Hirschsprung Disease Myomectomy

Hirschsprung disease myomectomy

Myomectomy of Hirschsprung disease is an operation involving transanal internal sphincter myectomy or stripping away of a strip of the internal anal sphincter muscle.

It is mainly used in:

• Hirschsprung disease: ultra-short segment.
• When the aganglionic segment is so narrow (on the verge of the anal canal)
• Patients who present with chronic obstructive symptoms despite conservative treatment.
• It is not a routine practice in long-segment Hirschsprung disease, in which pull-through operations are the choice.

Indications

Myomectomy can be taken onboard in case:

• Aganglionosis of internal sphincter/very low rectum alone is confirmed by biopsy.

Child presents with:

• Severe constipation
• Difficulty passing stool
• Repeat of abdominal distension.
• Contrast enema reveals that the colon and transition zone are normal with very small transition zone.
• There is no need of standard pull-through since the diseased part is very short.

Benefits

• Avoids abdominal surgery
• Short recovery time
• Is able to relieve the obstruction symptoms.
• Applicable in cases when no full pull-through operation is required.

Myomectomy procedure Hirschsprung disease

• In Hirschsprung disease, myomectomy generally means a transanal internal sphincter myectomy, applied in the case of ultra-short segment Hirschsprung disease.
• It aims to relieve the functional obstruction by debridement of a strip of internal anal sphincter muscle, which belongs to the aganglionic zone.
• This is a procedure that does not involve surgeries on the abdominal area but is implemented by the rectum.

Myomectomy (Step-by-Step) Procedure:

Anaesthesia and Positioning

• Child is put under general anaesthesia.
• Posture: Lithotomy (frog-leg).

Under Anaesthesia Examination

• Re-examination of the anal tone (usually very tight).
• Rule out greater level of obstruction.

Access via the Anal Canal

• An incision of the mucosa is done just above the dentate line and is normally located on the posterior midline.

Internal Anal Sphincter Exposure

• The internal sphincter muscle is then exposed by lifting the mucosa gently.
• Unexpectedly, care is taken not to harm the external sphincter.

Myomectomy (Resection of Muscle Strip)

• A piece of internal sphincter muscle-strip 1-2 cm long and 0.5-1 cm wide is taken very carefully.
• This discharges the obstructive zone by the aganglionic muscle.

Hemostasis

• Small hemorrhage is curtailed using cautery or pressure.

Closure

• Fine absorbable stitches are used to suture the mucosa up.
• External incisions are not done.

Duration: Usually 20–40 minutes

Postoperative Care

• Hospital stay: 1–2 days
• The pain is not severe; it is typical to use oral drugs.
• Sitz baths recommended.
• Short-term use of stool softeners or laxatives.
• In some cases anal dilatation can be prescribed.

Surgical myomectomy for Hirschsprung disease

• Transanal internal sphincter myectomy also known as surgical myomectomy is a minor surgery done via the anal canal, which serves to treat Hirschsprung disease.
• It can only be applied in ultra-short segment Hirschsprung disease, in which case the aganglionic segment occupies only the internal anal sphincter or the very low rectum.
• This is done to debride a strip of the nonfunctioning internal sphincter muscle and thus debride the obstruction.

Diagnostic myomectomy Hirschsprung disease

• Diagnostic myomectomy is a surgery biopsy method that the surgeon uses to determine ultra-short segment Hirschsprung disease in case the normal biopsies fail.
• It entails the excision of a piece of internal anal sphincter muscle to make a sufficient piece of tissue to be used in histopathology.

It serves a dual purpose:

• Diagnostic- gives deeper muscle tissue to be able to confirm or exclude aganglionosis in internal sphincter.
• Therapeutic - in case of the presence of ultra-short segment Hirschsprung disease, the obstruction is usually relieved by the procedure.

Indications of Diagnostic Myomectomy

It is performed when:

• The symptoms are very indicative of Hirschsprung disease.
• Routine rectal biopsies are inconclusive or insufficient.
• There is a suspicion of ultra-short segment Hirschsprung disease.
• Minor constriction is only observed in anal canal in contrast enema.
• High resting pressure is found on anal manometry (where it is performed).

Child has:

• Severe constipation
• Delay in passing meconium
• Abdominal distension which is chronic.
• This will provide a complete thickness sample of muscle of the internal sphincter.

Hirschsprung myomectomy recovery

A detailed, well-organized description of Hirschsprung myomectomy recovery, in the case of transanal internal sphincter myectomy as applied in ultra-short segment Hirschsprung disease, is provided below.

Hirschsprung Myomectomy Recovery:

Hospital Stay

• Most children stay 24–48 hours.
• Vital signs, stooling pattern and distension of abdomen are followed.
• Oral feeding generally begins on the same day or the following morning.

Pain and Comfort

• The pain in recovery is mild or minimal.

Managed with:

• Oral paracetamol
• The occasional use of ibuprofen (when permitted)
• Infants are able to rapidly resume normal food and action.

Bowel Movements Postoperative

First 24–48 hours:

• Soft frequent stools are anticipated.
• Other children spend 1-2 weeks in the toilet with loose stool.

Over the next few weeks:

• Stools gradually normalize.
• There is the relief of bowel movements because the obstruction is removed.

Wound Care

• None of the external injuries, as the operation is transanally performed.

Care instructions:

• Sitz baths 5-7 days 1-2 times daily.
• Wipe the anal region clean.
• Apply barrier cream in case the loose stools irritate the skin.

Medications

Your surgeon may prescribe:

• Stool softeners (for 1–4 weeks)
• Mild oral antibiotics (selectively)
• Pain relievers for 2–3 days

Anal Dilatation

• Not always required.
• It most commonly begins 1-2 weeks following surgery on need but varies according to the surgeon.
• Rationale: avoid anal stenosis.

Feeding and Activity

• Normal diet may be resumed nearly at once.
• Breastfeeding/formula feeding remains the same.
• It takes 2-3 days before toddlers are able to resume normal activities.

Best hospital for Hirschsprung myomectomy India

• Artemis Hospital, Gurgaon
• Medanta-The Medicity, Gurgaon
• Fortis Memorial Research Institute, Gurgaon
• Max Hospital, Saket

Conclusion

Hirschsprung myomectomy, which is most oftentimes a transanal internal sphincter myomectomy, is a safe, minimally invasive yet effective, treatment of the ultra-short segment Hirschsprung disease. The procedure eliminates the obstructive physiology, and major abdominal surgery is not required since a small section of the non-functional internal sphincter muscle is removed. The recovery process is usually fast and pain-free, and stool passage is fast. Myomectomy has great long-term results and may be definitive and is an excellent choice when applied to well-selected patients with disease confined to the anal canal or very distal rectum. The follow up guarantees that the healing process is not hampered, maintenance of normal bowel activity and early detection of any residual symptoms.

Affordable Hirschsprung myomectomy India GetWellGo

GetWellGo is regarded as a leading supplier of healthcare services. We help our foreign clients choose the best treatment locations that suit their needs both financially and medically.

We offer:

• Complete transparency
• Fair costs.
• 24 hour availability.
• Medical E-visas
• Online consultation from recognized Indian experts.
• Assistance in selecting India's top hospitals for hirschsprung myomectomy treatment.
• Top paediatric surgeons who have a proven record of success
• Support during and after treatment. 
• Language Support
• Travel and Accommodation Services
• Case manager assigned to every patient to provide seamless support in and out of the hospital like appointment booking
• Local SIM Cards
• Currency Exchange
• Arranging Patient’s local food

FAQ

1. Will my child require stool softeners?

• No, stool softeners or laxatives can be advised on a temporary basis (a few weeks) to facilitate bowel motions and cushion the injured part.

2. Should anal dilatement be done after myomectomy?

• Not always. Depending on the procedure of the surgeon and the pattern of healing, some children might require dilatement to avoid the narrowing of the anal.

3. When is the bowel functioning to improve?

• The majority of the children become able to pass stools in several days. The process of improvement lasts a number of weeks, as the obstruction is eliminated.

4. Do you consider this procedure to be a substitute of pull-through surgery?

• Only in the case of ultra-short segment Hirschsprung disease when aganglionic segment is longer then pull through procedure is necessary.