HIRSCHSPRUNG'S DISEASE / AGANGLIONOSIS / NEONATAL INTESTINAL OBSTRUCTION

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.

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Anorectal Anamoly / Anorectal Malformation / Imperforate Anus

Imperforate is commonly used term when a child is born without Anal opening at normal Anal site. This is one of the common major congenital abnormalities producing intestinal obstruction in neonate. Some of them communicate with urinary tract which needs to be tackled at the time of correction. If not diagnosed in time and managed properly, may end up with long term irreparable damage including death at times. It appears that it is difficult to miss this condition as the problem is obvious. However even today diagnosis is missed /delayed once in every 4 case which increases suffering and longterm poor control of sphincters, that can easily be avoided by simple observation.

It is very appropriate to note that lifetime misery can be avoided by the first doctor to diagnose the issue and the first Surgeon to manage the problem appropriately.

Usually there are three types in relation to sphincters: Low: can be operated from below in a single stage, Intermediate and High- Single/ multiple stages. 

Most of these cases are handled in single stage at Praanadah Speciality Hospital. On the contrary a poor assessment can result in multiple stages even in low type of Imperforate anus. Main issue is preserving sphincters which is important for continence of stools which is disturbed by inadequate assessment of the problem and multiple stages when not required.

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Services Offered at Praanadah Hospital

Praanadah Hospital offers varied services:

Neonatal Surgery

Neonatology & Pediatrics

Pediatric Thorasic surgery

Pediatric Urology and Neurosurgery

Pediatric Plastic Surgery

Pediatric Surgery

Pediatric Gastro-Intestinal Surgery

Pediatric Oncology

   Pediatric General Surgery

   Minimal Inclusion

Neonatal Surgeon

Pediatric Surgery

Laparoscopic Surgery

Colorectal Surgery

    

Surgery And Allied Specialties

Pediatric General Surgery

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Why Praanadah Hospital is Different ?

Praanadah Hospital is designed to take up and handle problems in women, obstetric care, health problems in all age groups with a personalized touch. Surgical problems in newborns and children at this hospital are handled with success by the senior most consultants, which is of higher specialty and state of art one.

The hospital advisory committee has consultants in pediatrics, obstetrics & gynecology, surgery, medicine and exclusive higher specialty in pediatric surgery, who have an extensive experience and qualifications from premier institutions of the country.

Praanadah Specialty Hospital was started with a holistic vision to child health as a unique mother and child hospital with children surgical center apart from other surgical and medical specialty services.

Praanadah hospital with special 36-bed provision was designed exclusive for neonatal and pediatric surgery care, that starts with the mother to preserve, sustain and empower life to attain its full bloom.

Praanadah hospital with eminent specialists and with their extensive experience, aesthetic approach and minimally invasive and innovative approach ensure timely and successful medicare. The specialized and unique approach adopted at Praanadah, as single stage correction for hirschsprung's disease, anorectal anomalies, and other complex problems in most of the cases motivated the people all over the country to utilize the services of Praanadah. Challenging, high-risk surgeries and revisional surgeries are undertaken with commitment and expertise that proved successful. 

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