Orbital Dystopia Due to Orbital Roof Defect

Abstract

We performed a retrospective review of patients who presented with delayed dystopia as a consequence of an orbital roof defect due to fractures and nontraumatic causes to search for a correlation between orbital roof defect size and surgical indications for the treatment thereof. Retrospective analyses were performed in 7 patients, all of whom presented with delayed dystopia due to orbital roof defects, between January 2001 and June 2011. The causes of orbital roof defects were displaced orbital roof fractures (5 cases), tumor (1 case), and congenital sphenoid dysplasia (1 case). All 7 patients had initially been treated conservatively and later presented with significant dystopia. The sizes of the defects were calculated on computed tomographic scans. Among the 7 patients, aspiration of cerebrospinal fluid, which caused ocular symptoms, in 1 patient with minimal displaced orbital roof and reconstruction with calvarial bone, titanium micromesh, or Medpor in 6 other patients were performed. The minimal size of the orbital roof in patients who underwent orbital roof reconstruction was 1.2 cm (defect height) x 1.0 cm (defect length), 0.94 cm(2). For all patients with orbital dystopia, displacement of the globe was corrected without any complications, regardless of whether the patient was evaluated grossly or by radiology. In this retrospective study, continuous monitoring of clinical signs and active surgical management should be considered for cases in which an orbital roof defect is detected, even if no definite symptoms are noted, to prevent delayed sequelae.

The Management of Orbital Roof Fractures and Defects: A Review

“…16 Dysplasia of the greater wing of the sphenoid, as seen in 5% to 10% of cases of neurofibromatosis type 1, results in an ORD and can cause pulsatile proptosis from the transmission of cerebral pulsation. 17,18 Although generally considered congenital, it has been suggested that it can also develop or progress postnatally. 19 Congenital absence of a portion of the orbital roof with no associated syndrome or disease identified is a rare occurrence and has been reported to result in, or present with, vertical orbital dystopia.…”

“…Rha et al 18 described a case series of 145 patients with ORF of which 47 (32.4%) had displaced fractures. Thirty-five of these (74.5%) were treated conservatively as they had no initial definite symptoms.…”

“…16,18,20,24 The timing and the development of these sequelae of ORD can vary significantly, with some patients remaining symptom free (or at least not knowing they have symptoms as they have always been present) or not being unduly troubled by the presence of very large defects. 18…”

“…However, although case series and reports describe the results and complications associated with intervention or conservative treatment, intervention rates vary widely. 7,8,10,18,[29][30][31][32] Moreover, there are no randomized controlled trials to help guide intervention decisions and intervention rates vary according to surgeons and establishments, particularly when the anterior cranial fossa is involved.…”

“…After addressing any progressive primary pathology, it has been suggested that management/intervention decisions can be guided by the defect size. 18,31 For example, the authors are aware of one individual with the absence of almost the entire orbital roof (see Fig. 1) due to a venolymphatic lesion, who had untroublesome symptoms of globe pulsation and had no ill-effects from conservative management over several years of follow up.…”