ABSTRACT
Background: Hernia of the umbilical cord is a rare clinical entity which presents with hernia of the small bowel into the proximal part of the umbilical cord. This is sporadically associated with other congenital malformations. This is usually poorly identified and mistakenly termed as 'omphalocele minor. Inadvertent clamping of the cord in these cases leads to iatrogenic bowel wall injury. The aim of this study is to present a spectrum of cases presenting with umbilical cord hernia in a tertiary care Govt medical college, along with demography, intra operative findings, associated malformations and postoperative outcome and 1 year follow up.Methods: This is an ambispective study on neonates who attended the Outpatient Department (OPD) or Emergency department. The babies were evaluated by age, birth weight, gender, any gross clinical malformation. They were also evaluated intraoperatively for any associated anomalies. Post-surgery the babies were followed up until 2 years of age in pediatric surgery OPD of the same institute and outcome was recorded.Results: Out of 90 babies, 88 babies (97.77%) had body weight more than 2.5 kg, and rest 2(2.22%) had bodyweight of 1.5kg and 1.8 kg. Regarding content of contents of umbilical cord hernia, out of 87 patients, 43 patients (49.42%) had ileal loop as, 32 patients (36.78%) had ileum with Meckel's diverticulum, 6 patients (6.39%) had cecum with appendix.Conclusions: Most of this study cases had ileal loops as content of the hernial sacs, and 36.78% cases had Meckel's diverticulum associated, which is a remnant of Vitello intestinal duct. Prompt identification of the condition and early intervention and adequate post-natal care are affective to prevent long term morbidity.
ABSTRACT
Background: Hirschsprung's Disease (HSCR) is the commonest congenital gut motility disorder and is characterized by the absence of ganglion cells in a variable length of the distal gut. According to literature , HRSCD may be associated with a chromosomal abnormality or other congenital anomalies in approximately 20% of cases HSCR appeared to be a multifactorial malformation with low, sex dependent penetrance and variable expression according to the length of the a ganglionic segment, suggesting the involvement of one or more gene (s) with low penetrance. So far, eight genes have been found to be involved in HSCR. This frequent congenital malformation now stands as a model for genetic disorders with complex patterns of inheritance. The objective of this study was to collect and study personal and family history and any information regarding known associated anomalies in patients diagnosed as Hirschsprung's disease.Methods: Here author present a series of 89 consecutive HSCR patients who were admitted or attended the Pediatric Surgery OPD of in a tertiary care Government medical college, who were evaluated for associated malformations with the help of available non-invasive investigation.Results: Congenital heart diseases and Down's syndrome were the two most common associated anomalies. Genitourinary anomalies, Limb anomalies and central nervous system anomalies were other associated anomalies. Conclusions: This study confirmed the underestimation of certain associated anomalies in Hirschsprung patients, such as CNS anomalies. Based on the results of this study, in addition to meticulous general examination author suggest performing cardiologic assessment in patients diagnosed with Hirschsprung's disease based on clinical features and according to the standards of care adopted for the general population.