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Surgical treatment of craniofacial fibrous dysplasia in adults

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Abstract

Craniofacial fibrous dysplasia (FD) is a rare disorder that may require neurosurgical expertise for definitive management; however, surgical management of FD in adult patients is uncommon. Although other therapies have been shown to slow progression, the only definitive cure for adult craniofacial FD is complete resection with subsequent reconstruction. The authors review the biological, epidemiologic, clinical, genetic, and radiographic characteristics of adult FD, with an emphasis on surgical management of FD. They present a small series of three adult patients with complex FD that highlights the surgical complexity required in some adult patients with FD. Because of the complex nature of these adult polyostotic craniofacial cases, the authors used neurosurgical techniques specific to the different surgical indications, including a transsphenoidal approach for resection of sphenoidal sinus FD, a transmaxillary approach to decompress the maxillary branch of the trigeminal nerve with widening of the foramen rotundum, and complete calvarial craniectomy with cranioplasty reconstruction. These cases exemplify the diverse range of skull base techniques required in the spectrum of surgical management of adult FD and demonstrate that novel variations on standard neurosurgical approaches to the skull base can provide successful outcomes with minimal complications in adults with complex craniofacial FD.

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Acknowledgments

We thank Kristin Kraus, M.Sc., for editorial assistance and Jennie Swensen, M.A., for illustrations.

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Correspondence to William T. Couldwell.

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Comments

Engelbert Knosp, Vienna, Austria

Polyostotic fibrous dysplasia in adults, with continuous growth, are rare in neurosurgical practice. This article highlights the disease in its epidemiological, biological, and genetic implications.

The common denominator of the three cases described here is a long-time progression of the disease, polyostotic involvement of the skull base, and one or more surgeries over the time. The requirement to treat such complex cases is to be familiar with a variety of skull base approaches.

A crucial point in adult FD is the indication for surgery and to which extent the resection should be performed. I agree with the philosophy of the authors to treat only symptomatic cases as cranial nerve compression, breathing problems, or severe headache and not cosmetic consideration. With the brilliant surgical technique shown here the authors could achieve an excellent outcome in these challenging cases.

Ralf A. Kockro and Yusuf Dawang, Zurich, Switzerland

Craniofacial fibrodysplasia is rare and manifests in diverse forms with a wide spectrum of locations and clinical features. This paper provides a comprehensive overview of the epidemiology and biology of the disease, discusses clinical and radiological features, and describes multidisciplinary treatment options. Treatment patterns tailored to the clinical and radiological presentation of the disease are illustrated in context of the author’s expertise in skull base surgery and three illustrative cases highlight surgical techniques and emphasize on pitfalls and strategies. We agree with the authors that complete resection with craniofacial reconstruction remains the definitive treatment for adult craniofacial fibrodysplasia with neuropathy, pain, and cosmesis being the lead indications for surgery. The complexity of the cases illustrated demonstrates the need for an experienced and multidisciplinary surgical team as a prerequisite for effective surgery, patient safety, and good outcome.

Kartik G. Krishnan, Giessen, Germany

Bowers et al. provide a comprehensive review of craniofacial dysplasia in their article and report on three challenging cases undergoing surgical management. Craniofacial fibrous dysplasia (CF FD) is a benign disease that is quite rare; the polyostotic form of presentation is slightly higher in frequency than the monofocal ones. CF FD as a part of a syndromal disease is still rarer. According to a recent retrospective analysis, the sphenoid bone is most frequently involved, followed by frontal, maxillary, ethmoid, parietal, and other bones (Fattah et al., 2013, doi: 10.1016/j.bjps.2013.05.031). Depending on the presenting symptoms, patients are first seen by plastic and maxillofacial surgeons (craniofacial asymmetry and perceivable masses of the skull), neurologists (headaches, incidental), ophthalmologists (impairment of vision), and ENT surgeons (snoring) in that order, as evidenced by the number of published literature produced by these groups. Surgical treatment solely depends on the severity of clinical symptoms and growth behavior of the tumor. The neurosurgeon is at the end of the service-providing chain—when cranial nerve decompression or dural and/or cranial reconstruction is called for. These aspects make publications on CF FD for the neurosurgical readership all the more necessary. The rarity of the condition would certainly not facilitate the development of generally applicable treatment guidelines based on one institution alone; however, publication of cases in periodic intervals would serve to increase awareness and perhaps initiate meta-analyses. Since multiple areas of the craniofacial bones tend to be involved, interdisciplinary logistics, and, above all, innovative and individually tailored surgical approaches are essential—as is the case in any type of surgical reconstruction.

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Bowers, C.A., Taussky, P. & Couldwell, W.T. Surgical treatment of craniofacial fibrous dysplasia in adults. Neurosurg Rev 37, 47–53 (2014). https://doi.org/10.1007/s10143-013-0500-z

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