Distinguishing craniomorphometric characteristics and severity in metopic synostosis patients

Author(s):  
L. Chandler ◽  
K.E. Park ◽  
O. Allam ◽  
M.A. Mozaffari ◽  
S. Khetpal ◽  
...  
Keyword(s):  
1991 ◽  
Vol 2 (3) ◽  
pp. 621-627 ◽  
Author(s):  
Mark E. Shaffrey ◽  
John A. Persing ◽  
Johnny B. Delashaw ◽  
Christopher I. Shaffrey ◽  
John A. Jane

1994 ◽  
Vol 93 (1) ◽  
pp. 16-24 ◽  
Author(s):  
Jeffrey C. Posnick ◽  
Kant Y. Lin ◽  
Phillip Chen ◽  
Derek Armstrong
Keyword(s):  

2013 ◽  
Vol 29 (12) ◽  
pp. 2165-2170 ◽  
Author(s):  
Pinar Karabagli
Keyword(s):  

2014 ◽  
Vol 67 (7) ◽  
pp. 900-905 ◽  
Author(s):  
Harib H. Ezaldein ◽  
Philipp Metzler ◽  
John A. Persing ◽  
Derek M. Steinbacher

2016 ◽  
Vol 137 (5) ◽  
pp. 1539-1547 ◽  
Author(s):  
Kamlesh B. Patel ◽  
Gary B. Skolnick ◽  
John B. Mulliken
Keyword(s):  

2016 ◽  
Vol 18 (3) ◽  
pp. 275-280 ◽  
Author(s):  
Joanna Y. Wang ◽  
Amir H. Dorafshar ◽  
Ann Liu ◽  
Mari L. Groves ◽  
Edward S. Ahn

OBJECTIVE Because the metopic suture normally fuses during infancy, there are varying degrees of severity in head shape abnormalities associated with premature fusion. A method for the objective and reproducible assessment of metopic synostosis is needed to guide management, as current methods are limited by their reliance on aesthetic markers. The object of this study was to describe the metopic index (MI), a simple anthropometric cranial measurement. The measurements can be obtained from CT scans and, more importantly, from palpable cranial landmarks, and the index provides a rapid tool for evaluating patients in both pre- and postoperative settings. METHODS High-resolution head CT scans obtained in 69 patients (age range 0–24 months) diagnosed with metopic craniosynostosis were retrospectively reviewed. Preoperative 3D reconstructions were available in 15 cases, and these were compared with 3D reconstructions of 324 CT scans obtained in a control group of 316 infants (age range 0–24 months) who did not have any condition that might affect head size or shape and also in a subset of this group, comprising 112 patients precisely matched to the craniosynostosis patients with respect to age and sex. Postoperative scans were available and reviewed in 9 of the craniosynostosis patients at a mean time of 7.1 months after surgical repair. 3D reconstructions of these scans were matched with controls based upon age and sex. RESULTS The mean preoperative MI for patients with trigonocephaly was 0.48 (SD 0.05), significantly lower than the mean values of 0.57 (SD 0.04) calculated on the basis of all 324 scans obtained in controls (p < 0.001) and 0.58 (SD 0.04) for the subset of 112 age- and sex-matched controls (p < 0.001). For 7 patients with both pre- and postoperative CT scans available for evaluation, the mean postoperative MI was 0.55 (SD 0.03), significantly greater than their preoperative MIs (mean 0.48 [SD 0.04], p = 0.001) and comparable to the mean MI of the controls (p = 0.30). In 4 patients, clinically obtained postoperative MIs by caliper measurement were comparable to measurements derived from CT (p = 0.141). CONCLUSIONS The MI is a useful measurement of the severity of trigonocephaly in patients with metopic synostosis. This simple quantitative assessment can potentially be used in the clinical setting to guide preoperative evaluation, surgical repair, and postoperative degree of correction.


2003 ◽  
Vol 51 (2) ◽  
pp. 167-172 ◽  
Author(s):  
Keith T. Paige ◽  
Steven R. Cohen ◽  
Catherine Simms ◽  
Fernando D. Burstein ◽  
Roger Hudgins ◽  
...  

2014 ◽  
Vol 25 (1) ◽  
pp. 262-266 ◽  
Author(s):  
Giovanni Maltese ◽  
Peter Tarnow ◽  
Emma Wikberg ◽  
Peter Bernhardt ◽  
Jakob Heydorn Lagerlöf ◽  
...  

2008 ◽  
Vol 45 (1) ◽  
pp. 101-104 ◽  
Author(s):  
Corstiaan C. Breugem ◽  
Donald F. Fitzpatrick ◽  
Cynthia Verchere

Apert syndrome results almost exclusively from one of two point mutations (Ser252Trp or Pro253Arg) in fibroblast growth factor receptor 2. Most patients with Apert syndrome have this as an autosomal dominant abnormality. The majority of cases are sporadic, resulting from new mutations. Although there have been some descriptions of familial Apert syndrome, we could find only one previous description in the English literature about twinning in Apert syndrome. This report demonstrates monozygotic twins affected by Apert syndrome with both boys having the Ser252Trp mutation. Although the general constellation of clinical findings was characteristic for Apert syndrome, this case report is unique since the twins had different craniofacial and hand features. One of our twins had a metopic synostosis while Apert syndrome is often characterized by the large metopic suture that closes much later when compared to normal children.


2015 ◽  
Vol 38 (5) ◽  
pp. E4 ◽  
Author(s):  
Shane K. F. Seal ◽  
Paul Steinbok ◽  
Douglas J. Courtemanche

OBJECT Current craniosynostosis procedures can result in complications due to absorbable plates and screws or other specialized expensive hardware. The authors propose the cranial orbital buttress (COB) technique of frontoorbital remodeling for metopic and unicoronal synostoses, wherein no plates or screws are used. They hypothesize that, with this technique, aesthetically acceptable outcomes for unicoronal and metopic synostosis can be achieved. In this article, they present this technique and compare the results with current frontoorbital remodeling practices. METHODS The authors conducted a retrospective chart review of cases in which patients with nonsyndromic unicoronal or metopic synostosis underwent cranio-orbital surgery at their institution from 1985 through 2009. Operative parameters, surgical variations, and complications were analyzed. The COB technique uses a 1-piece switch, hemiforeheads, or multiple pieces for forehead remodeling. The supraorbital bar is reconstructed in patients with metopic synostosis using a double wedge or greenstick fracture technique, and in patients with unicoronal synostosis a hinge procedure based on a 1.5-orbital osteotomy is used. The supraorbital bar is advanced and supported in place by bone graft(s) inserted at the lateral aspect(s) of the orbit(s) to form a buttress, with fixation done using absorbable sutures. RESULTS A total of 79 cases met the criteria for inclusion in the study. Twenty-nine patients had metopic synostosis, 3 had combined metopic and sagittal synostoses, and 47 had unicoronal synostosis. The patients’ mean age at surgery was 11.4 ± 10.1 months and the mean operative time was 183.4 ± 41.0 minutes. The mean length of hospital stay was 3.7 ± 1.2 days. The mean blood loss was 150.0 ± 125.6 ml, and 33% of patients required a blood transfusion (mean volume 206.9 ± 102.3 ml). In metopic synostosis, hemiforeheads were used most often (24/29, 83%), and the supraorbital bar was remodeled using a bilateral intracranial orbital osteotomy followed by a double wedge modification (23/29, 79%) or a greenstick fracture (4/29 14%) for milder cases. Forehead remodeling for unicoronal synostosis was by a forehead switch (39/47, 83%) and the supraorbital bar was remodeled using a 1.5-orbital intracranial orbital osteotomy (34/47, 72%) such that the bar was advanced on the abnormal side and hinged at the midline of the normal orbit. Perioperative complications occurred in 19% of cases and included dural tears (16%), inconsequential subdural hematoma (1.3%), and nasal greenstick fracture (1.3%). The total reoperation rate was 7.6% (cranioplasties for irregular contours, 6.3%; scar revision, 1.3%). CONCLUSIONS The COB remodeling technique is simple and efficient, gives acceptable outcomes, and is less resource intensive than previous techniques reported in the literature.


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