Rohit Shishodia
Doctors at Delhi’s Apollo Hospital have managed to successfully operate through robotic surgery upon a 31-year-old patient from Kota who suffered from a rare hereditary disease -- Multiple endocrine neoplasia type 2A (MEN2A).

It is an uncommon condition that affects approximately 1 in 35,000 individuals.The condition of the patient has improved as of now and she has been discharged.

MEN2A is an inherited condition that involves a tumor of excessive growth of at least two of the thyroid gland, parathyroid gland, and adrenal glands. It is understood that there are no specific symptoms that could pick up this disease early.

The patient presented with a history of hoarseness of voice and swelling in the neck which was developed 2 months ago. Post evaluation, it was revealed that she had developed tumour in the thyroid and another in adrenal gland (an organ that lies near the kidney and pancreas of the person in a remote place in the tummy).

A positive family history was noted as two very close relatives also had Medullary Carcinoma Thyroid. Tumor of adrenal can cause devastating fluctuations in blood pressure leading to stroke, bleeding in brain etc.

Dr Arun Prasad, senior consultant, Surgical Gastroenterology and Bariatric Surgery at the hospital, said, “In and around Kota (Rajasthan) there is a rare disease that is affecting the people. Ms Sarika (name changed) is one such unfortunate victim of this rare hereditary disease. In the past her brother and father both succumbed to this fatal disease.

“The condition known as Multiple Endocrine Neoplasia (MEN Type 2A) affects hormone producing glands of the body. Problem lies in the fact that there are no specific symptoms that could pick up this disease early. Hence public and healthcare awareness is of paramount importance. All blood relatives and people in the village need genetic and medical screening for the disease,” said Prasad.

“This kind of major surgery requires large incision with severe post-operative pain, need for ICU stay and long recovery period, but with robotic surgery patient was shifted directly to the room after surgery. Patient tolerated the procedure well, postoperatively was managed on medication with serial examination of serum calcium levels. Within 48 hours of the surgery, the patient was fit to be discharged,” explained Dr Prasad.

Dr Suresh Singh Naruka, Consultant, ENT, Indraprastha Apollo Hospitals, said, “An accurate diagnosis followed by surgery at an early stage is a useful intervention for management of MTC in MEN 2A mutation carriers. This early prophylactic surgery remains the only curative option as RET tumours are prone to metastasize early and are resistant to chemo as well as radiotherapy.”

“An early diagnosis and timely treatment are essential for their survival. Therefore, a radical approach, both from a therapeutic and surgical point of view, was undertaken,” said Dr Naruka.

“A rare disease like this requires a high grade of suspicion, advanced investigations and modern techniques like robotic surgery and nerve monitoring to save lives after early detection and early intervention. On January 14, 2021, the team conducted the very complicated surgery,” said the doctor.

“Thyroid surgery was very challenging as the right vocal cord was already damaged and the tumour was compressing major neck vessels saving the other vital organs in vicinity which were of paramount importance,” added Dr Naruka.

The doctors emphasized that adrenal surgery in such cases is very much complicated and life threatening due to high chances of rise in blood pressure while performing the surgery and serious complications. Robotic surgery was suggested to minimise the handling of gland and hence operation becomes of minimal risk.