Hirschsprung's disease
ICD-10 Q43.1 is a billable code used to indicate a diagnosis of hirschsprung's disease.
Hirschsprung's disease, also known as congenital aganglionic megacolon, is a congenital condition characterized by the absence of ganglion cells in a segment of the colon, leading to a lack of peristalsis and subsequent obstruction. This condition typically presents in neonates and infants, often manifesting as failure to pass meconium within the first 48 hours of life, abdominal distension, and vomiting. The affected segment of the bowel is unable to relax, causing a functional obstruction that can lead to severe constipation and enterocolitis if not treated. Diagnosis is confirmed through rectal biopsy, which reveals the absence of ganglion cells. Surgical intervention, usually involving resection of the aganglionic segment, is the primary treatment. Early diagnosis and management are crucial to prevent complications such as bowel perforation and sepsis.
Detailed clinical notes on symptoms, diagnostic tests, and treatment plans are essential. Documentation should include the age of onset, clinical presentation, and any associated conditions.
Common scenarios include a newborn presenting with failure to pass meconium, abdominal distension, and subsequent surgical intervention.
Pediatric coders must be aware of the developmental implications of Hirschsprung's disease and the need for long-term follow-up.
Genetic counseling notes should document any family history of congenital conditions, genetic testing results, and implications for future pregnancies.
Scenarios may include genetic counseling for families with a history of Hirschsprung's disease or associated syndromes.
Genetic coders should consider the potential for chromosomal abnormalities that may co-occur with Hirschsprung's disease.
Used in the surgical treatment of Hirschsprung's disease.
Operative report detailing the procedure, extent of resection, and any complications.
Pediatric surgeons must document the specific technique used and any intraoperative findings.
Common symptoms include failure to pass meconium within the first 48 hours of life, abdominal distension, vomiting, and severe constipation. Diagnosis is confirmed through rectal biopsy.
