History of multiple surgeries for a right eye deformity
The patient is a 38 year old male from Virajpet in India who first noticed a swelling that was gradually causing his right eye to become displaced outwardly around ten years ago. This had begun as a small swelling near his right eye with a gradual increase in size. The swelling had gradually grown in size to the point that the patient developed double vision. His day to day activities of daily living began to get disrupted and this was beginning to affect his work as well as family life. It was at this point that he decided to seek medical help.
Initial treatment history for the deformity
He sought medical help for the first time around ten years ago at a hospital near his hometown for this gradually developing swelling. Comprehensive imaging studies including CT scans had been obtained and a bony growth was noticed in the right orbital cavity. Subsequently, he had undergone two surgeries to address this condition at that same hospital.
The first surgery was through a bicoronal approach and the second was performed through the use of an endoscope. Both surgeries however were unsuccessful in addressing his complaints and he subsequently developed a severe infection in the area after surgery. Healing of the surgical site had been delayed due to the infection.
This was a very difficult period for him and his family as he was not able to work during this entire phase. He was also ultimately not satisfied with the results of the two surgeries and was beginning to go into a depression because of his deteriorating vision.
Referral to our hospital for management of his problem
It was then that he approached an oral and maxillofacial surgeon in his hometown. Realizing that this was also a craniofacial problem, the surgeon referred him to our hospital for definitive management of his problem. Our hospital is renowned for facial asymmetry surgery and craniofacial surgery in India. Cranial deformity surgery is performed routinely at our hospital for syndromic patients with Apert’s, Crouzon, Franceschetti and other syndromes that cause craniofacial deformities.
A center of excellence in craniofacial surgery
Craniofacial surgery is a superspecialty, which deals with surgical correction of deformities of the craniofacial skeleton including facial deformities. One of the aims of surgical correction of facial deformities is to establish facial symmetry for the patient. Craniofacial surgery also involves components of plastic surgery.
Many syndromes like Pfeiffer syndrome present with premature fusion of the coronal suture, sagittal suture and lambdoidal suture. These cause abnormal shapes to the top of the head leading to an abnormally shaped head. A skull deformity like posterior plagiocephaly can cause an abnormally shaped head amongst other deformities. This manifests itself in the infant skull itself and progressively gets worse with the passage of time. A molded helmet is fabricated for the patient to protect the skull during the healing phase after the surgery.
Initial examination and diagnostic studies at our hospital
Dr SM Balaji, Craniofacial Surgeon, examined the patient and obtained a detailed history. He then obtained a 3D CT scan to determine the exact extent of the bone disease. This revealed that there was overgrowth of the bone along the roof of the orbit and along the medial wall, which was pushing outwards and causing vision changes. The bony overgrowth appeared glass like and was diagnosed as ossifying fibroma. Our hospital is also renowned for ossifying fibroma surgery.
What is ossifying fibroma?
Ossifying fibroma is a benign fibroosseous lesion of the jaw that is very rare in occurrence. Normal bony tissue is replaced by fibrous tissue. This lesion is well demarcated from normal bony tissue and this is what differentiates it from fibrous dysplasia. Most common site of occurrence is in the craniofacial bones. It less commonly involves the maxilla, orbit or the paranasal air sinuses.
Ossifying fibroma occurs in two histological patterns, trabecular or psammomatoid. Irrespective of the histological pattern, ossifying fibroma can induce a substantial degree of orbital inflammation. This must thus be included in the differential diagnosis for acute orbital inflammation.
In the case of this patient, there was an overgrowth of fibrous bone extending into the roof the right orbit and also to the base of the skull up to the hypophyseal fossa. This excessive fibrous tissue had compressed the optic nerve thus resulting in blurred vision.
Meticulous treatment plan formulated and explained to the patient
A 3D stereolithographic model was obtained using the 3D CT scan. The exact extent of the bony overgrowth was determined extending into the intracranial regions and this was carefully marked. The planned surgery was explained in detail to the patient who agreed to the treatment plan and signed the informed consent.
It was planned to approach the bony overgrowth through the bicoronal approach through which it would be removed from the roof and medial wall of the orbit, thus returning the eye back to its normal position.
Successful completion of the surgical procedure
Under general anesthesia, an incision was made through the old bicoronal flap scar to avoid any new scar formation. The right frontal craniotomy defect with the mesh was exposed and the mesh removed. A lumbar puncture was made following which CSF was drained to reduce the intracranial pressure. Dura was then retracted and the intraorbital whitish bony hard tumor was trimmed. An osteotomy was next performed over the roof and along the medial wall of the orbit. The bony overgrowth was then excised completely and this resulted in complete correction of the proptosis without any damage to the optic nerve.
Pupillary function was then assessed and found to be normal. The frontal bone flap was then placed back in position followed by a titanium mesh over the bony defect. Skin closure was obtained through the use of skin staplers.
Patient expresses his complete satisfaction before discharge from the hospital
The patient was very happy with the outcome of the surgery as the results were immediate. The proptosis had been corrected and he said that his vision had also improved dramatically.
