News

New Hope for Patients with Bone Metabolic Disorder

Jan 23, 2007

A 25 year old woman is making a successful recovery in Westmead Private Hospital after a unique surgical operation to treat a disorder that was causing compression of her brainstem and spinal cord resulting in disabling headaches, weakness and swallowing difficulties.

Davina Knight had developed this disorder, known as basilar impression, as a result of her underlying disorder of osteogenesis imperfecta or bone metabolism.

Doctors who performed this new procedure are delighted with the progress for Davina who underwent a 15 hour operation at Westmead Private earlier this month.

“The procedure went extremely well with Davina suffering no complications. Her headaches and other symptoms have improved and she is now walking around the ward and looking forward to going home in the near future” Westmead Private Hospital neurosurgeon Dr Brian Owler reported.

The minimally invasive procedure, using image guidance technology and modern spinal instrumentation, is thought to be unique in the world and was performed by a collaborative team of surgeons.

“The development of this technique and procedure offers new hope to patients with osteogenesis imperfecta who develop this otherwise devastating condition. We hope that Davina is the first of many patients that will benefit from this operation in the future as we see this case as the first step in a surgical programme for the management of basilar impression,” Dr Owler said.

Children and adults with osteogenesis imperfecta are prone to develop basilar impression which means that the skull literally folds down over the spine. This results in compression of the brainstem and spinal cord and there is a high degree of morbidity and mortality associated with the condition.

Davina had developed basilar impression over a number of years as a result of her osteogenesis imperfecta. The basilar impression had become symptomatic with disabling headaches, weakness, clumsiness of her hands, swallowing difficulties and other neurological signs which were progressive.

“Surgery for basilar impression usually involves surgery through the mouth to remove the upper part of the spine and bottom of the skull which is known as a transoral approach. In cases such as Davina’s the spine is higher than normal and therefore a larger more morbid procedure involving dislocation of the midface of the skull has previously been used. Patients usually have to be sent to London at great expense and there is a high risk of complications including death associated with these procedures,” Dr Owler said.

The new procedure developed by doctors at Westmead is based on use of a minimally invasive endoscopic technique and the surgery involves an ENT surgeon (Associate Professor Melville Da Cruz) who provides exposure through the nose to allow the neurosurgeon (Dr Brian Owler) to resect the upper spinal column and base of the skull endoscopically.

The neurosurgeon then, through a separate incision posteriorly, performs a further decompression of the base of the brain and fuses the spine using screws and rods. Instead of a large incision along the face the patient has a tiny 4mm incision just under the nose.

DVD footage of the procedure using the endoscope is available.