Infant undergoes challenging surgery for rare disorder

Disease affected his calcium levels

May 22, 2020 12:13 am | Updated 12:13 am IST - Navi Mumbai

Baby Konain Ansari with his parents.

Baby Konain Ansari with his parents.

While the healthcare system is under severe strain during the COVID-19 pandemic, a nine-month-old baby from Mankhurd with a rare congenital disorder of the parathyroid glands underwent successful surgery at Apollo Hospital in Navi Mumbai earlier this month.

Baby Konain was diagnosed with neonatal severe hypoparathyroidism in February. “We initially took him to a local doctor, but treatment showed no results. He then referred us to Apollo. Considering our financial condition, our treatment charges were paid through the hospital’s crowd-funding schemes,” the baby’s father, Mohammad Mudassir Ansari, said.

Dr. Anil K. D’Cruz, director, oncology, Apollo Hospitals Group, said the child showed symptoms of lethargy, irritability and failure to thrive since birth. “[He] was diagnosed with a rare genetic disorder of the parathyroid glands that causes loss of regulation of calcium levels in the body.”

Challenging surgery

He said the condition is potentially lethal, with only a handful of cases reported globally. “While surgery offers good prognosis, the challenge was in the diagnosis given the rarity of the condition. We also needed to bring down the calcium levels with medical treatment to optimise the child for surgery,” Dr. D’Cruz said.

He said the surgery is complex as the minuscule, translucent, critical and variable position of the parathyroid glands in children makes it difficult to locate them. “However, we were successfully able to remove the abnormal glands and use a special technique of auto-transplantation of a small bit of parathyroid to improve long-term prognosis. This will help make him less dependent on calcium replacement as he grows,” the doctor said.

‘Lot of change’

Konain was admitted on May 3 and underwent surgery two days later. He was discharged on May 20. “There is a lot of change in him. He now plays and is active and less irritable. He has not started sitting up yet, but doctors have told us he will meet all his milestones gradually. We were worried initially whether due to the lockdown our son could get proper treatment,” Mr. Ansari said.

The parathyroid is responsible for regulating calcium levels in the body. When the body has low calcium, it secretes parathormone (PTH) which mobilises calcium from the bone, increases absorption from intestines and prevents excretion from the kidney to maintain calcium levels. Usually, a feedback mechanism stops this secretion when the calcium levels return to normal.

In rare cases such as neonatal severe hypoparathyroidism, however, a fault in a single gene eliminates this feedback mechanism, resulting in extremely high calcium and PTH levels. Surgery is required to remove the abnormal glands, but is challenging as the parathyroid is close to critical structures in the neck, including nerves linked to the voice box.

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