[Abnormalities of the maxillary sinus in type 1 neurofibromatosis].
OBJECTIVE
The aim of this study was to determine the malformations of the maxillary sinus in neurofibromatosis type 1 patients (NF1).


METHODS
Twenty-two patients with NF1 were investigated clinically and radiologically: 11 had an unilateral trigeminal plexiform neurofibroma and 11 had multiple cutaneous neurofibromas. The histological type of NF was ascertained in all cases following tumor resections. The malformation of the maxillary sinus was assessed on plain radiographs and computed or magnetic resonance tomograms. Intraindividual side comparison was used to judge the size of the sinus and its position in the midface.


RESULTS
In patients with cutaneous neurofibromas the maxillary sinus appeared symmetrical in size and position. The pneumatization of the sinus had no abnormalities on the radiographs. Malformations of the maxillary sinus were restricted to plexiform neurofibromas. On the side affected by a plexiform NF, the sinus appeared hypoplastic and caudally displaced due to an enlarged ipsilateral orbit. The expansion of the sinus to the lateral side was impaired, obviously due to tumor masses. Consecutively, the alveolar process of the affected side was also displaced leading to a complex malocclusion.


CONCLUSIONS
Malformations of the face are frequently presented as case reports in the literature. Emphasis is given to the elephantiasis-like tumor growth of the face in certain patients with NF1. The underlying pathology has not yet been fully understood. This report provides evidence that in the midfacial region the overgrowth is predominantly caused by the plexiform neurofibroma itself and that the bones can even be hypoplastic and show scoliosis-like malformation compared to the nonaffected side. These findings are relevant when debulking procedures of the face are planned for NF1 patients.
Friedrich
R E
RE
Klinik für Mund-, Kiefer- und Gesichtschirurgie, Nordwestdeutsche Kieferklinik, Universitätsklinikum Hamburg-Eppendorf, Martinistrasse 52, 20246 Hamburg. rfriedri@uke.uni-hamburg.de
Giese
M
M
Mautner
V F
VF
Schmelzle
R
R
Scheuer
H A
HA
D000293:Adolescent; D000328:Adult; D000539:Alveolar Process / Q000000981:diagnostic imaging; D002648:Child; D003390:Cranial Nerve Neoplasms / Q000000981:diagnostic imaging*; D005146:Facial Asymmetry / Q000000981:diagnostic imaging; D005153:Facial Neoplasms / Q000000981:diagnostic imaging*; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted; D021621:Imaging, Three-Dimensional; D008297:Male; D008443:Maxillary Sinus / Q000002:abnormalities* / Q000000981:diagnostic imaging; D008875:Middle Aged; D018318:Neurofibroma, Plexiform / Q000000981:diagnostic imaging*; D009456:Neurofibromatosis 1 / Q000000981:diagnostic imaging*; D014057:Tomography, X-Ray Computed; D020433:Trigeminal Nerve Diseases / Q000000981:diagnostic imaging*