NAGPUR: A rare tumour of adrenal gland was detected and treated successfully in an 18-month-old girl at AIIMS. The baby is from a tribal village near Gadchiroli.
She had gained excessive weight, hair (hirsutism), and was eating too much as compared to children of her age. She was also suffering from irritability.
Doctors detected that she had a double misfortune of two very rare conditions. First was an inherited condition present since birth called Beckwith Weidman Syndrome (BWS) that affects 1 in 13,700 newborns worldwide. The other was a large tumour of the adrenal gland that developed at around 1 year of her age. This type of tumour incidence is of 1 in 20 lakh births. It resulted in ‘Cushing’s Syndrome’ which is also a very rare condition in children. This syndrome occurs due to excessive hormones of the adrenal gland in the body.
“Considering the age of the child, effects of over produced hormones and large size of tumour, the peri-operative management and surgery was very challenging,” said senior paediatrician Dr Meenakshi Girish from AIIMS. The parents of the baby belong to low socio-economic strata and had visited multiple hospitals. At AIIMS, the child received complete treatment free of cost under MJPJY initiative.
The team of Dr Girish along with paediatric critical care specialist Dr Abhijit Chaudhary, paediatric residents and senior anesthesiologist Dr Amrusha Raipure, paediatric anesthesiologists Dr Bhuvaneswari B, Dr Sayli K and senior resident (Anesthesia) Dr Jagdish N stabilized the child for the surgery.
Paediatric surgeons Dr Nilesh Nagdeve and Dr Rafey A Rahman performed the complex surgery and excised the tumour successfully. “At AIIMS, we have experts for paediatric anesthesia, surgery, critical care to look after every aspect of the patient. That is the main reason we could save the girl who had many complications like hypertension,” said Dr Nagdeve.
In such children, post-operative course is often stormy and life threatening due to fluctuation in electrolytes and blood pressure. The case was managed successfully and the girl was discharged after proper recovery. She has started losing weight and even tried to walk in her first visit to AIIMS recently.
AIIMS Nagpur has paediatric ICU with advanced equipment. Director Maj Gen (Dr) Vibha Dutta, SM and medical superintendent Dr Manish Shrigiriwar encouraged the doctors to manage and successfully treat such challenging case.
