Max Hospital Patparganj treats rare deformity in skull

A 11 month Iraqi infant Aysar Ihab khaleel Al-Azzawi was brought to Max Hospital Patparganj by his parents. He had a deformed skull and his head looked abnormally shaped.

He was examined by the Doctors and was diagnosed as suffering from a birth defect called Crouzon's syndrome. The most notable characteristic of Crouzon syndrome is cranial synostosis (where the bones of the skull fuse prematurely, causing deformity of the skull and hampering the growth of brain), Exophthalmos (bulging eyes due to shallow eye sockets after early fusion of surrounding bones), hypertelorism (greater than normal distance between the eyes).

Because the skull cannot expand in the normal pattern, it compensates by growing more in the wrong direction. Sometimes the resulting growth pattern provides the necessary space for the growing brain, but results in an abnormal head shape and abnormal facial features.

In cases in which the compensation does not effectively provide enough space for the growing brain, craniosynostosis results in increased intracranial pressure leading possibly to visual impairment, sleeping impairment, eating difficulties, or an impairment of mental development combined with a significant reduction in IQ. It was decided by the Doctors at Max Hospital Patparganj to treat the child and relieve his symptoms.

The surgical team included was headed by Senior Neurosurgeon Dr Sanjiv Dua and Facio-Maxillary and Facial Plastic Surgeon Dr Rohit Chandra, along with Dr Manoj K. Johar, Director, Plastic Surgery.

"At the age of 1 year this child who had come from Iraq was suspected to be suffering from Crouzon's Syndrome with cranial synostosis. In these cases , there are diverse congenital abnormalities mainly involving the facial bones and cranial voult. In this patient, the coronal sutures and anterior basal sutures were fused prematurely and were hampering the growth of the brain, and the skull was developing a grossly abnormal shape. The orbits were very shallow. The challenge here was to open up the coronal suture, advance the frontal bones and ensure that the bones do not fuse back quickly. The orbital advancement too had to be done. For this purpose, a wide bicoronal flap was raised and multiple segmental osteotomics were done. The orbital rim was separated from skull and fixed in a more physiological position. The strud grafts were used as spacers for the multiple segments of bone. This surgery took about 8-10 hours and required a combination of neurosurgery and plastic re-constructive skills.we are happy that it was a success and the baby is healthy" - Dr. Sanjeev Dua (Director-Department of Neurosurgery)

In an operation that lasted 7 hours, the affected bones of the skull were delicately cut, and removed from the skull, to allow the brain to expand and gain its normal shape.

The forehead bones and the upper part of orbit bones were also carefully cut, and removed from the skull.

The cut bone segments of the skull, the forehead and the orbits were then placed into the new correct position, without damaging the brain.

This resulted in the bones being placed in a more upward and forward position, and then fixed in the new position. This manoeuvre allowed the compressed brain to expand to its normal size and minimize the risks of brain damage, and gave a normal shape to the eyes of the infant.

The infant is successfully recovering and awaiting discharge.

This surgery is a rare and complex surgery and is a 1st for Max Hospital Patparganj, and probably also for the East Delhi region.

"The baby was suffering from Crouzon's disease and had very severe cranial synostosis, we did a combined surgery with the Neuro and the plastic reconstructive teams.The cranial bones were cut, removed and remodelled and advanced in the correct position along with the forehead to allow the brain to expand. The orbits were advanced to allow space for eyeball. The surgery is complex, unique and is done for the first time in Max Patparganj. The baby is now fine and on way to recovery." - Dr.Rohit Chandra (Senior consultant- (Facio-Maxillary & Facial Plastic))-Department of Plastic Surgery)

"This case is probably one of the typical cases where you need great experience and expertise in reconstituting form and function. Basically, it is a functional correction. So one has to have complete knowledge about not only the cosmetic element but also about the functional part. Reconstruction has to be done in such a manner that not only the form is corrected but the function is also improved and enhanced. This is one of the most challenging cases even for plastic surgeons as you are dealing with a very complex 3D type reconstruction. We worked very closely with the neuro team to achieve the desired results and make a difference to this baby's life."- Dr. Manoj K Johar (Director & Head- Department of Plastic Surgery)

"Our baby is fine now. Earlier his eyes were bulging out which made him look very different and it really scared us. We came all the way from Iraq. It was a big surgery and we are thankful to the almighty that our baby is on the way to recovery." -Mr. Ghadfer Khaleel Mahmood, patient's relative from Iraq. Max Hospital Patparganj has the infrastructure and the skilled surgeons to handle complex and rare surgeries successfully, even in infants. The Dept of Neurosurgery and Plastic Surgery have a team of Specialist surgeons who perform rare and complex surgeries to serve the populace of East Delhi and allied areas,