). The prevalences of and the risk factor for SID from in infants with intestinal malrotation are much higher than expected. Because has been associated with SIDS, 9 an estimate of the prevalence of and the risk factor for SID from in the general infant population can be made with certain assumptions. The risk of sudden death from was 9.1% in untreated infants with the risk factor, 9 and the risk of SIDS before the avoidance of the prone-flat position was 1.3 per 1000 live births. 10 By assuming that all SIDS deaths occur from having the risk factor for SID from, 750

5 Table 3. Course of Treated Gastroesophageal Reflux Disease () by Age 1 Year According to Reflux Pattern, Symptoms, and Intestinal Malrotation* Reflux Pattern Symptomatic Infants Without Intestinal Malrotation and With Infants With Intestinal Malrotation Symptoms (n = 35) Asymptomatic (n = 29) Type I Antireflux operation needed Fate of in nonoperated-on infants present absent Type II Antireflux operation needed Fate of in nonoperated-on infants present absent Type III Antireflux operation needed Fate of in nonoperated-on infants present 40 Unknown 50 absent 10 Unknown 50 *Data are given as percentage of patients. From Jolley. 15 Results of extended esophageal ph monitoring. then the prevalence of the risk factor for SID from in the infant population would be 1.4%. Because the prevalence of the risk factor for SID from in infants with is 37%, 11 the prevalence of in the infant population would be 3.9%. With these assumptions regarding the infant population, the prevalence of would be at least 24 times higher and the prevalence of the risk factor for SID from would be at least 25 times higher in infants with intestinal malrotation alone. The prevalence of associated with intestinal malrotation is lower in infants with repaired congenital diaphragmatic hernia. This lower prevalence of suggests that factors other than intestinal malrotation are responsible for the observed prevalence. One possible factor may be the high mortality associated with repaired congenital diaphragmatic hernia. Patients with and the risk factor for SID from may be less likely to survive congenital diaphragmatic hernia repair and thereby give the impression that survivors have a lower prevalence of. Although the prevalence of the risk factor for SID from was not significantly lower with repaired congenital diaphragmatic hernia, statistical significance might be achieved with a larger series of infants. A higher prevalence for was expected in infants with repaired congenital diaphragmatic hernia because of the deficient diaphragmatic attachments and function near the esophageal hiatus. 12,13 Infants with repaired abdominal wall defects have a lower prevalence of the risk factor for SID from. This finding also points to factors other than intestinal malrotation as a cause for the in these infants. Prematurity can be a factor in the ability to detect a prolonged ZMD. 14 It is unlikely that this was a factor because 39% (9/23) of infants with a repaired abdominal wall defect had a prolonged ZMD. The disproportionately high prevalence of the type II reflux pattern with a prolonged ZMD was responsible for the lower prevalence of the risk factor for SID from. A shortened intra-abdominal esophagus from increased intraabdominal pressure following repair of the abdominal wall defect probably does not contribute to the development of because a higher prevalence of the type I or III reflux pattern would be expected because of their association with hiatal hernia. 6 The type II reflux pattern is not associated with a hiatal hernia or a decreased lower esophageal sphincter pressure. 6 The high prevalence of in infants with intestinal malrotation suggests that these infants usually have a defective gastroesophageal junction that may result from the same conditions that lead to the interference of normal intestinal rotation and fixation during fetal development. Such simultaneous defects in the function of the foregut and anatomy of the midgut do not necessarily result in an anatomic defect of the foregut. This concept is further supported by the gastric emptying abnormalities often seen in patients with intestinal malrotation. 2 Mechanical gastric-outlet obstruction does not appear to be a factor because a high prevalence of persists despite the relief of gastric-outlet obstruction with a successful Ladd operation. The in these patients can also be seen with rapid and slow gastric emptying. 2 The factors responsible for the development of the risk for SID from are unknown, but problems with swallowing, esophageal emptying, and gastric acid secretion are likely major components. Unfortunately, the measurement of these factors was not part of this study. The course of treated infants with and intestinal malrotation depends on the presence or absence of symptoms to suggest. The course in symptomatic infants is similar to that in infants with without intestinal malrotation. Their outcome depends on the reflux pattern type (Table 3). 15 In contrast, asymptomatic patients with have a better prognosis than symptomatic patients. Although these infants have a bet- 751

6 ter prognosis, a significant number of infants with the type I and III reflux patterns continue to have on follow-up. Because Barrett esophagus in children is seen primarily in association with the type I reflux pattern, 16 infants with persistent on follow-up remain at risk for this disorder. The observed course of in symptomatic and asymptomatic patients included some treatment of the. Without any treatment of the in our infant population with intestinal malrotation, we would have expected at least 2 sudden deaths. CONCLUSIONS Gastroesophageal reflux disease and the risk factor for SID from are highly prevalent in infants with intestinal malrotation. This high prevalence of indicates that infants with intestinal malrotation require careful evaluation and follow-up for problems from. Presented at the 106th Scientific Session of the Western Surgical Association, Indianapolis, Ind, November 18, Reprints: Stephen G. Jolley, MD, 3201 S Maryland Pkwy, Suite 414, Las Vegas, NV REFERENCES 1. Rescorla FJ, Shedd FJ, Grosfeld JL, Vane DW, West KW. Anomalies of intestinal rotation in childhood: analysis of 447 cases. Surgery. 1990;108: Jolley SG, Tunell WP, Thomas S, Young J, Smith EI. The significance of gastric emptying in children with intestinal malrotation. J Pediatr Surg. 1985;20: Kumar D, Brereton RJ, Spitz L, Hall CM. Gastro-oesophageal reflux and intestinal malrotation in children. Br J Surg. 1988;75: Chung C, Bautista N, Rowe V, Krishnamoorthy M, Applebaum H. Simultaneous correction of malrotation and gastroesophageal reflux in infants. Am Surg. 1996; 62: Jolley SG, Johnson DG, Herbst JJ, Pena A, Garnier R. An assessment of gastroesophageal reflux in children by extended ph monitoring of the distal esophagus. Surgery. 1978;84: Jolley SG, Herbst JJ, Johnson DG, Book LS, Matlak ME, Condon VR. Patterns of postcibal gastroesophageal reflux in symptomatic infants. Am J Surg. 1979;138: Jolley SG, Johnson DG, Herbst JJ, Matlak ME. The significance of gastroesophageal reflux patterns in children. J Pediatr Surg. 1981;16: Halpern LM, Jolley SG, Tunell WP, Johnson DG, Sterling CE. The mean duration of gastroesophageal reflux during sleep as an indicator of respiratory symptoms from gastroesophageal reflux in children. J Pediatr Surg. 1991;26: Jolley SG, Halpern LM, Tunell WP, Johnson DG, Sterling CE. The risk of sudden infant death from gastroesophageal reflux. J Pediatr Surg. 1991;26: Wegman ME. Annual summary of vital statistics Pediatrics. 1992;90: Jolley SG. Esophagus: gastroesophageal reflux disease. In: Ziegler MM, Azizkhan RG, Gauderer MWL, Weber TR, eds. Operative Pediatric Surgery. Stamford, Conn: Appleton & Lange. In press. 12. Kieffer J, Sapin E, Berg A, Beaudoin S, Bargy F, Helardot PG. Gastroesophageal reflux after repair of congenital diaphragmatic hernia. J Pediatr Surg. 1995;30: Qi B, Soto C, Diez-Pardo JA, Tovar JA. An experimental study on the pathogenesis of gastroesophageal reflux after repair of diaphragmatic hernia. J Pediatr Surg. 1997;32: Jolley SG, Halpern CT, Sterling CE, Feldman BH. The relationship of respiratory complications from gastroesophageal reflux to prematurity in infants. J Pediatr Surg. 1990;25: Jolley SG. Gastroesophageal reflux disease as a cause for emesis in infants. Semin Pediatr Surg. 1995;4: Jolley SG. Indications for surgery in childhood Barrett s esophagus. Acta Endosc. 1993;23: DISCUSSION Thomas R. Weber, MD, St Louis, Mo: Steve Jolley and his colleagues in Las Vegas have been leaders in bringing science to the confusing and frequently controversial topic of reflux in children. Through the extensive use of ph monitoring, they have been able to identify groups of high-risk patients who benefit from early and aggressive antireflux therapy, including surgical therapy, involving a variety of scenarios. The present study extends these observations to the group of children with intestinal rotational disorders and severe reflux, and they have identified a subgroup who they feel is at great risk for SID from aspiration. The data in this study, although convincing, generate several questions. Why do so many infants and children with intestinal rotational anomalies have such severe reflux? Is it due in part to duodenal obstruction from congenital bands? Or is there abnormal duodenal or gastric peristalsis? Do you have any gastric emptying studies to explain the high incidence of reflux in these patients? Second, you elected to treat nonoperatively several infants whom you have identified as being at high risk for SID. Many pediatric surgeons would consider near SID as an absolute indication for immediate fundoplication. Aren t you somewhat nervous about treating these high-risk patients medically? This is particularly worrisome in the group of asymptomatic patients in whom you have found continued reflux by ph studies. These are precisely the children who seem to be at greatest risk for near or complete SID episodes. You obviously used ph monitoring as the gold standard for the detection of reflux in children and have great experience in its application and interpretation. Do you use other diagnostic studies in these patients? How do they correlate with your ph studies? Several patients in your series were found to have intestinal rotation abnormalities at the time of fundoplication. More liberal use of upper gastrointestinal (GI) contrast studies, for example, would probably have identified these anomalies preoperatively, thus eliminating this surprise factor intraoperatively. Jay S. Grosfeld, MD, Indianapolis, Ind: I get concerned about correlating malrotation and gastroesophageal reflux with the SID syndrome (SIDS). If you wanted to say these conditions are associated with aspiration or the threat of aspiration and death, that is one thing, but the SIDS is different. Especially since the publication of the book by Firstman and Talen, The Death of Innocence, which documents that, unfortunately, many of the infants who were considered to have SIDs were subsequently shown to be suffocated by their parents. The relationship of malrotation to SIDS leaves me cold. I would rather you consider this as a threat for aspiration than suggest that it is a cause of SIDS. Many pediatric specialists do not believe gastroesophageal reflux has anything to do with SIDS. Would you please comment on this? Dr Jolley, does the motility problem in the foregut associated with gastroesophageal reflux extend to the duodenum in patients who have intestinal malrotation? Did some of the patients who were previously operated on for malrotation before the diagnosis of gastroesophageal reflux have a midgut volvulus? Did they require bowel resection? Did they have delayed return of gastric emptying after their last procedure? Dr Jolley: I do not understand why children with malrotation have a high prevalence of reflux. We do use other modalities to study these patients. All the patients who were symptomatic had an upper GI series and an esophagram, including a small bowel follow-through. In answer to Dr Grosfeld s question, an x-ray was done to be sure they did not have an obstruction postoperatively. None had the resection of bowel, even though they have had a volvulus, because the bowel was viable and did not need to be resected. Regarding gastric emptying studies in these patients, we have published this previously and have shown that these patients can have slow gastric emptying, normal gastric empty- 752

7 ing, or rapid gastric emptying. There is no pattern of the gastric emptying to suggest that they may have persistent chronic obstruction. It is clear that they do not always have completely normal motility of their stomach. Why do we treat patients who are at risk for SID medically? This applies only to patients who are asymptomatic. The main reason for treating those patients medically is that we do not know that we should treat them surgically because the limited data that we have on those patients was coupled with the fact that their families discontinued the medical antireflux therapy that they were on. At this point in time, we do not have any evidence that asymptomatic patients at risk for SID will die despite medical antireflux therapy. The upper GI findings on these patients are typical for nonrotation postoperatively. I can tell you that of the 19 children in whom we were surprised to find intestinal malrotation, all had an upper GI series performed before the antireflux operation, and these were read as normal. Intestinal malrotation was not detected on the upper GI series by either the radiologist or surgeon. With respect to labeling these patients as SIDS victims, I agree with Dr Grosfeld in that the term SIDS has a negative connotation, primarily because it is a catch-all category, and there is no question that there are infants who have been ascribed as SIDS victims who have been the victims of trauma or suffocation at home. We also see patients who are victims of Munchausen by proxy. However, the patients who have reflux and who have died at home have been legitimate SIDS victims. They are victims who have not been the subject of these other factors. The only reason that I can stand up here and say that is that I have been doing esophageal ph monitoring in children for 20 years. We have done close to 3000 esophageal ph studies. In those 20 years and in all those studies, there has not been a single child who has had an autopsy by a pediatric pathologist (who is well trained in the SIDS autopsy) and who was called a SIDS victim who has had any findings on their esophageal ph study other than type I or type III reflux and a prolonged ZMD. This now totals 5 SIDS victims and 4 infants with death from (total of 9 infants). This is probably the most compelling evidence that reflux is a cause of SID in these patients, and it is certainly a large portion of what has been called SIDS victims, with other portions being those alluded to by Dr Grosfeld. IN OTHER AMA JOURNALS ARCHIVES OF INTERNAL MEDICINE Do Symptoms Predict Cardiac Arrhythmias and Mortality in Patients With Syncope? Jeong H. Oh, MD; Barbara H. Hanusa, PhD; Wishwa N. Kapoor, MD, MPH Background: Patients with syncope frequently present with multitude of other symptoms but their significance in predicting morbidity or mortality has not been previously studied. Objective: To determine if certain symptoms can be used to identify syncope patients at risk for cardiac arrhythmias, mortality, or recurrence of syncope. Patients and Methods: From August 1987 to February 1991, we prospectively evaluated patients with syncope from outpatient, inpatient, and emergency department services of a university medical center. These patients were interviewed, charts were reviewed, and detailed information on 19 symptoms and comorbidities was obtained. A cause of syncope was assigned using standardized diagnostic criteria. All patients were followed up at 3-month intervals for at least 1 year for recurrence of syncope and mortality. Patients in whom the cause of syncope was determined by medical history and physical examination alone were not included in our analysis. Results: History and physical examination led to the cause of syncope in 222 of 497 patients enrolled. In the remaining 275 patients, the absence of nausea and vomiting before syncope (odds ratio, 7.1) and electrocardiographic abnormalities (odds ratio, 23.5) were predictors of arrhythmic syncope. Underlying cardiac disease was the only predictor of 1-year mortality. No symptom remained as independent predictor for 1-year mortality or syncope recurrence. Conclusions: Symptoms, although important in assigning many noncardiac causes, are not useful in risk-stratifying patients whose cause of syncope cannot be identified by other history and physical examination. Triage decisions and management plans should be based on preexisting cardiac disease or electrocardiographic abnormalities, which are important predictors of arrhythmic syncope and mortality. (1999;159: ) Reprints: Wishwa N. Kapoor, MD, MPH, Montefiore University Hospital, Suite W933, 200 Lothrop St, University of Pittsburgh Medical Center, Pittsburgh, PA ( Wnk@med.pitt.edu). 753