~ The girl was suffering from severe ‘Rigid Torticollis’ since infancy after two surgeries failed to address a fibrous tumor in neck muscle ~
Navi Mumbai,2 August 2021,: Somya Tiwari, seven-year-old girl, from Valsad, Gujarat, had her neck fixed in almost 90 degrees angle after two consecutive surgeries failed to address her neck tilt caused by a neck muscle tumour. The presence of this tumor led to neck tilting and rotation which is called as ‘Torticollis’, also known as wry neck. However, in this case the muscle had calcified, and the collar bone and skull bone were united by a bony bar which had fixed her head to body without any movement. Such a complicated case has not been reported in any orthopaedic/ medical journal/ literature and this for the first time a multi-staged surgery for the condition has been performed.
The child was successfully treated at Apollo Hospitals Navi Mumbai through a complex multi-stage surgery carried out by a specialized multi-disciplinary team. Before visiting Apollo Hospitals Navi Mumbai, she had been suffering from this condition for over five and a half years. The family brought the girl to Apollo Hospitals Navi Mumbai as their last hope to correct the deformity so that the girl could live a normal life.
The girl was examined by team of doctors from Spine Surgery and Paediatric Orthopaedic. A detailed treatment plan of multi-stage correction was drafted but it needed support from ENT, pediatrics, pediatrics, plastic surgery department. The child’s head was completely tilted and fixed with no movement at all. MRI / CT scan imaging showed a bony bar extending from the collarbone to the mastoid bone located behind the ear. After detailed consultation, a complex multi-stage surgery was planned.
Dr. Agnivesh Tikoo, Spine Surgeon, Apollo Hospitals Navi Mumbai said, “When the child was six months of age, the family had noticed a lump on the right side of neck which gradually started increasing in size and caused a tilt in the neck. At 9 months of age, she underwent her first surgery. This appears to have been a condition called as benign fibrobastic proliferation of sternocleidomastoid muscle, also known as ‘fibromatosis colli’. This is a congenital fibrotic
process that is rarely seen and affects infants with an incidence of 0.4% of live births. It is usually on one side, affects the right side in 3/4th of cases, and usually, male infants are affected slightly more often than the females.
Dr. Swapnil Keny, Paediatric Orthopaedic Surgeon, Apollo Hospitals Navi Mumbai added that in the cases of “Fibromatosis Colli’ there usually is a history of complicated delivery and birth injury in more than half of the cases. This causes an injury to the neck muscle that scars as it heals with the amount of scar in the muscle determining the tightness of the muscle and severity of the torticollis. The child in this case underwent a second surgery at 15 months of age elsewhere, but she had a fall and could not follow up the treatment and this resulted in severe neck deformity. “The neck tilt and rotation were so bad that the 1st and 2nd cervical spine bones (vertebra) had slipped from their original position” said Dr. Tikoo.
The family took the child to many hospitals, which refused intervention citing high risk The family gave up any hope of getting better and stopped seeking medical intervention. The patient was then brought to Apollo Hospitals Navi Mumbai as the last hope for treatment.
The multi-stage surgery was carried out in three stages, led by Dr Agnivesh Tikoo, Spine surgeon and Dr. Swapnil Keny, Paediatric Orthopaedic Surgeon. The sternocleidomastoid, a rope shaped muscle that extends from the inner end of collar bones to part of skull behind the ears, had become contracted and calcified. In the first stage, the bony bar was excised and removed along with tight muscle fibres were removed with help of ENT and plastic surgery team. The first stage of the surgery resulted in an immediate partial correction of the neck tilt but it was just the beginning of the treatment. Correcting the neck in one stage, could make the child completely paralyzed. A Pediatric Halo, which holds the head via metal pins was applied to child’s skull and gradual traction with weights was applied which was given gradually over the next three weeks to gradually correct the deformity.
The second stage was carried out 3 weeks after the first stage surgery. The second stage involved fixing the slipped cervical vertebrae which was performed under neuromonitoring ( a device/ system which monitors the spinal cord and nerves during the surgery). The child was
protected in Halo Vest to let the bones heal. The child remained in the Halo Vest for three months before it was removed to be replaced with a special made collar.
Child underwent a third minor procedure few days ago to make her neck more flexible to aid in physiotherapy. The child can see straight with both the eyes after 5 and a half years of ‘one eyed vision’ and can hold her neck straight.
Nilesh Tiwari, Father of the Child, said, “It was a difficult time for the family, to see the child suffering. We went to multiple places across India, but nobody was willing to operate considering it was a high risk case. One of my colleagues, recommended to meet the specialists at Apollo Hospitals. The Doctors, explained the case in detail and multiple surgeries were performed. We are happy to see our daughter to hold her head straight and be normal like any other child.”
Santosh Marathe, COO & Unit Head, Apollo Hospitals Navi Mumbai said, “The child’s deformity is today significantly better. She is able to live a normal life without the neck deformity and is able to move her neck without any limitation. We are happy that we were able to treat the child and live up to the family’s hopes and expectations with which they had brought her to Apollo Hospitals. It is cases like this that inspire us to continue to create accessibility to advanced medical technology for our team of finest medical experts to consistently deliver best in class clinical outcomes and set new benchmarks in healthcare delivery and patient experience.”