Reconstruction of Sphenoid Wing Dysplasia with Pulsating Exophthalmos in a Case of Neurofibromatosis Type 1 Supported by Intraoperative Navigation Using a New Skull Reference System

ABSTRACT

A patient with neurofibromatosis type 1 had pulsating exophthalmos of the right eye with diplopia resulting from severe dysplasia of the sphenoid bone and consecutive herniation of the right temporal lobe. The right orbital tectum was reconstructed with titanium mesh and iliac spongiosa via a lateral orbitotomy using intraoperative navigation. For intraoperative referencing a cortical fixed-reference system and a skin scanning laser device were used. Postoperatively, the diplopia was reduced, but the patient asked for further treatment and the procedure was repeated 6 months later. Intraoperatively, the previously implanted titanium mesh was deformed and most of the transplanted bone was resorbed, probably because of pressure. A more extended mesh was implanted and iliac spongiosa was placed on both sides. Intraoperative navigation was used during both procedures. The adverse effects of diplopia were minimized and follow-up computed tomography after seven months confirmed that the bone graft was in place.

KEYWORDS

Neurofibromatosis type 1 - sphenoid dysplasia - intraoperative navigation

REFERENCES

• 1 Poole M D. Experiences in the surgical treatment of cranio-orbital neurofibromatosis.  Br J Plast Surg . 1989;  42 155-162
• 2 Friedrich R E. Einseitige Fehlbildungen der vorderen Schädelbasis bei Neurofibromatose Typ 1. In: Schmelzle R, ed. Schädelbasischirurgie Damaskus: Al-Budoor 1998: 49-53
• 3 Riccardi V M. Neurofibromatosis. Phenotype, Natural History, and Pathogenesis Baltimore, MD: John Hopkins University Press; 1992
• 4 Jackson I T, Carbonnel A, Potparic Z, Shaw K. Orbitotemporal neurofibromatosis: classification and treatment.  Plast Reconstr Surg . 1993;  92 1-11
• 5 Sießegger M, Mischkowski R A, Schneider B T, Krug B, Klesper B, Zöller J E. Image guided surgical navigation for removal of foreign bodies in the head and neck.  J Craniomaxillofac Surg . 2001;  29 321-325
• 6 Hassfeld S, Zöller J, Albert F K, Wirtz C R, Knauth M, Mühling J. Preoperative planning and intraoperative navigation in skull base surgery.  J Craniomaxillofac Surg . 1998;  26 220-225
• 7 Gunkel A R, Freysinger W, Thumfart W F. Experience with various 3-dimensional navigation systems in head and neck surgery.  Arch Otolaryngol Head Neck Surg . 2000;  126 390-395
• 8 Raabe A, Krishnan R, Wolff R, Hermann E, Zimmermann M, Seifert V. Laser surface scanning for patient registration in intracranial image-guided surgery.  Neurosurgery . 2002;  50 797-801
• 9 Maroon J C, Kennerdell J S. Surgical approaches to the orbit.  J Neurosurg . 1984;  60 1226-1235
• 10 Carrau R L, Snyderman C H, Curtin H D, Janecka I P, Stechison M, Weissman J L. Computer-assisted intraoperative navigation during skull base surgery.  Am J Otolaryngol . 1996;  17 95-101
• 11 Nimsky C, Ganslandt O, Cerny S, Hastreiter P, Greiner G, Fahlbusch R. Quantification of, visualization of, and compensation for brain shift using intraoperative magnetic resonance imaging.  Neurosurgery . 2000;  47 1070-1080
• 12 Jödicke A, Deinsberger W, Erbe H, Kriete A, Böker D K. Intraoperative three-dimensional ultrasonography: an approach to register brain shift using multidimensional image processing.  Minim Invasive Neurosurg . 1998;  41 13-19
• 13 Heiland M, Schulze D, Adam G, Schmelzle R. 3D-imaging of the facial skeleton with an isocentric mobile C-arm system (Siremobil Iso-C^3D).  Dentomaxillofac Radiol . 2003;  32 21-25