Gorham disease involving the maxillofacial bones: A perplexing entity

Abstract

Gorham disease is a rare disorder with progressive osteolysis which leads to the vanishing of bones. Its etiology and ideal management strategy are still an enigma. A case of Gorham disease involving the maxillofacial region in a 25-year-old male with an emphasis on etiology and diagnosis is discussed.

Unilateral Creeping Destruction of Deformed Mandibular Ramus and Angle Associated with Extensive Facial Plexiform Neurofibroma in Neurofibromatosis Type 1: A Case Report with Analysis of the Literature for Diagnosing Osteolytic Events of the Mandible in Tumor-suppressor Gene Syndrome

“…The discussion of this recently published case is of interest for the analysis of the current case because it allows various aspects of the diagnosis to be discussed: i) The importance of clinical findings in confirming the diagnosis of NF1; ii) the certain exclusion of syndromic disorders for the establishment of the diagnosis of Gorham-Stout syndrome; and iii) the variability of skeletal malformations of the mandible, in particular in NF1. The radiographic illustrations of that report (43) show several important findings on the affected mandibular side: rarefication of processus muscularis and processus articularis, deep sigmoid notch, shortening of the mandibular corpus and retained molars with significant spacing from the anterior teeth, associated deformities of the glenoid fossa, and a cuplike osseous defect at the lower edge of the mandible near the angle. Severe hypoplasia of the mandibular corpus in the region of the retained molars in all dimensions was also shown (43).…”

“…Reference to the delineation of NF1-associated osteolysis to Gorham-Stout syndrome is not only of theoretical interest. Recently there was a case reported of unilateral deformation of the facial skull, which, according to the authors, fulfilled the criteria of Gorham-Stout syndrome (43). The discussion of this recently published case is of interest for the analysis of the current case because it allows various aspects of the diagnosis to be discussed: i) The importance of clinical findings in confirming the diagnosis of NF1; ii) the certain exclusion of syndromic disorders for the establishment of the diagnosis of Gorham-Stout syndrome; and iii) the variability of skeletal malformations of the mandible, in particular in NF1.…”

Dental management of lymphangiomatosis: case report

“…3–5). Failure of eruption and mobile teeth are characteristic features of this condition, which contribute to an increased likelihood of substantial malocclusion. Orthodontic treatment and maxillofacial surgery are likely to be pillars in the treatment of such cases.…”

“…Lymphangiomatosis has been associated with anaemia, which has a variety of oral manifestations including generalised stomatitis, mucosal pallor, atrophy of the filiform papillae of the tongue, glossitis, dysgeusia, angular cheilitis, gingivitis, recurrent aphthous ulcers and Plummer‐Vinson syndrome. Increased fibrovascular connective tissues and vascular spaces resulting from unregulated lymphatic proliferation indicate the potential for gingival overgrowth and mucosal thickening. The importance of immaculate oral hygiene and frequent visits to the GDP is paramount, as is a referral to a periodontist for gingivectomy if needed.…”

“…Anaesthesia and airway management are further complicated in cases of respiratory involvement. Other considerations for the surgical management of these cases include the differential diagnoses of osteolytic lesions in the jaws, TMJ pain and dysfunction, and spontaneous pathologic fractures.…”