Hirschsprung disease is absence of ganglion cells (Nerve cells) in Colon( large intestine), it is one of the common causes of Intestinal obstruction that results in obstruction to the passage of contents (faeces) in the baby/child. Unfortunately even today this problem is inadequately managed / mismanaged inspite of so many advances in diagnostics and technology.
Baby / child develops abdominal distension (Tummy), Vomits , and if untreated succumbs, but if diagnosed and managed in time the issue is curable and the baby /child leads a normal life. Being aware of the disease and simple diagnostic means the condition can be handled successfully. This problem manifests in the newborn/neonatal period as well as in childhood which are different patterns of the same disease.
In neonatal period presentation is acute intestinal obstruction that needs immediate intervention. Constipation is the presentation in childhood.
How to Diagnose?
Full term normal delivery baby passes meconium (First stool of baby) within 24hrs after birth, otherwise it is abnormal unless proved other wise. Simplest investigation like contrast study of large intestine confirms/excludes the problem.
How to Manage?
Priority is to relieve the obstruction and save the life. Usually conservative medical management is limited to preoperative preparation. Surgical options are to correct in two/three stages where the first stage is making colostomy. Better option is single stage correction which is possible in majority of cases.
A novel transanal single stage correction without opening abdomen , a unique approach, is the preference at Praanadah specialty hospital.
1.Full term, normal delivery newborn passed meconium 48hrs after birth, became symptomatic with abdominal distension and vomiting. Normal anal opening is there. Awareness and contrast study of colon confirmed Hirschsprung's and excluded other causes of intestinal obstruction in a neonate.
What are the options?
Colostomy to relieve obstruction and save the baby.Corrective procedure; resection of aganglionic segment and restoring ganglionic colon down to rectoanal area by any of the procedures before the age of 1 yr. Primary single stage technique- a technique to be handled by an experienced surgeon Transanal endorectal primary singlestage reconstruction , done in this newborn . Post operatively , baby accepted orally within 24hrs and isfit to be sent home within 72 hrs.
2. Full term normal delivery baby did not pass meconium until 48hr after birth. Baby developed distension of tummy, became sick with suggestion of perionitis subsequent to rupture of Large Intestine. Suggestion of rare presentation of Hirschsprungs was made, the baby was operated, ruptured colon and aganglionic colon was resected, abdomino perineal pull through was done, which is unusual approach in contrast to staged procedures advocated by many in a similar situation. The baby settled in 7 days and was sent home on the 10th day.