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Von Hippel-Lindau Syndrome is an inherited disorder characterized by the formation of tumors and fluid-filled sacs (cysts) in different parts of the body

• Global incidence rate of VHL syndrome is around 2 to 2.5 people per lakh population, and the chance of inheritance to the next generation is as high as 50 %
• A 31 year old mother and her 11 year old son treated for same condition.

Chennai, 30th August 2022: Kauvery Hospital Chennai, a unit of Kauvery Group of Hospitals, a leading multispecialty healthcare chain in Tamil Nadu, today announced the successful treatment of an inherited disorder called Von Hippel-Lindau Syndrome (VHL Syndrome) in a woman and her child. Von Hippel-Lindau Syndrome primarily causes the growth of tumours and cysts in certain parts of the body, including the brain, spinal cord, eyes, inner ear, adrenal glands, pancreas, kidney, and the reproductive tract.

The woman aged 31 years presented with complaints of headache, blurring of vision, and vomiting that persisted for 3 weeks. Initial examinations and MRI imaging showed a large Left Cerebellar Lesion, which was identified as a Hemangioblastoma, a benign, highly vascular tumour that occurs in the brain, spinal cord, and retina and has the tendency to bleed a lot. Further inspection revealed that the lesion on the brain was around 4 cm and was attached to the brainstem. Surgery was performed where the complete tumor was excised and she soon recovered.

A few weeks later, her son aged 11 years approached the hospital with complaints of headache and vomiting. MRI Imaging showed a similar Left Cerebellar lesion of Hemangioblastoma in the same location as the mother. A surgery was soon performed and the tumor was excised from the boy.

The woman has been further informed by her team of treating doctors that she has two more lesions, one in the brain of size 1-2mm (far away from the treated area) and the other one, a lesion in the Cervical Spinal Cord, both of which are not now causing symptoms and can be treated later if required.

VHL Syndrome is a rare inherited disease, which the common population is unaware of. The global incidence rate is around 2 to 2.5 people per lakh population with the chance of inheritance by the next generation being as high as 50 percent. This explains occurrence of the same condition in the mother and her son in this instance. Speaking on the condition, Dr. Aravindan Selvaraj, Co-Founder & Executive Director, Kauvery hospital Chennai, “Surgeries for treating VHL are often complex and are a huge risk to life. There are also instances where people with VHL have cystic tumors and benign tumors in other parts of the body like the Kidneys and Pancreas which are regularly followed up by the doctors through CT scans or MRI with a view to monitor their growth and also to pick up early signs of any malignancy.”

Von Hippel-Lindau syndrome is also associated with a type of non-cancerous tumor called a pheochromocytoma, which most commonly occurs in the adrenal glands (small hormone-producing glands located on top of each kidney). The hormones that these tumors secrete can cause a hypertensive crisis (sudden, severe increase in blood pressure) and related complications like brain hemorrhage,kidney and heart failure.

“The treatment of this syndrome is quite complex as there are numerous requirements to be met, including the need for an expert anaesthesia team to manage the patient during surgery, regular screening facilities for the patient and their family members, genetic counselling and emotional support. I would like to commend the efforts of Dr. Arunkumar Karthikayan, Neurosurgeon and his team of medical experts for providing comprehensive evaluation and treatment for both the mother and child, resolving their condition and for providing all the necessary support required for their complete recovery, added Dr. Aravindan.

The mother and son have recovered and are undergoing regular follow – ups.