This young girl was born with Crouzon syndrome and resultant craniostenosis. Crouzon syndrome is a rare genetic disorder that affects the development of the skull. The junctions of the skull bones are prematurely fused leading to skull abnormalities.
The patient had the typical appearance of a child with Crouzon syndrome. These included increased distance between the eyes (hypertelorism) and bulging eyes. She also had a retruded upper jaw resulting in a concave appearance to the face.
This deformity resulted in decreased posterior pharyngeal space. As a result, she had chronic obstructive sleep apnea and snoring. She has also had a tracheostomy tube for over 10 years now. Her suffering had caused enormous distress in her parents.
She first underwent surgical correction in Cairo, Egypt. The surgery had however been a failure and had not corrected any of her complaints. Her parents had subsequently approached the US-based World Craniofacial Foundation (World CF).
The WorldCF had subsequently referred her to us for corrective surgery. We are a leading hospital for distraction osteogenesis in India. Many patients with Crouzon syndrome have undergone successful surgery in our hospital.
A 3D sterolithographic model of her skull was obtained after examination in our hospital. Detailed surgical planning with a mock surgery was performed on the 3D skull model. Our hospital is the pioneer of craniofacial distraction osteogenesis in India.
Attention was first paid to her long standing tracheostomy tube. This is the first time in the world that a patient was successfully weaned off a 10-year tracheostomy tube.
A bicoronal incision was first raised. Plates from the previous surgery were removed. This was followed by bone cuts to enable en-block advancement of the midfacial region. Kawamoto midface distractors were utilized to perform a Le Fort III midface advancement surgery. A total advancement of 3 cm was performed.
There was also a bony defect in her skull that was a result of her previous surgery in Cairo. This defect was closed utilizing a titanium mesh. Her nasal bridge was also augmented to improve her flattened nose. All scalp adhesions were also released at the time of her surgery.
The midface advancement surgery resulted in increased posterior pharyngeal space. There was complete resolution of her obstructive sleep apnea and snoring. She could breathe normally without a tracheostomy tube for the first time following her midface advancement surgery.
Kawamoto midface distractors were removed after three months following bony consolidation. The patient and her father were very happy with the results of her surgery.
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