Introduction. Cir Cir 2013;81: Abstract

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1 Cir Cir 2013;81: Description of morbidity and mortality of intestinal atresia in the neonatal period María Petra Gutiérrez-Carrillo, 1 José Manuel Zertuche-Coindreau, 1 Carmen Leticia Santana- Cárdenas, 1 Carlos Esparza-Ponce, 1 Yamid Brajim Sánchez-Rodríguez, 1 Juan Carlos Barrera-de León 2 Abstract Background: Intestinal atresia is one of the most common congenital malformations that obstruct the digestive tract, representing one third of cases of neonatal intestinal obstruction. The aim of this study was to describe the morbidity and mortality of intestinal atresia in the neonatal period. Methods: A descriptive cross-sectional study was conducted in neonate carriers of intestinal atresia seen at a referral hospital from January 2007 to August We performed a review of records selected from a database of the Pediatric Surgery Department and carried out non-probabilistic sampling of consecutive cases in addition to qualitative analyses with frequencies and percentages and quantitative medians and ranges. SPSS 20.0 statistical software was utilized. Results: One hundred thirteen patients were included, among whom there were 55 males (49%) and 58 females (51%): median age at diagnosis of intestinal atresia was 1 day (range: 1 13 days) and median age at surgery was 3 days (range: 1 41 days). The condition was found in duodenum in 47 (42%) patients, jejunum in 26 (23%), ileum in 27 (24%), and colon in 13 (11%). The majority were infants born at term weighing >2,500 g (80 patients, 71%). Type I duodenal atresia was the most frequent type of intestinal atresia found in 20 patients (18%) followed by annular pancreas in 17 patients (15%). Complicated forms include types III-b and IV (13 patients, 13%), mainly jejunum. Primary anastomosis was found in 75 infants (85%). The most common surgical complication was dehiscence 24 (21%), and sepsis care was administered to 65 patients (58%). Overall mortality was 15 patients (13%). Conclusions: The most frequent diagnosis was type I duodenal atresia and the most common surgical complications were dehiscence and medical sepsis. Key words: Intestinal atresia, newborn, congenital malformations. 1 Servicio de Cirugía Pediátrica, 2 División de Educación en Salud, Servicio de Neonatología, Unidad Médica de Alta Especialidad, Hospital de Pediatría, Centro Médico Nacional de Occidente, Instituto Mexicano del Seguro Social, Guadalajara, Jalisco, México Correspondence: Juan Carlos Barrera de León Av. Mariano Otero Zapopan, Jalisco, México Tel: (+52) /Fax: (+52) jcbarrer@hotmail.com Received: Accepted: Introduction Intestinal atresia (IA) describes a defect in a segment of the intestine that breaks the continuity of the intestinal lumen tube during development. 1 IA in newborns is found in nearly one third of the cases of neonatal intestinal obstruction. 2,3 The incidence of IA ranges from one case/ live births and even 2.8/10,000 NB. This means that in 50% of the cases the atresia occurs in the duodenum, in 36% they occur in the jejunum-ileal segment, and in 7% in the colon; in only 5% of the cases the atresia has multiple manifestations. 4-6 The mortality rate has declined significantly in recent years from 36.5% in 1969 to 10% in subsequent reviews. 6 Historically, Gray and Skandalakis 7 classified duodenal lesions by identifying three types of injuries: a) Type I represents a membrane or band with stenosis or incomplete obstruction, b) Type II is characterized by the complete destruction of a segment of the duodenum with proximal and distal portions connected by a fibrous cord. c) Type III demonstrates a complete separation of the distal ends with distal collapsed duodenum. The classification of jejuno-ileal atresia initially proposed by Louw catalogues IA into the following: a) Type I atresia: represented by a membrane or diaphragm, wherein the proximal or distal segments of the intestine are in continuity and the mesentery is intact. b) Type II atresia: represented by cecum sacs, joined by a band in which the two fundus of the cecum sacs are attached by a fibrous band, and c) Type III atresia: composed of cecum sacs that are completely separated. 8,9 This classification is modified by Grosfeld et al. 10 who added subtypes to the type III malformation, and in multiple atresia subdivided into the fo- Volume 81, No. 6, November-December

2 Gutiérrez-Carrillo MP et al. llowing: 1) Type IIIa characterized by separate segments and defect in the mesentery in a V form, 2) Type IIIb that resembles the form of apple skin, and 3) Type IV that involves multiple atresias. 5,10 Technological advances in high-resolution ultrasound equipment have allowed better definition of fetal anatomy and identification of birth defects 11 including prenatal stages and suspected duodenal atresia when identifying maternal polyhydramnios, which coexists in 20 35% of cases 4 with distension of the stomach and duodenum due to amniotic fluid. Rarely is ultrasonography able to determine lower bowel obstructions; therefore, exclusion of malformation at this level with this study does not rule out its existence. 12 After birth, jejunum-ileal and colonic atresia is characterized radiographically by proximal dilated intestinal asas associated with fluid levels. Symptoms of patients with any form of IA or stenosis are consistent with intestinal obstruction including bilious vomiting and abdominal distension at birth, which usually indicates cystic meconium peritonitis with failure to pass meconium distally. Patients with duodenal obstruction have minor abdominal distension, and this is evident h after birth in distal atresias. 11 Neonatal mortality due to IA depends on several factors including weight, age at the time of surgical intervention, anatomic site of atresia, 13 and associated anomalies such as prematurity, short intestine, and postoperative obstruction due to adhesions or volvulus. Romero and Beltran 8 from the Hospital Infantil de Mexico reported that mortality in the first year of life was associated with intestinal obstruction among the complications of this type of surgery along with medical grade sepsis and cholestasis, the latter related to progressive liver damage in patients with short intestine secondary to multiple atresia. The objective of the study was to describe the morbidity and mortality of infants with IA treated at a referral hospital over a 5-year period. Subjects and Methods This cross-sectional descriptive study was conducted from January 1, 2007 to August 31, 2012 in the Division of Pediatric Surgery, Medical Unit of High Specialty Hospital of Pediatrics, West CMN, IMSS Guadalajara, Jalisco, Mexico. Inclusion criteria were newborns who are beneficiary children of the IMSS, diagnosed with IA and who underwent surgery at the hospital. We excluded patients >28 days old, those coming from another unit where they underwent surgery, those with trisomies 13 and 18, or those with complex congenital heart disease. Study Development To enlist patients diagnosed with IA, the service database was reviewed. A data collection spreadsheet was developed and, subsequently, details of the physical and electronic records of each patient were gathered. For analysis, the information was placed in an electronic database. The relevant variables were type of atresia (a: duodenal type I, II and III; b: jejuno-ileal I, II, III-A, III-B and IV; and c: colon I, II, III-A, III-B, and IV). Medical morbidity included pneumonia, sepsis and cholestasis; surgical morbidity included wound infection, dehiscence, anastomotic leakage, anastomotic stenosis, short intestine syndrome, and intestinal occlusion, and mortality. Ethics According to the General Law of Health, the study is considered safe so no information merited parental informed consent. The study met the international research principles established by the Declaration of Helsinki. The protocol was approved by the Local Research and Ethics of Health Committee (folio ). Sample Size Sample size was not calculated because the study included all infants with IA in the study period. We had non-probability sampling of consecutive cases. Statistical Analysis Descriptive statistics were performed by calculating frequencies and percentages for qualitative variables, medians and ranges for quantitative variables. Data collected from the records were analyzed using the statistical program SPSS v for Windows. Results One hundred thirty seven patients with a diagnosis of IA were included in the study. From these, 24 infants were excluded: 21 due to incomplete records and three patients for clinical instability that prevented surgery (these three patients died). Of the remaining 113 children, 65 had some type of complication, and 15 of these children died (13%). These data illustrate the general profile of the study (Figure 1). Table 1 shows the sociodemographic and clinical cha- 462 Cirugía y Cirujanos

3 Intestinal atresia in newborns Excluidos n= 24 Expedientes incompletos n= 21 No manejo quirúrgico n= 3 Diagnóstico de Atresia intestinal n= 137 Complicaciones n= 65 Incluidos n= 113 Fallecidos n= 15 Figure 1. General profile of morbidity and mortality of neonates with intestinal atresia in the Neonatology Division. racteristics of the patients in the study. Most of the study population were full-term infants, n = 84 (63%), weighing >2500 g, (median 2795 g). Similarity was found in the percentages of gender (49 vs. 51%) including a patient with ambiguous genitalia, but ultimately was diagnosed by karyotype as a female. The most affected anatomic portion was the duodenum in 42%, and the least affected was the colon in 11%. In 67% of the patients, diagnosis was established by an obstetric prenatal sonogram. In most cases, a double bubble or polyhydramnios image was found. The most relevant clinical data at diagnosis were vomiting, abdominal distension and no bowel movements. Age (in days) at the time of diagnosis ranged from 1 13 days and from surgical intervention 1 41 days. The requirement for parenteral nutritional support was days and the start of oral nutrition was 0 to 64 days. The maximum length of hospital stay was 232 days. Table 2 describes the diagnoses according to surgical findings in neonates undergoing surgery for initial diagnosis of IA. The most common type was duodenal atresia type I in 18% of children followed by annular pancreas in 15%. The most common associated malformations were mainly represented by Down syndrome in 17 children followed by malrotation in nine children. Other associated malformations were not specified and are shown in Table 3: Meckel s diverticulum, hydrocephalus, cryptorchidism, clubfoot and esophageal atresia. Table 4 shows the documented surgeries performed: primary anastomosis in 85% of the cases including end-to-end anastomosis, diamond-shaped anastomosis and other forms. In 33% of surgical events, intestinal bypass was performed Table 1. Sociodemographic characteristics of neonates with IA surgically treated in the Division of Pediatric Surgery Weight (g), median (range) 2795 ( ) Gestational age at birth, weeks, median (range) 38 (29 40) Prematurity, n (%) 29 (37) Male, n (%)/Female, n (%) 55 (49) / 58 (51) Affected segment: Duodenum, n (%) 47 (42) Jejunum, n (%) 26 (23) Ileum, n (%) 27 (24) Colon, n (%) 13 (11) Prenatal diagnosis, n (%) 75 (67) Clinical picture at admission Vomiting, n (%) 87 (77) Abdominal distention, n (%) 50 (44) Absence of evacuations, n (%) 41 (36) Age at admission, days, median (range) 1 (1 13) Age at time of surgery, days, median (range) 3 (1 41) Days of parenteral nutrition, median (range) 27 (0-225) Start of oral feeding, median (range) 17 (0-64) Hospital stay, median (range) 37 (6-232) Volume 81, No. 6, November-December

4 Gutiérrez-Carrillo MP et al. Table 2. Diagnosis of neonates with IA according to type of atresia and localization postoperatively with reoperation in 24% of all referrals. Nine patients had intestinal obstruction as a complication of the initial surgery, which was the reason for reoperation. Finally, complications in infants during surgery are shown in Table 5. The most common surgical complication was wound dehiscence, followed by surgical wound infection, leakage and stenosis. The most common medical complication was neonatal sepsis followed by cholestasis in 21% of the children. Such complications are related to multiple factors such as infection, parenteral nutrition and prolonged fasting. Pneumonia and short intestine syndrome often followed as medical complications. The same table included the 15 deaths, corresponding to 13% of the study population. Direct causes of death were systemic infectious processes referred to as sepsis, cardiogenic shock, pneumonia and pneumothorax. Discussion Duodenum I, n (%) 20 (18) Duodenum II, n (%) 7 (6) Duodenum III, n (%) 2 (2) Annular pancreas, n (%) 17 (15) Portal vein, n (%) 1 (1) Jejunum I, n (%) 6 (5) Jejunum II, n (%) 1 (1) Jejunum III-A, n (%) 6 (5) Jejunum III-B, n (%) 3 (3) Jejunum IV, n (%) 10 (9) Ileum I, n (%) 7 (6) Ileum II, n (%) 5 (4) Ileum III-A, n (%) 13 (11) Ileum IV, n (%) 2 (2) Colon I, n (%) 2 (2) Colon II, n (%) 4 (3) Colon III-A, n (%) 7 (6) Location and type of IA will determine the clinical course, surgical findings, and interventions required for each child with IA. These depend on postoperative complications, which may vary considerably. 6 Reports show that IA is more Table 3. Congenital malformations associated with neonates with IA treated surgically in the Neonatology Division None, n (%) 64 (57) Down syndrome, n (%) 17 (15) Malrotation, n (%) 9 (8) Gastroschisis, n (%) 7 (6) Meconial disease, n (%) 6 (5) Cardiopathies, n (%) 3 (3) High anorectal malformation, n (%) 2 (2) Others, n (%) 5 (5) Table 4. Most common surgeries in neonates with IA in a referral hospital Included neonates Primary anastomosis, n (%) 75 (85) Termino-terminal anastomosis, n (%) 43 (51) Diamond-shaped anastomosis, n 28 (33) (%) Other types of anastomosis, n (%) 14 (16) Intestinal derivation, n (%) 37 (33) First surgical intervention, n (%) 28 (76) Re-intervention, n (%) 9 (24) Second intervention, n (%) 28 (25) Intestinal occlusion, n (%) 9 (32) common in premature infants and children with low birth weight; this does not correspond with those of our study in which most patients were full term and weighed >2500 g. 2 This condition can be identified initially with polyhydramnios, prematurity and low birth weight. 2 Vomiting of bile content and abdominal distension at the time of birth requires a simple abdominal x-ray of the base of the abdomen (except from duodenal involvement where there may be no distension) with which the image can be displayed of the double bubble in the duodenal atresia. Findings in jejuno-ileal and colonic atresia are distended loops and airfluid levels proximal to the obstruction. Under these conditions, children are more likely to suffer complications or even death due to a delay in diagnosis Cirugía y Cirujanos

5 Intestinal atresia in newborns Table 5. Postsurgical and medical complications of neonates with IA in the Neonatology Division Surgical complications Wound dehiscence, n (%) 24 (21) Wound infection, n (%) 22 (19) Leakage, n (%) 14 (12) Stenosis, n (%) 4 (4) Medical complications Neonatal sepsis, n (%) 65 (58) Cholestasis, n (%) 24 (21) Pneumonia, n (%) 16 (14) Short intestine syndrome, n (%) 15 (13) Death, n (%) 15 (13) Sepsis, n (%) 11 (73) Cardiogenic shock, n (%) 2 (13) Pneumothorax, n (%) 1 (7) Pneumonia, n (%) 1 (7) IA, intestinal atresia: n, number. Reynoso et al. in the General Hospital of Mexico 14 reported that 50% of cases with IA are located in the duodenum; 36% are found in the jejunum-ileal segment, 7% atresia is in the colon, and, in only 5% of the cases, is multiple atresia. In our study we found the duodenum to be affected in 42%, jejunum in 23%, ileum in 24%, and colonic atresia in 11%. In comparison with what is found in the medical literature, we found less involvement in the duodenum because of the addition of the jejuno-ileal segments in 46.8% of our cases, a higher incidence of involvement in the colon compared to that found in the literature. 2 The key to success in the treatment of infants with IA consists of early diagnosis (even prenatal) by a multidisciplinary team to provide presurgical care for the mother and the patient. After birth, diagnosis of IA should be confirmed and type of atresia should be elucidated in order to select the appropriate surgical procedure 14 to achieve intestinal continuity with as long intestine as possible so that the surface absorption is cared for. It is important when performing an anastomosis not to damage the edges and the junction of the bowel ends must be sealed and not stenosed. 13 In our study, primary anastomosis was performed in 85 cases, of which 14 had leakage and four had stenosis. Children with initial enterostomy require longer antibiotic treatment because their intestine is not in a suitable condition for primary anastomosis, as in cases of peritonitis or when there is a large size difference between the intestinal segments, with significant dilation of proximal zone preventing early peristaltic function. 6 In our study we observed a higher amount of reoperations than that reported in the literature (24% vs. 10.2%) and we experienced the same causes of reoperation as those reported in previous studies such as intestinal obstruction and anastomotic leakage. 4 Medical complications described in the literature are, mainly, sepsis and pneumonia and short intestine syndrome. 6 Causes of death are consistent with those reported in the literature. The main cause in our study was sepsis and in our studies we reported additional causes (cardiogenic shock, pneumonia and pneumothorax). Despite the advances, sepsis and cholestasis remain being some major challenges in the treatment of children with IA, especially those with short intestine syndrome requiring prolonged parenteral nutrition. Placement of intravascular catheters is essential, which are a risk factor for infection, and treatment with medications, fasting, and some components of parenteral nutrition, which leads to progressive liver failure and eventually death. 6 In conclusion, diagnosis of IA predominated in full-term infants. The most affected segment was the duodenum. The most common surgical complication was wound dehiscence along with medical sepsis. Acknowledgments We thank the personnel from the Neonatology and Pediatric Surgery Services for their help in carrying out this study. We also appreciate the facilities provided by the Archives Department for reviewing the patient clinical files. References 1. Nichol PF, Reeder A, Botham R. Humans, mice, and mechanism of intestinal atresias: a window into understanding early intestinal development. J Gastrointest Surg 2011;15: Rodríguez-García R, Rodríguez-García FC. Diagnóstico prenatal de atresia intestinal múltiple. Rev Mex Pediatr 2005;72: Jiménez JH. Atresia intestinal, experiencia del Hospital Infantil de Sonora. Rev Mex Pediatr 2005;12: Prasad TRS, Bajpai M. Intestinal atresia. Indian J Pediatr 2000;67: Martínez SN, Martínez Hernández-Magro PM, Hernández-Magro NM. Atresia congénita de colon. Una causa rara de obstrucción intestinal neonatal. Rev Mex Pediatr 2002;69: García H, Franco-Gutiérrez M, Rodríguez-Mejía EJ, González-Lara CD. Comorbilidad y letalidad en el primer año de vida en niños con atresia yeyunoileal. Rev Invest Clin 2006;58: Gray SW, Skandalakis JE. Embryology for Surgeons. Philadelphia: WB Saunders; pp Romero JRM, Beltrán BF. Atresia y estenosis del intestino delgado y colon. Bol Med Hosp Infant Mex 1968;25: Volume 81, No. 6, November-December

6 Gutiérrez-Carrillo MP et al. 9. Louw JH. Resection and end-to-end anastomosis in the management of atresia and stenosis of the small bowel. Surgery 1967; 62: Grosfeld JL, Ballantine TVN, Shoemaker R. Operative management of intestinal atresia and stenosis based on pathologic findings. J Pediatr Surg 1979;14: Touloukian RJ. Diagnosis and treatment of jejunoileal atresia. World J Surg 1993;17: Dalla Vecchia LK, Grosfeld JL, West KW, Rescorla FJ, Scherer LR, Engum SA. Intestial atresia and stenosis: a 25-year experience with 277 cases. Arch Surg 1998;133: Ramírez PG. Atresia de intestino. Contribución a la resolución de un problema. Bol Med Hosp Infant 1975;23: Reynoso AE, Camargo GFD, Rangel LMC, Guzmán AAN. Atresia intestinal múltiple: Reporte de un caso. Rev Méd Hosp Gen Méx 2008;71: Cirugía y Cirujanos