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Home , Blind loop syndrome, Hypotonia, Meconium, Meconium peritonitis

Long-term Outcome After Neonatal Obstruction

Julie R. Fuchs, MD, and Jacob C. Langer, MD

ABSTRACT. Objective. It is unclear whether children meconium and those undergoing resection or enter- with (CF) who present with neonatal ostomy. Patients with meconium obstruction who do not meconium ileus have a different long-term outcome from have CF have an excellent long-term prognosis. This those presenting later in childhood with pulmonary com- information will be useful in counseling the families of plications or . We examined a cohort of presenting with neonatal meconium obstruction. patients with meconium ileus, and compared their long- 1998;101(4). URL: http://www.pediatrics.org/ term outcome with children who had CF without meco- cgi/content/full/101/4/e7; cystic fibrosis, meconium ileus, nium ileus and neonates who had meconium obstruction meconium plug syndrome. without CF (meconium plug syndrome). Study Design. Comparative study using retrospective and follow-up interview data. ABBREVIATION. CF, cystic fibrosis. Patients. Group 1 consisted of 35 surviving CF pa- tients who presented with meconium ileus between 1966 econium obstruction in the neonate is a and 1992. Two control groups were also studied: 35 age- spectrum of disease that includes meco- and sex-matched CF patients without meconium ileus 1 (group 2), and 12 infants presenting with meconium plug Mnium ileus and meconium plug syndrome. syndrome during the same time period (group 3). Meconium ileus is characterized by extremely viscid, Outcome Measures. Pulmonary, gastrointestinal, nu- protein-rich inspissated meconium causing terminal tritional, and functional status were reviewed, and sur- ileal obstruction, and accounts for approximately gical complications were recorded. 20% of neonatal intestinal obstructions.2 Although Results. Mean follow-up was 12.6 ؎ 6, 12.6 ؎ 6, and meconium ileus can rarely occur in otherwise normal years in groups 1, 2, and 3, respectively. Patients children,3 the majority of patients with meconium 4 ؎ 9.3 without CF (group 3) demonstrated better growth and ileus have cystic fibrosis (CF). Meconium ileus is the functional status, and had a lower incidence of pulmo- presenting symptom in 10% to 15% of these patients.4 nary and gastrointestinal problems. Although the pres- ence of meconium ileus among CF patients was associ- Meconium plug syndrome is more commonly seen ated with an earlier diagnosis, there were no significant in premature infants, is characterized by colonic ob- differences between groups 1 and 2 with respect to hepa- struction, and is only rarely associated with CF. tobiliary, nutritional, functional, or respiratory status. Approximately half of neonates with meconium Meconium ileus was associated with a higher risk of ileus present with uncomplicated intestinal obstruc- meconium ileus equivalent (20% vs 6%), although this tion. In many of these children, the obstruction can difference was not statistically significant. Long-term be relieved using a water-soluble contrast enema, surgical complications (adhesive small bowel obstruc- and surgery is reserved for those who fail nonopera- tion and blind loop syndrome) were seen in 27% of tive management. Patients presenting with compli- children with meconium ileus; there were no long-term cated meconium ileus, including , atresia, surgical complications in groups 2 or 3, because these gangrene, perforation, and meconium al- infants did not have any neonatal surgical procedures. 5 Children presenting with complicated meconium ileus ways require operative intervention. A variety of had a higher rate of long-term surgical complications procedures have been described, including enterot- than those with uncomplicated meconium ileus (36% vs omy and lavage, placement of a T-tube, resection 17%), and those managed with resection or enterostomy with primary anastomosis, and various types of en- had more complications than those treated by enterot- terostomies, including the Bishop-Koop ileostomy, omy and lavage (33% vs 0%). Mikulicz double-barreled enterostomy, Santulli Conclusions. Long-term outcome is similar in CF pa- proximal chimney enterostomy, and the double en- tients who present with meconium ileus and those who terostomy (proximal enterostomy and fistu- do not, except for a slightly higher incidence of meco- la).6 In contrast, virtually all infants with meconium nium ileus equivalent, and a significantly higher rate of surgical complications. The risk of surgical complica- plug syndrome are cured of their obstruction after a tions is highest in those presenting with complicated water-soluble contrast enema. Survival of neonates with meconium ileus has im- proved over the last few decades because of im- From the Department of Surgery, Washington University School of Medi- proved surgical technique, neonatal intensive care, cine, St Louis, Missouri. nutritional support, and medical management of the Received for publication Jun 27, 1997; accepted Jan 5, 1998. pulmonary complications of CF.7 In addition, it is Address correspondence to: Jacob C. Langer, MD, Division of Pediatric increasingly being recognized that CF is a heteroge- Surgery, St Louis Children’s Hospital, Room 5W12, One Childrens Place, St Louis, MO 63110. neous disease. The characteristics and severity of PEDIATRICS (ISSN 0031 4005). Copyright © 1998 by the American Acad- gastrointestinal and pulmonary complications vary emy of Pediatrics. considerably among patients in long-term follow-up http://www.pediatrics.org/cgi/content/full/101/4/Downloaded from www.aappublications.org/newse7 by guestPEDIATRICS on September Vol. 26, 2021 101 No. 4 April 1998 1of6 studies.8 It would be helpful for both families and nium plug syndrome, 3 had died from complications clinicians if it were possible to predict the likelihood of prematurity, 4 did not have confirmed meconium of long-term problems at the time of diagnosis. This plug syndrome, and 9 patients could not be located study was designed to determine whether neonates for long-term follow-up, leaving 12 patients for anal- with CF who present with meconium obstruction ysis in group 3. have a different long-term outcome than those who Characteristics of the three groups are shown in do not. To control for the effect of CF itself on out- Table 1. Gestational age at birth was significantly come, we also sought to determine long-term out- higher in group 2 compared to group 1. Although the come in patients presenting with neonatal meconium same trend was present for birth weight, the differ- obstruction who did not have CF. ence was not statistically significant. Children in group 1 were significantly younger when the diag- MATERIAL AND METHODS nosis of CF was made than those in group 2. In group 1, meconium ileus was uncomplicated in Charts of all newborns presenting with meconium ileus be- ϭ ϭ tween 1966 and 1992 were reviewed (group 1). Two control 60% (n 21) and complicated in 40% (n 14). The groups were also studied: CF patients without meconium ileus complicated cases included patients with proximal (group 2), and infants presenting with meconium plug syndrome volvulus (n ϭ 7), jejunal atresia (n ϭ 6), ileal atresia (group 3). Group 2 was generated by randomly matching each of (n ϭ 2), perforation (n ϭ 7), the meconium ileus patients by sex and age to a patient being ϭ ϭ followed in the CF clinic who had not presented with meconium (n 7), and ascites (n 2). Some cases had multiple ileus. Group 3 included infants with a diagnosis of meconium complications. In group 1, 33 of 35 patients had CF plug syndrome presenting between 1966 and 1992. For the pur- diagnosed by sweat chloride, and the other 2 had poses of this study, obstruction was defined as meconium ileus if meconium ileus with normal sweat chloride levels; 1 the meconium obstruction was ileal, and as meconium plug syn- drome if the obstruction was primarily colonic. of these presented with complicated meconium ileus, For each patient, the hospital and clinic chart (if available) were and the other was uncomplicated. Sweat chlorides reviewed. Information gleaned included sex, date of birth, gesta- were elevated in all group 2 patients and in none of tional age, birth weight, associated anomalies, initial presentation, the group 3 patients. and radiologic or surgical procedure. All patients or families were Of the 21 patients with uncomplicated meconium also contacted by telephone, and all families contacted agreed to participate in the study. Gastrointestinal complaints and abdom- ileus, 9 (43%) were successfully treated by contrast inal symptomatology were discussed in detail, with specific ques- enema alone, and 12 (57%) required surgical inter- tions regarding meconium ileus equivalent, recurrent , vention [intestinal resection with Mikulicz ileostomy , abdominal pain, , and gastrointestinal (n ϭ 2), intestinal resection and Bishop-Koop ileos- . Meconium ileus equivalent was strictly defined for our tomy (n ϭ 3), enterotomy with N-acetylcysteine or purposes as partial or complete intestinal obstruction with evi- ϭ dence of impacted in the right colon or terminal on saline lavage (n 4), resection with primary anasto- radiographic examination, necessitating treatment by either an mosis (n ϭ 2), and milking of meconium into the enema or intestinal lavage solution by mouth. Diarrhea, constipa- colon without enterostomy (n ϭ 1)]. There was one tion, and abdominal pain were recorded as positive if they were early postoperative complication in this group, con- severe enough to negatively impact the patient’s quality of life, or if the symptoms were recurrent over many years. sisting of an anastomotic leak requiring revision.The Nutritional status and general health were determined by as- 14 patients with complicated meconium ileus all un- sessing current height and weight, both of which were expressed derwent surgery [resection with primary anastomo- as population percentile using standard National Center for sis (n ϭ 6), resection with Mikulicz ileostomy (n ϭ 1), Health Sciences growth curves. Placement of a gastrostomy tube resection with Bishop-Koop enterostomy (n ϭ 2), and the use of nutritional supplements for poor weight gain were ϭ recorded. The severity of a patient’s pulmonary course was and enterostomy with mucus (n 5)]. There gauged by the number of pulmonary-related hospital admissions. was one early postoperative complication, consisting Existence of other medical complications and procedures related of enterostomy treated by resection and pri- to CF such as diabetes mellitus and biliary , as well as mary anastomosis. The various nonoperative and unrelated conditions and procedures were also recorded. Social and functional status was assessed with a series of questions surgical procedures were evenly distributed over relating to school or work attendance and performance, quality of time. life as described by the patient, and activity level that included All group 3 patients presented with mild abdom- exercise tolerance and general fatiguability. Patients were rated on inal distention and failure to pass meconium at 24 to a scale from 0 to 2, where 0 ϭ no limitations (similar to siblings without CF), 1 ϭ mild limitations, and 2 ϭ severe limitations. 48 hours of life. In 3 of 12 patients, thick meconium plugs were eventually passed spontaneously. In the remaining 9 patients, a contrast enema showed nu- RESULTS Characteristics of Patient Groups TABLE 1. Patient Characteristics Of the 82 patients coded with a diagnosis of meco- Group 1 Group 2 Group 3 nium ileus, 17 did not have meconium ileus, 12 had ϭ ϭ ϭ incomplete records, and 6 could not be located for (n 35) (n 35) (n 12) long-term follow-up. Of the remaining 47 patients Sex (M:F) 18:17 18:17 5:7 with well-documented follow-up, 12 had died, leav- Birth weight (kg) 3.18 3.32 2.64 Gestational age at 38.6 Ϯ 2.5* 40.0 Ϯ 1.6* 37.6 Ϯ 2.21 ing 35 surviving patients for analysis. These patients delivery (wk) were classified as group 1. Group 2 consisted of 35 Positive sweat chloride 33/35 35/35 0/12 age- and sex-matched patients chosen randomly Age at diagnosis of 7.7* 92.8* from the CF clinic population, who had not pre- CF (mo) Ϯ Ϯ Ϯ sented in the newborn period with meconium ileus. Age at follow-up (y) 12.6 6.39 12.6 6.29 9.3 4.11 Of the 28 patients coded with a diagnosis of meco- * P Ͻ .05, Student’s t test.

2of6 LONG-TERM OUTCOMEDownloaded AFTER from www.aappublications.org/news NEONATAL MECONIUM by guest OBSTRUCTION on September 26, 2021 merous filling defects in the colon with free reflux of TABLE 3. Gastrointestinal and Nutritional Status contrast into the terminal ileum, followed by spon- Group 1 Group 2 Group 3 taneous passage of one or more thick meconium (n ϭ 35) (n ϭ 35) (n ϭ 12) plugs. Hirschsprung’s disease was ruled out by rec- Liver dysfunction 2 (6%) 5 (14%) 0 tal in 4 patients. In all 12 patients, prompt Hypertrophic pyloric 2 (6%) 0 0 relief of abdominal distention occurred after the pas- stenosis sage of the meconium plugs. None of the patients in Meconium ileus 7 (20%) 2 (6%) 0 group 3 required neonatal abdominal surgery. equivalent Recurrent abdominal 21 (60%) 22 (63%) 1 (8%) pain Long-term Follow-up Constipation 5 (14%) 6 (17%) 1 (8%) Mean length of follow-up was 12.6 Ϯ 6 years in Recurrent diarrhea 18 (51%) 12 (34%) 0 group 1, 12.6 Ϯ 6 years in group 2, and 9.3 Ϯ 4 years Rectal prolapse 6 (17%) 4 (11%) 0 Rectal bleeding 5 (14%) 4 (11%) 0 in group 3. Sixty-six percent of the patients received Feeding gastrostomy 3 (9%) 1 (3%) 0 all of their care at our institution. The other patients Nutritional supplements 5 (14%) 4 (11%) 0 were initially treated elsewhere, and were subse- without gastrostomy quently cared for by us. Only 30% of the latter group received more than half of their follow-up care at another hospital. nutritional supplementation. The differences among groups with respect to meconium ileus equivalent General Medical Status did not reach statistical significance (P ϭ .15). Within General medical status is summarized in Table 2. group 1, meconium ileus equivalent was reported by There was no significant difference between groups 1 14% of those with complicated meconium ileus and and 2 with respect to height and weight. Group 3 24% of those with uncomplicated meconium ileus patients were taller and heavier, and were close to (P ϭ .80). There were no significant differences be- the 50th percentile for the general population. tween group 1 and group 2 with respect to other gastrointestinal symptoms or need for nutritional Pulmonary Status support, and these issues were rarely seen in group Mean number of admissions for pulmonary exac- 3. erbations was 4.2 Ϯ 4 in group 1 and 5.1 Ϯ 4 in group 2. Two patients in group 1 have undergone lung Social and Functional Status transplantation, and 1 additional patient is on the The mean social/functional status rating was 0.514 transplant list. None of the patients in group 2 have in group 1 and 0.417 in group 2 (P ϭ .7743, Mann undergone lung transplantation, and 1 is on the Whitney U test). In group 1, 19 patients were rated 0, transplant list. None of these differences were statis- 14 were rated 1, and 2 were rated 2. In group 2, 20 tically significant. None of the patients in group 3 patients were rated 0, 14 were rated 1, and 1 was have had pulmonary problems. rated 2. In group 3, 10 of 12 patients were rated 0 (highest functioning). A patient with Down syn- Gastrointestinal and Nutritional Status drome had mild limitation (rating of 1), and a patient Recurrent abnormalities in liver function tests and with chromosomal translocation was severely lim- hepatosplenomegaly were present in 2 group 1 pa- ited (rating of 2). tients (6%) and in 5 group 2 patients (14%). Two of these 5 patients have progressed to cirrhosis; 1 has Long-term Surgical Complications undergone liver transplantation, and 1 is on the Long-term surgical complications are summarized transplant waiting list. Liver problems were not in Table 4. There were seven complications in group present in any of the group 3 patients. 1 (long-term complication rate of 26.9%). Complica- Incidence of , meconium ileus tions occurred between 1.5 months and 18.5 years of equivalent, recurrent abdominal pain, constipation, age, and included 5 small bowel obstructions and 2 diarrhea, rectal prolapse, and rectal bleeding or me- blind loop syndromes. There were no long-term sur- lena are shown in Table 3, along with the need for gical complications in groups 2 or 3, because these

TABLE 2. General Medical Status Group 1 Group 2 Group 3 (n ϭ 35) (n ϭ 35) (n ϭ 12) Mean height percentile 30.6 Ϯ 34 30.1 Ϯ 35 42.5 Ϯ 34 Mean weight percentile 26.1 Ϯ 24 30.3 Ϯ 32 47.1 Ϯ 29 Insulin-dependent diabetes 3 (9%) 0 0 Attention deficit disorder 2 (6%) 1 (3%) 0 Allergic bronchopulmonary aspergillosis 2 (6%) 2 (6%) 0 Periorbital cellulitis 2 (6%) 0 0 Kawasaki disease 1 (3%) 0 0 1 (3%) 0 0 Arthralgias 0 1 (3%) 0 Hip synovitis 0 1 (3%) 0 Genetic anomalies 0 0 3 (25%)

Downloaded from www.aappublications.org/newshttp://www.pediatrics.org/cgi/content/full/101/4/ by guest on September 26, 2021 e7 3of6 TABLE 4. Long-term Surgical Complications in Group 1* Original Procedure Complication 1 Mikulicz enterostomy SBO requiring adhesiolysis and resection 2† Mikulicz enterostomy Multiple SBOs, one requiring adhesiolysis and resection 3 Bishop-Koop ileostomy SBO requiring adhesiolysis 4 Resection and anastomosis SBO requiring adhesiolysis and resection 5 Resection and anastomosis SBO requiring adhesiolysis 6 Double enterostomy Blind loop syndrome requiring resection 7† Mikulicz enterostomy Blind loop syndrome requiring resection Abbreviation: SBO, small . * There were no surgical complications in group 2. † Same patient. infants did not have any neonatal surgical proce- neonatal meconium obstruction was significantly af- dures. fected by the presence of CF. When analyzed according to original operation, Our data showed that the major cause of morbid- long-term complications were seen in 1 of 5 patients ity and mortality in children with meconium ileus undergoing Bishop-Koop ileostomy, 2 of 3 patients un- remained the pulmonary complications of CF. These dergoing Mikulicz enterostomy, 2 of 8 patients under- complications were unrelated to the presence or ab- going resection and primary end-to-end anastomosis, sence of meconium ileus, despite the fact that chil- and 1 of 5 patients undergoing double enterostomy. dren with meconium ileus were diagnosed at a sig- There were no complications among the 4 patients who nificantly younger age. This finding is consistent underwent enterotomy and lavage. Long-term surgical with the several other groups, who noted that early complication rate was 36% in the patients with compli- diagnosis of CF prevented serious deterioration and cated meconium ileus, and 17% in the patients with death at a young age from pulmonary disease, but uncomplicated meconium ileus. did not affect the long-term progression of lung dis- ease or survival when excluding infants presenting Mortality and dying at an age of 6 months or less.18,19 Meco- At the time of our study, 12 group 1 patients had nium ileus was related to the cause of death in only died. These patients ranged in age from 1 month to 1 of the 12 group 1 patients who died in our series, 26 years at the time of death, with a mean age of 8.14 providing further evidence that meconium ileus does years. Nine patients had uncomplicated (75%), and 3 not affect long-term prognosis in patients with CF. had complicated (25%) meconium ileus. In 2 patients, We also found that meconium ileus did not affect the cause of death was not documented. In the re- long-term functional or social status in children with maining 10 children the causes of death included: CF. Quality of life seemed to be directly related to the staphylococcal and pulmonary interstitial em- extent of pulmonary involvement, because the pa- physema in a 1-month-old, aspiration tients with the most significant limitation (missed and Pseudomonas sepsis after anastomotic leak in a days of school, time spent bedridden, severity of 6.5-month-old, bronchopneumonia and pulmonary exercise intolerance), were those with the most se- sepsis in a 7-month-old, fungal sepsis in a 1-year-old, vere pulmonary disease, particularly those listed for asphyxiation secondary to a displaced tracheostomy and awaiting lung transplantation. The lack of effect tube in a 4-year-old, respiratory failure and cor pul- of meconium ileus on height and weight percentiles monale in 3 patients aged 8, 24, and 26 years, and or the need for nutritional supplementation supports complications of lung transplantation in 2 patients the observation that the presence of meconium ileus aged 11.5 and 12 years. Therefore, only one death does not adversely affect the long-term nutritional was directly related to the presence of meconium outcome in patients with CF.11,20 ileus. There have been conflicting opinions as to whether patients with meconium ileus experience more fre- DISCUSSION quent or severe episodes of long-term gastrointesti- A number of authors have reported a decrease in nal dysfunction than CF patients without meconium morbidity and mortality for patients with meconium ileus.21–24 This issue has never been the central focus ileus in recent decades.7–11 The present study was of a study, and a group of CF patients has never been stimulated by a desire to provide more accurate in- included as a control. Although our study did not formation about long-term prognosis for families document any adverse effect of meconium ileus on with a child afflicted with CF. We were particularly most gastrointestinal problems, we did note statisti- interested in whether the earlier diagnosis that re- cally insignificant increases in hypertrophic pyloric sulted from meconium ileus would result in a better stenosis and meconium ileus equivalent. An associ- outcome because of more prompt treatment of pul- ation of meconium ileus with hypertrophic pyloric monary disease, as reported in some series,12–17 and stenosis has been noted by one group in the past,8 but whether the presence of meconium obstruction our numbers are too small to draw a definitive con- would be associated with a higher rate of gastroin- clusion. testinal complications. Finally, we wished to docu- Meconium ileus equivalent, also known as distal ment the degree to which the natural history of intestinal obstruction syndrome, is most often de-

4of6 LONG-TERM OUTCOMEDownloaded AFTER from www.aappublications.org/news NEONATAL MECONIUM by guest OBSTRUCTION on September 26, 2021 fined as partial or complete intestinal obstruction reflects a higher incidence of peritonitis and intes- occurring in CF patients beyond the neonatal period, tinal ischemia. Surgical complications occurred af- resulting from abnormally thick, viscid material in ter a wide range of procedures, although none of the terminal ileum and right colon.24 The reported the infants undergoing enterotomy and irrigation incidence of meconium ileus equivalent in CF pa- had long-term surgical morbidity. These data sug- tients ranges from 2.1% to 41.3%, depending on the gest that resection and stoma formation should be specific definition used.12,16,21,22,24–26 In uncontrolled avoided if possible,35 and that families and pedia- studies, some authors have found no increase in tricians must be counseled about the risk of long- meconium ileus equivalent in patients who pre- term morbidity after surgical intervention for sented with meconium ileus,22,24,27 and others have meconium ileus. found an increased incidence of meconium ileus equivalent.8,12,16,21 In our study, using an age- and REFERENCES sex-matched control group, there was a higher, al- 1. Olsen MM, Luck SR, Lloyd-Still J, Raffensperger JG. The spectrum of though statistically insignificant, incidence of meco- meconium disease in infancy. J Pediatr Surg. 1982;17:479–481 nium ileus equivalent in group 1. It is unclear 2. Caniano DA, Beaver BL. 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6of6 LONG-TERM OUTCOMEDownloaded AFTER from www.aappublications.org/news NEONATAL MECONIUM by guest OBSTRUCTION on September 26, 2021 Long-term Outcome After Neonatal Meconium Obstruction Julie R. Fuchs and Jacob C. Langer Pediatrics 1998;101;e7 DOI: 10.1542/peds.101.4.e7

Updated Information & including high resolution figures, can be found at: Services http://pediatrics.aappublications.org/content/101/4/e7 References This article cites 34 articles, 6 of which you can access for free at: http://pediatrics.aappublications.org/content/101/4/e7#BIBL Subspecialty Collections This article, along with others on similar topics, appears in the following collection(s): Fetus/Newborn http://www.aappublications.org/cgi/collection/fetus:newborn_infant_ sub Neonatology http://www.aappublications.org/cgi/collection/neonatology_sub Gastroenterology http://www.aappublications.org/cgi/collection/gastroenterology_sub Permissions & Licensing Information about reproducing this article in parts (figures, tables) or in its entirety can be found online at: http://www.aappublications.org/site/misc/Permissions.xhtml Reprints Information about ordering reprints can be found online: http://www.aappublications.org/site/misc/reprints.xhtml

Downloaded from www.aappublications.org/news by guest on September 26, 2021 Long-term Outcome After Neonatal Meconium Obstruction Julie R. Fuchs and Jacob C. Langer Pediatrics 1998;101;e7 DOI: 10.1542/peds.101.4.e7

The online version of this article, along with updated information and services, is located on the World Wide Web at: http://pediatrics.aappublications.org/content/101/4/e7

Pediatrics is the official journal of the American Academy of Pediatrics. A monthly publication, it has been published continuously since 1948. Pediatrics is owned, published, and trademarked by the American Academy of Pediatrics, 345 Park Avenue, Itasca, Illinois, 60143. Copyright © 1998 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 1073-0397.

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