OPERATIVE CORRECTION BY OSTEOTOMY OF RECESSED MALAR MAXILLARY COMPOUND IN A CASE OF OXYCEPHALY

Transcription

1 OPERATIVE CORRECTION BY OSTEOTOMY OF RECESSED MALAR MAXILLARY COMPOUND IN A CASE OF OXYCEPHALY By Sir HAROLD GILLIES, C.B.E., F.R.C.S., and STEWART H. HARRISON, F.R.C.S., L.D.S., R.C.S. From the Plastic and Jaw Unit, Rooksdown House, Park Prewett Hospital, Basingstoke ~TIOLOGY OXYCEPHALY is a condition of cranial dysostosis produced by premature synostosis of the coronal suture. The effect of early closure of the coronal suture is to produce a " turret-shaped skull," thus the condition is sometimes called turricephaly. This deformity is often associated with some degree of hydrocephalus. The raised intracranial pressure may give rise to headaches which are a common feature of the disease. Cerebral symptoms may be present. The prominence of the eyes is due to the forward projection of the great wing of the sphenoid which accompanies expansion of the middle cranial fossa. The deformity of the sphenoid may also give rise to a narrowing of the optic canal and defective vision (Thoma, 1946). Recession of the maxilla gives the appearance of an apparent prognathism. It is often associated with a high-vaulted palate due to premature synostosis of the vomer and palate. Separation of the eyes may occur secondarily. Oxycephaly is said to be familial, although this is often difficult to elicit as minor degrees of this deformity are apt to pass unrecognised. A Mendelian dominant is said to be the causative factor (Ellis, 1948). DIAGNOSIS Early diagnosis can be made radiologically. The synostosis can be seen, and digital markings, the result of raised intracranial pressure, can often be identified. A raised cerebrospinal fluid pressure may also be present. CASE REPORT A female, aged 14, was referred to H. D. G. in She was suffering from the condition of oxycephaly, and presented the following features :- The head was turret-shaped, and there was a marked retrusion of the upper jaw, giving rise to an apparent prognathism. The degree of retrusion was so marked that the tip of the nose nearly touched the upper lip. The nose itself was asymmetrically developed. The palate was very high and narrow. The eyes were so prominent that the lids were well below the iris, and the patient was inclined to walk with her head down. She did not complain of eye symptoms and there was no history of headaches. There was slight deafness, but no loss of the sense of smell. Mental development was within normal limits, and she was anxious to train as a nurse. 123

2 12 4 BRITISH JOURNAL OF PLASTIC SURGERY There was slight abnormality of speech, particularly in regard to sibilants. This was thought to be due to two factors : firstly, the high arched palate which prevented, to some extent, the tongue from making full contact ; and secondly, the two upper lateral incisors were displaced to the palatal side of the arch, thus preventing the action of the tongue in the pronunciation of the consonant " s." There was no family history and there were no associated abnormalities. The patient was referred first to Lieutenant-Colonel Botterell, C.A.M.C., for a neurosurgical examination. The examination proved negative and the cerebrospinal fluid pressure was normal. The condition was described as being quiescent. An operation for decompression of the orbital contents was considered but not advised. Radiological examination showed the typical appearance of turricephaly. FIG. i FIG. 2 Osteotomies before repositioning. Osteotomies after repositioning. FIG. 3 Osteotomy lines shown diagrammatically-- occipito-mental projection. Operation (H. D. G.).--The operation was designed to bring the whole face and palate forward en bloc (Figs. I, 2, and 3). Mr Fred. A. Walker, F.D.S., collaborated on the dental aspects of the case. An incision was made over the naso-maxillary junctions on both sides of the nose. Through this incision a nasal infracture was performed by osteotomy of the frontal process of the maxilla in the normal manner. This enabled a marked improvement in

3 OPERATIVE CORRECTION BY OSTEOTOMY 12 5 the shape of the nose to occur. While working laterally through the same incision the floor of the orbit was exposed, and after subperiosteal separation the osteotomy was continued just within the infraorbital margin, across the floor of the orbit to its outer wall, and eventually through the orbital ring at the fronto-malar suture. FIG. 4 FIG. 5 Before operation. After operation. FIG. 6 FIG. 7 Before operation. After operation. To complete this osteotomy, a second incision was made commencing in the region of the fronto-malar suture and passing downwards and laterally to overlie the zygomatic process of the temporal bone. Through this incision the fronto-malar process was divided, and the osteotomy continued medially to enter the infraorbital foramen. The zygomatic process of the temporal bone was divided through the infero-latera[ part of this incision. An osteotome was then introduced and directed downwards and medially to locate the spheno-maxiuary fissure. 2D

4 126 BRITISH JOURNAL OF PLASTIC SURGERY By directing this instrument slightly forwards and downwards, the pterygo-maxillary junction was divided. An incision was now made transversely across the hard palate in front of the posterior palatine arteries and over the alveolus just posterior to the last molar tooth in order to separate the soft tissues in continuity with the osteotomy in the region of the pterygo-maxillary fissure. Through the palatal incision the hard palate was divided with an osteotome introduced through the mouth. To complete the separation it was necessary to divide the septum, and this was done by introducing a large pair of scissors through the incision at the root of the nose, and cutting downwards and backwards to join the palatal incision. All layers of the septum were divided, and some difficulty was encountered in cutting through the bone. To facilitate mobility, bone elevators were inserted through the fronto-malar incisions, passed downwards behind the palate, and forward leverage was obtained. This manoeuvre was associated with forward rocking of the palate, as performed in the method of disimpaction when dealing with middle-third fractures. The malar maxillary component of the face with the palate and septum attached was now completely mobile. Forward correction was obtained and easily held with intermaxillary wiring. Post-operatively.--The patient made an uninterrupted recovery. The wounds healed per primam, and the retrusion of the upper teeth was corrected to normal incisor occlusion. Proptosis of the eyes was improved. Fourteen days after the operation a metal cap splint was attached by a mast to a plaster head cap, and the intermaxiuary wires were removed. All fixation was finally removed five weeks after the operation. Three and a half years later the articulation had stabilised in an edge-to-edge position, thus indicating that some recession had taken place. The articulation of the incisors is still edge-to-edge seven years after operation. Figs. 4 and 5, 6 and 7 show the patient before and after operation. DISCUSSION The forward repositioning of the maxilla by osteotomy is a subject of considerable interest to plastic surgeons of the present day, as the recessed position is a common finding in many of the congenital abnormalities which come under the generic heading of cranial dysostosis. Mobility and correction of the malar maxillary segment of the face were obtained in this case, and although some recession followed, immobilisation in this case could with advantage have been continued for a much longer period. Forward repositioning of the upper alveolus has been performed by H. D. G. in a case of Treacher Collins syndrome, and in a case of high contracted palate following cleft palate repair, by osteotomy of the upper alveolus producing a Guerin's type of fracture. It is hoped that these two cases will be published in the Journal in due course. The latter type of correction does not improve the upper facial configuration, but does much to improve the lower half and the function of the jaws. In this case it is interesting to note the improvement in the proptosis of the eyes as well as the marked repositioning and remodelling of the nose. It is, of course, only an apparent proptosis due to recession of the infraorbital ridges: but it constitutes one of the most prominent and unsightly features in cranial dysostosis, and causes more distress to the patient than any of the other deformities. The psychological improvement in this patient was very marked.

5 OPERATIVE CORRECTION BY OSTEOTOMY 12 7 SUMMARY An operation has been described, designed to reposition by osteotomy the recessed malar maxillary component of the face in a case of oxycephaly. An improvement in the upper incisor occlusion from a position of maxillary retrusion to an edge-to-edge bite was obtained over a seven-year follow-up period. Proptosis of the eyes was improved, and the overall effect of the operation was one of general improvement in the facial configuration. We would like to acknowledge our thanks to Miss Farmer for the diagrams, and to Mr Ferrill for the photographs. REFERENCES ELLIS, R. W. B. (1948). "Errors of Development and Growth." "British Surgical Practice," vol. ii. London : Butterworth & Co. TrlOMA, K. H. (1946). " Oral Pathology." Second Edition. London : Henry Kimpton.