Slepov, Migur, and Soroka: Associated Malformations and Mortality in Newborns with Congenital Small Intestine Obstruction



Problem statement and analysis of the recent research

Congenital obstruction of the small intestine is life-threatening malformation characterized by congenital violation of its patency and requires urgent surgical treatment during the first days of life. Its frequency may be different in various literary sources, ranging from 1: 7,000 to 1: 10,000 newborns in case of high small intestinal obstruction (duodenum), and up to 1: 1000 in case of low obstruction (jejunum and ileum) [1, 2]. CIO occurs equally among boys and girls. The frequency of associated malformations with CIO varies [3-5]. About 50% of patients with congenital duodenal obstruction have associated malformations, among them about 40% of patients have jejunal obstruction and 10% of patients suffer from ileal obstruction [3].

According to different literary sources, levels of postoperative mortality reported among patients with CIO are different. According to the authors, total mortality in the United States constitutes 5%, in China – 6%, in Western Europe – 10%, in India – 21%, in African countries – 35% [6-9]. Factors that may affect the mortality rate include: multiple congenital malformations, heart defects, prematurity, sepsis, pneumonia, gastroduodenal dysfunction and surgical complications as well as short bowel syndrome, anastomotic dehiscence and gastroduodenal dysfunction [6, 9, 10]. The researches aimed at mortality risk factors determination are continued at present.

The objective of research was to determine the frequency and nature of associated malformations and mortality rate in infants with CIO.

Materials and methods of the research

72 infants with CIO, namely 38 boys and 34 girls, were treated in the Department of Surgical Correction of Congenital Malformations in Children at SI “Institute of Pediatrics, Obstetrics and Gynecology of NAMS of Ukraine” from 1988 to 2015. In order to identify associated malformations the following methods were used: clinical assessment, X-ray examination, ultrasonography of the abdominal cavity and retroperitoneal space, echocardiography, neurosonography; morphological and histological (at autopsy) and statistical methods. Newborns were consulted by neurologist, pediatrician, geneticist and other specialists (when needed).

Results of the research and their discussion

Among 72 patients, high intestinal obstruction was diagnosed in 58.3% of patients (n = 42), low intestinal obstruction was observed in 41.7% of children (n = 30); jejunal obstruction was detected in 9.7% of patients (n = 7) and ileal obstruction was diagnosed in 31.9% of children (n = 23).

Associated malformations were found in 33.3% (n = 24) of all cases. Among newborns with high CIO associated malformations were diagnosed in 25.0% of cases (n = 18) and low obstruction of the small intestine was observed in 8.3% (n = 6) of patients. All cases of jejunal obstruction had isolated defects.

After examination of the patients, associated gastrointestinal malformations (GIM) were diagnosed in 16.7% of patients (n = 12) (in 13.9% (n = 10) of high CIO cases and in 2.8% (n = 2) - low CIO cases); genetic defects (GD) were detected in 9.7% of cases (n = 7) (high CIO – 6.9% (n = 5); low CIO – 2.8% (n = 2)); abdominal wall defects (AWD) were observed in 6.9% of children (n = 5) (high CIO constituted 1.4% (n = 1); low CIO comprised 5.5% (n = 4)); congenital heart defects (CHD) were diagnosed in 4.2% of patients (n = 3) (high CIO constituted 2.8% (n = 2); low CIO comprised 1.4% (n = 1)); urogenital malformations (UM) were detected in 2.8% (n = 2) (high CIO amounted 1.4% (n = 1); low CIO comprised 1.4% (n = 1)); orthopedic malformations were noted in 2.8% of cases (n = 2) (high CIO) and other developmental anomalies in children with high CIO: neurological abnormality (NA) was observed in 1.4% of children (n = 1) and diaphragm defect was found in 1.4% of patients (n = 1).

Analyzing the nosology forms, the following illnesses were determined: Down syndrome in 5.5% of cases (n = 4), gastroshisis in 5.5% of patients (n = 4), Meckel’s diverticulum in 4.2% (n = 3), cystic fibrosis in 2.4% (n = 2), esophageal atresia in 2.4% (n = 2), Hirschsprung’s disease in 2.4% (n = 2), abnormalities of upper and lower limbs in 2.4 % (n = 2), biliary atresia in 1.4% (n = 1), choledochal cyst in 1.4% (n = 1), omphalocele in 1.4% (n = 1), dextrocardia in 1.4% (n = 1), multiple ventricular septal defects in 1.4% (n = 1), coarctation of the aorta in 1.4% (n = 1), polycystic kidney disease in 1.4% (n = 1), cryptorchidism in 1.4% (n = 1), right-sided diaphragmatic hernia in 1.4% (n = 1), intestinal duplication in 1.4% (n = 1), ectopic anus in 1.4% (n = 1 ), spina bifida in 1.4% (n = 1).

Postoperative mortality rate constituted 31.9 % (n = 23) (Table 1). Among infants with congenital high CIO 13.9% (n = 10) died after surgery, and among patients with low CIO 18.1% of patients died (n = 13). Postoperative mortality constituted 20.8% (n = 15) among cases of isolated malformation and 11.1% (n = 8) among patients with associated CIO. Thus, associated CIO postoperative mortality was not significantly higher than in newborns with isolated CIO.

Table 1

Mortality in case of congenital intestinal obstruction in isolated and associated with congenital malformations forms

Form of malformation Number of patients Died
CVS CNS GIT GUS AAWD GM All
Isolated high CIO 24 - - - - - - 5
Associated high CIO 18 1 1 3 1 - 2 5
Isolated low CIO 24 - - - - - - 10
Associated low CIO 6 1 - - 1 1 1 3
Total 72 2 1 3 2 1 3 23

Note.

*GUS - Genito-urinary system

*AAWD - anterior abdominal wall defects

*GM – genetic malformations

Associated malformations were detected in 6.9% (n = 5) of patients with high CIO who died postoperatively. Among them gastrointestinal malformations were diagnosed more often, namely in 4.2% (n = 3) and genetic defects were detected in 2.8% (n = 2). Among patients with low CIO associated malformations were observed in 4.2% (n = 3) of cases, congenital heart defects (1.4%) (n = 1), urogenital malformations (1.4%) (n = 1), abdominal wall defects (1.4%) (n = 1) and genetic defects (1.4%) (n = 1) occurred with the same frequency. Morbidity in patients with associated musculoskeletal system malformations and diaphragm defects was not observed.

Conclusions

Associated malformations were observed in 33.3% of patients with congenital intestinal obstruction. Gastrointestinal malformations (16.4%), genetic defects (9.6%), abdominal wall defects (6.8%) and congenital heart defects (4.1 %) were diagnosed the most often. Associated CIO postoperative mortality was not significantly higher than in newborns with isolated CIO.

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