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

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.

Monobloc Le Fort III Distraction Osteogenesis for Correction of Severe Fronto-orbital and Midface Hypoplasia in Pediatric Crouzon Syndrome

2016 ◽  
Vol 53 (1) ◽  
pp. 118-125 ◽  
Author(s):  
Firdaus Hariri ◽  
Lim Kwong Cheung ◽  
Zainal Ariff Abdul Rahman ◽  
Vickneswaran Mathaneswaran ◽  
Dharmendra Ganesan
Keyword(s):  
Distraction Osteogenesis ◽  
Crouzon Syndrome ◽  
Le Fort ◽  
Midface Hypoplasia ◽  
Le Fort Iii

Endonasal Endoscopic Closure of Cerebrospinal Fluid Fistulas at the Anterior Cranial Base

1996 ◽  
Vol 105 (8) ◽  
pp. 620-623 ◽  
Author(s):  
Mislav Gjuric ◽  
Henning Keimer ◽  
Ulrich Goede ◽  
Malte Erik Wigand
Keyword(s):  
Cerebrospinal Fluid ◽  
Soft Tissue Injuries ◽  
Inferior Turbinate ◽  
Cranial Base ◽  
Csf Leak ◽  
Facial Soft Tissue ◽  
Csf Rhinorrhea ◽  
Endoscopic Closure ◽  
Anterior Cranial Base ◽  
Dural Tears

This study reports our indications and limits for endonasal endoscopic closure of dural defects with a cerebrospinal fluid (CSF) leak at the anterior cranial base, and demonstrates our surgical technique. Fifty-three patients with CSF rhinorrhea were reassessed for the success rate of closure of the CSF leak. Surgery was successful in 98%, and 68% of fistulas were closed endoscopically. A free graft of autogenous mucoperiosteum of the inferior turbinate was the most frequently used tissue for defect closure. The endonasal endoscopic route proved relatively safe for the closure of dural tears, irrespective of the cause, up to about 10 × 10 mm. It is characterized by minimal morbidity because of the preservation of sinus ventilation and bony structures, supraorbital nerves, and olfactory fibers. Defects larger in size, predominantly of traumatic origin, were closed via the transfacial approach. The decision on the surgical approach was additionally based on the extent of the facial soft tissue injuries and the localization of the leak.

Surgical Strategy for Cerebrospinal Fluid Rhinorrhea Repair

2010 ◽  
Vol 66 (suppl_2) ◽  
pp. ons281-ons286 ◽  
Author(s):  
Pinan Liu ◽  
Shengtian Wu ◽  
Zhi Li ◽  
Bo Wang
Keyword(s):  
Cerebrospinal Fluid ◽  
Nerve Injury ◽  
Frontal Sinus ◽  
Cranial Base ◽  
Neurological Deficits ◽  
Csf Rhinorrhea ◽  
Initial Surgery ◽  
Additional Surgery ◽  
Anterior Cranial Base ◽  
Sphenoid Sinuses

Abstract OBJECTIVE Cerebrospinal fluid (CSF) rhinorrhea is leakage of CSF from the nasal cavity caused by cranial base or meningeal defects. Surgical treatment of CSF rhinorrhea is still problematic. We evaluated the clinical outcomes of 132 consecutive cases of CSF rhinorrhea treated via transcranial or transnasal endoscopic approaches according to the patient's condition. The indications for the approaches are discussed. METHODS Of 132 patients with CSF rhinorrhea, a transnasal endoscopic approach was used in 98 to repair cranial base defects in the ethmoid and sphenoid sinuses. A transcranial intradural approach was used in the remaining 34 patients for frontal sinus defects, multiple fractures of the cranial base, or combination nerve injury. RESULTS CSF rhinorrhea resolved after initial surgery in 124 of 132 patients, giving a success rate of 94%. Of the 8 failures or recurrent cases, 4 were successfully repaired by repeat endoscopic surgery, 2 were cured by transcranial revision surgery, and 2 refused additional surgery (the condition subsequently resolved without treatment in these patients). Postoperative complications included intracranial infection (8 patients) and anosmia (1 patient). No neurological deficits were apparent over the 10-month mean follow-up period. CONCLUSION Transnasal endoscopic repair is a reliable method for CSF rhinorrhea patients whose fistulae are located in the ethmoid and sphenoid sinuses. The transcranial procedure should be the treatment of choice for patients with frontal sinus fracture, multiple or complex anterior cranial base fractures, or nerve injury. A satisfactory surgical outcome depends on exact diagnosis, proper operative approach, and the surgeon's skill and experience.

scholarly journals Loeys-Dietz and Shprintzen-Goldberg syndromes: analysis of TGF-β-opathies with craniofacial manifestations using an innovative multimodality method

2021 ◽  
pp. jmedgenet-2021-107695
Author(s):  
Konstantinia Almpani ◽  
Denise K. Liberton ◽  
Priyam Jani ◽  
Cyrus Keyvanfar ◽  
Rashmi Mishra ◽  
...  
Keyword(s):  
Transforming Growth Factor ◽  
Cranial Base ◽  
Temporomandibular Joint Disorders ◽  
Geometric Morphometric ◽  
Pharyngeal Airway ◽  
Anterior Cranial Base ◽  
Obstructive Sleep ◽  
Functional Features ◽  
Maxilla And Mandible ◽  
The Impact

BackgroundElevated transforming growth factor-beta (TGF-β) signalling has been implicated in the pathogenesis of Loeys-Dietz syndrome (LDS) and Shprintzen-Goldberg syndrome (SGS). In this study, we provide a qualitative and quantitative analysis of the craniofacial and functional features among the LDS subtypes and SGS.MethodsWe explore the variability within and across a cohort of 44 patients through deep clinical phenotyping, three-dimensional (3D) facial photo surface analysis, cephalometric and geometric morphometric analyses of cone-beam CT scans.ResultsThe most common craniofacial features detected in this cohort include mandibular retrognathism (84%), flat midface projection (84%), abnormal eye shape (73%), low-set ears (73%), abnormal nose (66%) and lip shape (64%), hypertelorism (41%) and a relatively high prevalence of nystagmus/strabismus (43%), temporomandibular joint disorders (38%) and obstructive sleep apnoea (23%). 3D cephalometric analysis demonstrated an increased cranial base angle with shortened anterior cranial base and underdevelopment of the maxilla and mandible, with evidence of a reduced pharyngeal airway in 55% of those analysed. Geometric morphometric analysis confirmed that the greatest craniofacial shape variation was among patients with LDS type 2, with distinct clustering of patients with SGS.ConclusionsThis comprehensive phenotypic approach identifies developmental abnormalities that segregate to mutation variants along the TGF-β signalling pathway, with a particularly severe phenotype associated with TGFBR2 and SKI mutations. Multimodality assessment of craniofacial anomalies objectively reveals the impact of mutations of the TGF-β pathway with perturbations associated with the cranium and cranial base with severe downstream effects on the orbit, maxilla and mandible with the resultant clinical phenotypes.

scholarly journals Early detection possibilities of obstructive sleep apnoea syndrome

2012 ◽  
Vol 140 (3-4) ◽  
pp. 159-163 ◽  
Author(s):  
Predrag Vucinic ◽  
Branka Vukic-Culafic ◽  
Stojan Ivic
Keyword(s):  
Clinical Features ◽  
Obstructive Sleep Apnoea ◽  
Cranial Base ◽  
Sleep Apnoea ◽  
Mouth Breathing ◽  
Base Length ◽  
Anterior Cranial Base ◽  
Obstructive Sleep ◽  
Apical Base ◽  
Maxillary Arch

Introduction. Obstructive sleep apnoea (OSA) syndrome represents a significant medical problem due to numerous consequences that may follow it. Objective. The aim of the study was to analyze morphology of the maxilla in children with mouth breathing, and to assess possible characteristics in persons with marked clinical features of OSA. Methods. The sample comprised of 60 examinees aged from 8-10 years, all mouth-breathers. The following X-ray cephalometric parameters were measured: angle of maxillary pragmatism, cranial base angle, angle between the palatal plane and the anterior cranial base, maxillary length, distance from the most prominent labial surface of the maxillary central incisor to NA line, angle between the axis of the upper maxillary incisor and NA line. Following parameters were obtained from the casts: anterior and posterior width of the maxillary arch, height of the maxillary arch, index of the palatal height, as well as the apical base length. Assessed values were then compared to the corresponding norms. Results. Compared to the corresponding norms, statistically significant lower values were determined for the following parameters of the sample: SpP/SN, AW, PW, AB. Conclusion. Analysis of the morphological characteristics of the maxilla in mouth breathing children showed characteristics also present in persons with marked clinical features of OSA syndrome, such as a narrow maxilla, insufficient apical base length, as well as the reduced angle of the palatal plane angle to the anterior cranial base. All these suggest a possible increased risk of developing OSA syndrome in children?s later age.

SURGICAL MANAGEMENT OF ANTERIOR CRANIAL BASE FRACTURES WITH CEREBROSPINAL FLUID FISTULAE

Neurosurgery ◽  
2008 ◽  
Vol 62 (2) ◽  
pp. 463-471 ◽  
Author(s):  
Martin Scholsem ◽  
Felix Scholtes ◽  
Frèderick Collignon ◽  
Pierre Robe ◽  
Annie Dubuisson ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Treatment Algorithm ◽  
Cranial Base ◽  
University Hospital ◽  
Csf Leak ◽  
Dural Tear ◽  
Csf Rhinorrhea ◽  
Csf Fistula ◽  
Associated Lesions ◽  
Anterior Cranial Base

Abstract OBJECTIVE The management of cerebrospinal fluid (CSF) fistulae after anterior cranial base fracture remains a surgical challenge. We reviewed our results in the repair of CSF fistulae complicating multiple anterior cranial base fractures via a combined intracranial extradural and intradural approach and describe a treatment algorithm derived from this experience. METHODS We retrospectively reviewed the files of 209 patients with an anterior cranial base fracture complicated by a CSF fistula who were admitted between 1980 and 2003 to Liège State University Hospital. Among those patients, 109 had a persistent CSF leak or radiological signs of an unhealed dural tear. All underwent the same surgical procedure, with combined extradural and intradural closure of the dural tear. RESULTS Of the 109 patients, 98 patients (90%) were cured after the first operation. Persistent postoperative CSF rhinorrhea occurred in 11 patients (10%), necessitating an early complementary surgery via a transsphenoidal approach (7 patients) or a second-look intracranial approach (4 patients). No postoperative neurological deterioration attributable to increasing frontocerebral edema occurred. During the mean follow-up period of 36 months, recurrence of CSF fistula was observed in five patients and required an additional surgical repair procedure. CONCLUSION The closure of CSF fistulae after an anterior cranial base fracture via a combined intracranial extradural and intradural approach, which allows the visualization and repair of the entire anterior base, is safe and effective. It is essentially indicated for patients with extensive bone defects in the cranial base, multiple fractures of the ethmoid bone and the posterior wall of the frontal sinus, cranial nerve involvement, associated lesions necessitating surgery such as intracranial hematomas, and post-traumatic intracranial infection. Rhinorrhea caused by a precisely located small tear may be treated with endoscopy.

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