Current Approaches to Diagnosis and Treatment of Hirschsprung Disease in Newborns and Infants (Literature Review and First-Hand Experience)

Abstract

The aim: To analyze the current state of the issue of Hirschsprung disease in newborns and infants on the basis of literature data and first-hand experience. Conclusions: 1. Hirschsprung disease should be suspected in all newborns with late meconium passage. 2. The main methods of diagnosing Hirschsprung disease in newborns and infants are the assessment of anamnestic data, clinical manifestations and features of the clinical course of the pathology, contrast enema, morphological examination of rectal biopsies and immunohistochemistry for ACE. 3. The presence of enterocolitis in newborns and infants should raise suspicion of Hirschsprung disease. 4. Low intestinal obstruction, perforation of the cecum, ascending or terminal small intestine, and peritonitis in the first days of a child’s life may be complications of Hirschsprung disease. 5. In newborns and infants, early diagnosis of Hirschsprung disease and timely surgical correction by one-stage surgery help improve treatment outcomes

Evaluation of Calretinin and enumeration of mast cells in rectum tissue biopsies of Hirschsprung and non-Hirschsprung disease in neonate and infant

“…Calretinin IHC was applied to all 36 studied cases, and 27 (75%) of the cases were identified as HD, while the remaining 9 (25%) cases were identified as non-HD Table (2) The IHC for Calretinin was applied to all 36 cases after the application of Calretinin IHC; out of 36 cases, 27 (75%) cases were confirmed as HD while the remaining 9 (25%) cases were confirmed as non-HD; hence, all suspicious HD cases had been confirmed and categorized in HD and non-HD. In our study, strong calretinin immunoreactivity was observed in all ganglionic segments (non-HD cases), figure (4), showing positive expression between the two muscularis layers and positive expression in the submucosa layer. Whereas any immunoreactivity was not observed in almost all aganglionic segments (HD cases), (Figure 5) shows a negative expression of Calretinin in the two layers of the muscularis layer while showing a complete absence of staining expression of Calretinin in the mucosa and submucosa layers.…”

“…In a recent study, some worrying maternal risk factors were highlighted during pregnancy were the age of the mother at conception, shown in Table (4), maternal illness, intake of drugs, type of Childbirth, and number of previous maternal abortions, all of them offer a non-significant difference (p>0.05) between the HD group and non-HD group. Still, consanguineous marriage was detected and showed a significant difference (p<0.05) between the HD group and non-HD group shown in table (5).…”

“…The Latin name for HD is megacolon congenitum 1,2 . According to reports, HD has a 4:1 male-to-female ratio and impacts 1 case out of every 5,000 live births globally 3,4 . The gold standard for diagnosing HD is a rectal biopsy 5 .…”

Investigation of The Histopathological and Histometric Changes in Rectum Tissue Biopsies of Hirschsprung and Non-Hirschsprung Disease in Neonate and Infant

“…Numerous cytoplasmic cells, an eccentric circular nucleus, a noticeable nucleolus, a perinuclear pallor, and peripheral chromatin are examples of classic cytological characteristics. Ganglion cells are typically, but not always, clustered in ganglia and/or in contact with the neuropil (neurites and glia) [33].…”

A Rare Case Report on Postoperative Rehabilitation in Hirschsprung Disease

“…HD is categorized into short-segment HD when the aganglionic segment is confined to the rectum and sigmoid colon. On the other hand, long-segment HD extends beyond the sigmoid colon and encompasses more extensive portions of the colon [ 7 ].…”