An 11-year old girl previously diagnosed of a congenital facial deformity disorder- Crouzons syndrome approached the center for deformity correction. Crouzon’s syndrome results from a genetic defect that causes early closure/fusion of the skull bones. This leads to uncoordinated and improper growth of face. Most of her orbital contents (eyes) were pushed out. 5years back, due to early fusion of the cranial sutures, she had an acute episode of increased intra-cranial pressure. If untreated, severe brain damage, vision damage due to optic nerve atrophy and possible life threatening situation would have been presented. To relieve this and to give a solution for this problem, a Ventriculoperitoneal shunting surgery was performed. This is to treat excess cerebrospinal fluid (CSF) causing the intra-cranial pressure. In this procedure a miniature – valve- fluid pump is implanted in layers of the skin behind the ear. This pump has two extensions via catheters – one placed in the ventricle of brain and the other in the peritoenal cavity (stomach). When extra pressure builds up around the brain, the mechanism opens, and the excess fluid drains through the tube in to the stomach, lowering the intracranial pressure. For this young girl, the amount of advancement required was large. Conventional therapy for such deformities is Lefort III advancement surgery, which is not applicable for this case as the deficit was large. Hence the treatment for this girl was to surgically advance the midface by Lefort III advancement and then employing Distraction osteogenesis, to advance the midface. For this treatment, a bicoronal incision was placed and the entire skin was reflected. Care was taken to preserve the periosteum of the cranial bone intact. The supra-orbital -trochlear region was identified and the cut was started from there on. Using gentle surgical manipulations, the entire content of the orbit was closely removed in such a way that the entire components of the eye were with the coronal flap. The bone cut was extended in to the frontozygomatic region creating a traditional Lefort III cut. Care was taken to place the bone cut along the frontonasal region so that the nasal architecture is not affected. Following this the zygoma (cheek bone) is split carefully preserving the nasal bone regions. Then through the intra-oral approach, the pterygoid- maxillary dysjunction was carried out. The dysjunction was then completed from the extra-oral approach too. Using appropriate instruments, all the delicate structures were persevered. This type of approach led to the entire maxillary/ nasal component to be made mobile as a monobloc. Hence certain Surgeons also refer this technique as - Monobloc advancement also. A special type of distractor - Kawamottos distractor was used to advance the dislodged maxillary/nasal complex. The arms of the distractor were carefully placed on the thin temporal bone (only 4mm long screw used) and on the zygoma also. This is an internal distractor where only the active arm is only visible externally, that too along the scalp region. Such arrangement removes the requirement of cumbersome traditional rigid external framework based craniofacial distraction osteogenesis. After careful checking of the distractors, all the layers were closed. A drain was placed and combination of subcutaneous stitches and staples were used to close. The VP shunting machine was preserved till healing.
Posted on: Mon, 22 Dec 2014 18:38:53 +0000
Trending Topics
Recently Viewed Topics
© 2015