Le Fort III distraction for Crouzon syndrome with a modular internal distraction system

Midface hypoplasia in a 5-year-old boy with Crouzon syndrome was treated using a rigid external distraction device. Seven months prior to this surgery, a bone allograft was used during frontal-orbital bar advancement. Three weeks after the Le Fort III distraction a depression fracture was diagnosed, associated with an intracerebral screw penetration of 3.5 cm. This case report cautions against the use of distraction osteogenesis in patients when previous allografts were used for calvarial reconstruction. We suggest that a preoperative computed tomography scan is mandatory to accurately delineate the location for placement of the pins.

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Meningoencephalocele: a late complication of Le Fort III midfacial advancement in a patient with Crouzon syndrome

Journal of Neurosurgery Pediatrics ◽

10.3171/2010.8.peds10155 ◽

2010 ◽

Vol 6 (4) ◽

pp. 368-371

Author(s):

Emily B. Ridgway ◽

Alexander E. Ropper ◽

John B. Mulliken ◽

Bonnie L. Padwa ◽

Liliana C. Goumnerova

Keyword(s):

Cranial Base ◽

Cerebral Injury ◽

Unusual Complication ◽

Crouzon Syndrome ◽

Csf Rhinorrhea ◽

Le Fort ◽

Le Fort Iii ◽

Anterior Cranial Base ◽

Obstructive Sleep ◽

Computed Tomography Scanning

Complications of Le Fort III midfacial advancement include CSF rhinorrhea, meningitis, and ocular and cerebral injury. This report reviews the anatomy of the Le Fort III osteotomies and their relevance to the unusual complication of meningoencephalocele. In this report, a young male patient with Crouzon syndrome underwent subcranial midfacial advancement at the age of 10 years for obstructive sleep apnea and ocular exposure. He presented 4 years later complaining of nasal obstruction. On physical examination, a mucous-covered mass was noted in the left upper nasal vault medial to the turbinates. Computed tomography scanning and MR imaging confirmed the diagnosis of frontoethmoidal meningoencephalocele. Repair of the meningoencephalocele was accomplished using a combined neurosurgery and plastic surgery approach. Meningoencephalocele is a rare complication of subcranial midfacial advancement. The abnormal anatomy of the anterior cranial base in patients with syndromic craniosynostosis places them at greater risk for fracture of the cribriform plate and dural tears during this procedure. Unrecognized dural injury is the etiology of this complication in this young patient; however, elevated intracranial pressure may have been a confounding factor. Attention to the anatomy of the anterior cranial base, as seen on sagittal CT images, will aid in preventing this complication.

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Patient with Crouzon Syndrome Treated with Modified Le Fort III Osteotomy without Previous Orthodontic Treatment: Case Report and a Review of the Literature

Case Reports in Dentistry ◽

10.1155/2020/6248971 ◽

2020 ◽

Vol 2020 ◽

pp. 1-9

Author(s):

Farnoosh Mohammadi ◽

Afrooz Javanmard ◽

Hamid Mojtahedi

Keyword(s):

Orthodontic Treatment ◽

Common Type ◽

Crouzon Syndrome ◽

Review Of The Literature ◽

Upper Airways ◽

Le Fort ◽

Intraoral Approach ◽

Le Fort Iii ◽

Craniofacial Dysostosis ◽

History Of

Crouzon syndrome is the most common type of craniofacial dysostosis anomaly which presents a great challenge for clinicians since birth. Multiple synostoses in the sutures of the cranial base in this syndrome result in the hypoplasia of the midface, shallow orbits, a short nasal dorsum, maxillary hypoplasia, and, in severe cases, obstruction of the upper airways. Apart from esthetic and functional problems, these patients suffer from various psychological problems which mandate correction of midface deformities at younger ages. The aim of this report is to describe the case of a 26-year-old female patient with Crouzon syndrome displaying severe midface hypoplasia and proptosis with no history of orthodontic treatment, who was treated with modified Le Fort III osteotomy with a coronal and intraoral approach without periocular incisions.

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Le Fort III Distraction With Internal vs External Distractors

The Cleft Palate-Craniofacial Journal ◽

10.1177/1055665617754460 ◽

2018 ◽

Vol 55 (5) ◽

pp. 721-727 ◽

Cited By ~ 1

Author(s):

Kevin J. Robertson ◽

Bernardino M. Mendez ◽

William J. Bruce ◽

Brendan D. McDonnell ◽

Michael V. Chiodo ◽

...

Keyword(s):

Outcome Measure ◽

Clockwise Rotation ◽

Le Fort ◽

Main Outcome Measure ◽

Anterior Nasal Spine ◽

Le Fort Iii ◽

Lateral Cephalograms ◽

Group 2 ◽

Internal Distraction ◽

Group 1

Objective: This study compares the change in midface position following Le Fort III advancement using either rigid external distraction (group 1) or internal distraction (group 2). We hypothesized that, with reference to right-facing cephalometry, internal distraction would result in increased clockwise rotation and inferior displacement of the midface. Design: Le Fort III osteotomies and standardized distraction protocols were performed on 10 cadaveric specimens per group. Right-facing lateral cephalograms were traced and compared across time points to determine change in position at points orbitale, anterior nasal spine (ANS), A-point, and angle ANB. Setting: Institutional. Patients, Participants: Twenty cadaveric head specimens. Interventions: Standard subcranial Le Fort III osteotomies were performed from a coronal approach and adequately mobilized. The specified distraction mechanism was applied and advanced by 15 mm. Main Outcome Measure(s): Changes of position were calculated at various skeletal landmarks: orbitale, ANS, A-point, and ANB. Results: Group 1 demonstrated relatively uniform x-axis advancement with minimal inferior repositioning at the A-point, ANS, and orbitale. Group 2 demonstrated marked variation in x-axis advancement among the 3 points, along with a significant inferior repositioning and clockwise rotation of the midface ( P < .0001). Conclusion: External distraction resulted in more uniform advancement of the midface, whereas internal distraction resulted in greater clockwise rotation and inferior displacement. External distraction appears to provide increased vector control of the midface, which is important in creating a customized distraction plan based on the patient’s individual occlusal and skeletal needs.

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