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Pontell ME, Wagner CS, Reddy N, Salinero LK, Barrero CE, Taylor JA, Chen SSL, Swanson JW, Bartlett SP. Isolated Squamosal Synostosis: Defining the Phenotype. Ann Plast Surg 2025:00000637-990000000-00788. [PMID: 40388841 DOI: 10.1097/sap.0000000000004356] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 05/21/2025]
Abstract
ABSTRACT This study aims to characterize the clinical manifestations of isolated squamosal synostosis (ISS) and review the relevant published literature. Computed tomography imaging from 2008 to 2022 was reviewed to identify patients with ISS. Patients were reviewed for age at presentation, signs of intracranial pressure, and management. A systematic review was conducted of all studies on ISS from 1990 to 2023. Eighteen patients from our institution were included. Three unilateral cases were diagnosed at 1.4 ± 0.6 years. None had signs of intracranial hypertension nor required surgery. Of the 15 bilateral cases, 6 were completely fused and 9 were partially fused. Concern for intracranial hypertension was more frequent in patients with bilateral complete ISS (66%) than partial (22%). Vault remodeling was pursued in 5/6 patients with bilateral complete ISS and 4/9 patients with bilateral partial ISS. Systematic review yielded 13 cases of ISS. Four had unilateral ISS diagnosed at 5.2 ± 2.8 months. None had intracranial hypertension. Nine had bilateral ISS diagnosed at 31 ± 47.3 months. Three patients had signs of elevated intracranial pressure. ISS presentation seems to vary based on laterality. Unilateral ISS typically presents without intracranial hypertension. Bilateral ISS is often associated with intracranial hypertension, more so in cases of complete suture fusion. Bilateral ISS warrants close observation.
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Affiliation(s)
- Matthew E Pontell
- From the Division of Plastic, Reconstructive, & Oral Surgery, Children's Hospital of Philadelphia, Philadelphia, PA
| | - Connor S Wagner
- From the Division of Plastic, Reconstructive, & Oral Surgery, Children's Hospital of Philadelphia, Philadelphia, PA
| | - Neil Reddy
- From the Division of Plastic, Reconstructive, & Oral Surgery, Children's Hospital of Philadelphia, Philadelphia, PA
| | - Lauren K Salinero
- From the Division of Plastic, Reconstructive, & Oral Surgery, Children's Hospital of Philadelphia, Philadelphia, PA
| | - Carlos E Barrero
- From the Division of Plastic, Reconstructive, & Oral Surgery, Children's Hospital of Philadelphia, Philadelphia, PA
| | - Jesse A Taylor
- From the Division of Plastic, Reconstructive, & Oral Surgery, Children's Hospital of Philadelphia, Philadelphia, PA
| | - Shih-Shan Lang Chen
- Division of Neurosurgery, Children's Hospital of Philadelphia, Philadelphia, PA
| | - Jordan W Swanson
- From the Division of Plastic, Reconstructive, & Oral Surgery, Children's Hospital of Philadelphia, Philadelphia, PA
| | - Scott P Bartlett
- From the Division of Plastic, Reconstructive, & Oral Surgery, Children's Hospital of Philadelphia, Philadelphia, PA
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Chen S, Kleiven S, Thiblin I, Li X. Quantitative morphological analysis framework of infant cranial sutures and fontanelles based on CT images. J Anat 2024; 245:377-391. [PMID: 38720634 PMCID: PMC11306764 DOI: 10.1111/joa.14056] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/02/2023] [Revised: 04/16/2024] [Accepted: 04/22/2024] [Indexed: 08/09/2024] Open
Abstract
Characterizing the suture morphological variation is a crucial step to investigate the influence of sutures on infant head biomechanics. This study aimed to establish a comprehensive quantitative framework for accurately capturing the cranial suture and fontanelle morphologies in infants. A total of 69 CT scans of 2-4 month-old infant heads were segmented to identify semilandmarks at the borders of cranial sutures and fontanelles. Morphological characteristics, including length, width, sinuosity index (SI), and surface area, were measured. For this, an automatic method was developed to determine the junction points between sutures and fontanelles, and thin-plate-spline (TPS) was utilized for area calculation. Different dimensionality reduction methods were compared, including nonlinear and linear principal component analysis (PCA), as well as deep-learning-based variational autoencoder (VAE). Finally, the significance of various covariates was analyzed, and regression analysis was performed to establish a statistical model relating morphological parameters with global parameters. This study successfully developed a quantitative morphological framework and demonstrate its application in quantifying morphologies of infant sutures and fontanelles, which were shown to significantly relate to global parameters of cranial size, suture SI, and surface area for infants aged 2-4 months. The developed framework proved to be reliable and applicable in extracting infant suture morphology features from CT scans. The demonstrated application highlighted its potential to provide valuable insights into the morphologies of infant cranial sutures and fontanelles, aiding in the diagnosis of suture-related skull fractures. Infant suture, Infant fontanelle, Morphological variation, Morphology analysis framework, Statistical model.
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Affiliation(s)
- Siyuan Chen
- Division of Neuronic Engineering, Department of Biomedical Engineering and Health SystemsKTH – Royal Institute of TechnologyHuddingeSweden
| | - Svein Kleiven
- Division of Neuronic Engineering, Department of Biomedical Engineering and Health SystemsKTH – Royal Institute of TechnologyHuddingeSweden
| | - Ingemar Thiblin
- Forensic Medicine, Department of Surgical SciencesUppsala UniversityUppsalaSweden
| | - Xiaogai Li
- Division of Neuronic Engineering, Department of Biomedical Engineering and Health SystemsKTH – Royal Institute of TechnologyHuddingeSweden
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Fallahian F, Meyer A, Tadisina KK, Lin AY. Surgical Management in Isolated Squamosal Craniosynostosis: A Systematic Review. Ann Plast Surg 2023; 91:493-496. [PMID: 37553899 DOI: 10.1097/sap.0000000000003642] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 08/10/2023]
Abstract
BACKGROUND Although craniosynostoses involving the major sutures have been well described, the frequency of isolated minor suture craniosynostoses is much lower. Squamosal craniosynostosis (SQS) is a rare form of cranial synostosis, and the paucity of literature has made the creation of a standardized treatment plan difficult. We present a systematic review of the literature on isolated SQS to identify disease characteristics that lead to a need for operative intervention and to delineate patterns in surgical management. METHODS A systematic literature review was performed using the electronic databases of PubMed, Scopus, and MEDLINE and the key words "squamosal AND craniosynostosis," "squamous AND craniosynostosis," "squamosal craniosynostosis, "squamosal suture craniosynostosis," and "isolated squamosal craniosynostosis." Only human studies that described presentation and management of SQS were included. A blinded, 2-reviewer analysis of the articles was performed. Data collected included patient and disease characteristics, imaging workup, and treatment specifics, which were analyzed by descriptive statistics. RESULTS A total of 19 studies examining 119 patients with SQS were reviewed, with 97 (82%) multisutural cases and 22 isolated cases (18%). Of the isolated cases, 6 (27%) required surgical craniosynostosis repair, of which 1 (17%) had unilateral sutural involvement and 5 (83%) had bilateral involvement. Of the patients with isolated SQS, 7 (32%) had a congenital syndrome and comprised 33% of patients who required surgical intervention. The nonsyndromic patients with isolated SQS who required surgery presented with a wide array of phenotypic findings; 3 patients underwent some form of cranial vault remodeling, whereas 1 patient underwent ventriculoperitoneal shunt only. Of the 4 nonsyndromic patients with isolated SQS who underwent surgical repair, half required operative intervention because of elevated intracranial pressure and the other half because of dysmorphic head shape. CONCLUSION The findings of this updated systematic review suggest a trend toward surgical management in bilateral SQS versus unilateral SQS, and that patients with isolated SQS, previously considered to be a nonsurgical finding, should be carefully monitored, as there remains risk of increased intracranial pressure. Pooled systematic review data suggest isolated SQS has a 27% operative intervention rate, with the presence of coexisting syndromic diagnoses increasing that risk.
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Affiliation(s)
- Fedra Fallahian
- From the Department of Surgery, Saint Louis University School of Medicine, St Louis, MO
| | - Anne Meyer
- Department of Plastic Surgery, University of Kansas School of Medicine, Kansas City, KS
| | | | - Alexander Y Lin
- Division of Plastic Surgery, University of California San Francisco School of Medicine, San Francisco, CA
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Wilkinson CC, Belanger K, Elbadry R, Hoffman J, Ryan M, Stence NV, Graber SJ, Ridder T, Batista L, French BM. Fusion of Lateral Calvarial Sutures on Volume-Rendered Computed Tomography Reconstructions in Patients With Known Craniosynostosis. J Craniofac Surg 2023; 34:969-975. [PMID: 36939862 DOI: 10.1097/scs.0000000000009278] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/02/2022] [Accepted: 11/04/2022] [Indexed: 03/21/2023] Open
Abstract
INTRODUCTION After treating a child with familial sagittal craniosynostosis, clinocephaly, and bilateral parietomastoid/posterior squamosal suture fusion, the authors wondered if major-suture synostosis and clinocephaly were associated with abnormal fusion of minor lateral calvarial sutures. METHODS The authors reviewed all preoperative volume-rendered head computed tomography reconstructions performed for craniosynostosis at their institution from 2010 through 2014 and determined whether the sphenoparietal, squamosal, and parietomastoid sutures were open, partially fused, or fused. The authors determined whether any sutures were abnormally fused based upon a previous study from their center, in which abnormal fusion was defined as either 1 of 3 abnormal fusion patterns or abnormally-early fusion. The authors then determined the rate of abnormal fusion of these sutures and whether abnormal fusion was associated with (1) major-suture craniosynostosis, (2) type of craniosynostosis (sutures involved; single-suture versus multisuture; syndromic versus nonsyndromic), and (3) clinocephaly. RESULTS In 97 included children, minor lateral sutures were abnormally fused in 8, or 8.2%, which was significantly higher than in children without craniosynostosis from our earlier study. Abnormal minor lateral suture fusion was not associated with the type of single-suture synostosis or with multisuture synostosis but was associated with syndromic synostosis. Four of 8 children with abnormal minor lateral suture fusion had multisuture synostosis and 6 had syndromic synostosis. Lateral sutures were abnormally fused in 1 of 4 subjects with clinocephaly, which was not significant. CONCLUSION Abnormal minor lateral calvarial suture fusion is significantly associated with major-suture craniosynostosis, especially syndromic synostosis.
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Affiliation(s)
- C Corbett Wilkinson
- Department of Neurosurgery, Children's Hospital Colorado
- Department of Neurosurgery, University of Colorado School of Medicine
| | | | - Rasha Elbadry
- Department of Neurosurgery, Loma Linda University Health Care, Loma Linda, CA
| | - Jessa Hoffman
- Department of Neurosurgery, University of Colorado School of Medicine
| | - Megan Ryan
- Rocky Vista University College of Osteopathic Medicine, Parker
| | | | - Sarah J Graber
- Department of Neurosurgery, Children's Hospital Colorado
| | - Thomas Ridder
- Department of Neurosurgery, Children's Hospital Colorado
| | - Ligia Batista
- University of Colorado School of Medicine, Aurora, CO
| | - Brooke M French
- Division of Plastic Surgery, Children's Hospital Colorado, Aurora, CO
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Girard AO, Yang R. Management of Minor Suture Craniosynostosis. Oral Maxillofac Surg Clin North Am 2022; 34:435-442. [PMID: 35786531 DOI: 10.1016/j.coms.2022.02.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Abstract
Although most reported cases of minor suture involvement include multiple sutures, isolated suture involvement has been reported. Morphologic differences such as scaphocephaly and anterior plagiocephaly have been reported. Management should involve proper identification and multidisciplinary treatment. Surgical treatment should involve the expansion of the cranial vault as well as the correction of the deformity.
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Affiliation(s)
- Alisa O Girard
- Department of Plastic and Reconstructive Surgery, Johns Hopkins University, Baltimore, MD, USA
| | - Robin Yang
- Department of Plastic and Reconstructive Surgery, Johns Hopkins University, Baltimore, MD, USA; Department of Plastic and Reconstructive Surgery, Johns Hopkins Hospital, 601 North Caroline Street, Baltimore, MD 21231, USA.
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Park SY, Hwang HJ, Park KN. Development of a Cranial Suture Traction Therapy Program for Facial Asymmetry Correction Using the New Delphi Technique. Medicina (B Aires) 2022; 58:medicina58070869. [PMID: 35888588 PMCID: PMC9315778 DOI: 10.3390/medicina58070869] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/22/2022] [Revised: 06/16/2022] [Accepted: 06/24/2022] [Indexed: 11/26/2022] Open
Abstract
Background and Objectives: We aimed to develop a cranial suture traction therapy program, a non-surgical therapeutic method for facial asymmetry correction. Materials and Methods: Six experts, including rehabilitation medicine specialists, oriental medical doctors, dentistry specialists, five experts, including Master’s or doctoral degree holders in skin care and cosmetology with more than 10 years of experience in the field, 4 experts including educators in the field of skin care, a total of 15 people participated in the validation of the development of the cranial suture traction therapy program in stages 1 to 3. Open questions were used in the primary survey. In the second survey, the results of the first survey were summarized and the degree of agreement regarding the questions in each category was presented. In the third survey, the degree of agreement for each item in the questionnaire was analyzed statistically. Results: Most of the questions attained a certain level of consensus by the experts (average of ≥ 4.0). The difference between the mean values was the highest for the third survey at 0.33 and was the lowest between the second and third surveys at 0.47. The results regarding the perceived degree of importance for each point of the evaluation in both the second and third stages of the cranial suture traction therapy program were verified using the content validity ratio. The ratio for the 13 evaluation points was within the range of 0.40−1.00; thus, the Delphi program for cranial suture traction therapy verified that the content was valid. Conclusions: As most questions attained a certain level of consensus by the experts, it can be concluded that these questions are suitable, relevant, and important. The commercialization of the cranial suture traction treatment program will contribute to the correction and prevention of facial dislocations or asymmetry, and the developed treatment will be referred to as cranial suture traction therapy (CSTT).
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Affiliation(s)
- Seong-Yeon Park
- Majoring in Public Health, Department of Medicine, General Graduate School, Cha University, Seongnam-si 13503, Korea;
| | - Hea-Ju Hwang
- Majoring in Public Health, Department of Medicine, General Graduate School, Cha University, Seongnam-si 13503, Korea;
- Correspondence: (H.-J.H.); (K.-N.P.); Tel.: +82-010-5507-3953 (H.-J.H.); +82-010-5180-0751 (K.-N.P.)
| | - Kyu-Nam Park
- Majoring in Medical Beauty Industry, Graduate School of Public Health Industry, CHA University, Seongnam-si 13503, Korea
- Correspondence: (H.-J.H.); (K.-N.P.); Tel.: +82-010-5507-3953 (H.-J.H.); +82-010-5180-0751 (K.-N.P.)
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Deraje V, Tandon A. The significance or insignificance of prematurely fused bilateral squamosal sutures. JOURNAL OF CLEFT LIP PALATE AND CRANIOFACIAL ANOMALIES 2022. [DOI: 10.4103/jclpca.jclpca_28_21] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
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Chaisrisawadisuk S, Vatanavicharn N, Praphanphoj V, Anderson PJ, Moore MH. Bilateral squamosal synostosis: unusual presentation of chromosome 1p12–1p13.3 deletion. Illustrative case. JOURNAL OF NEUROSURGERY: CASE LESSONS 2021; 1:CASE20102. [PMID: 36034505 PMCID: PMC9394163 DOI: 10.3171/case20102] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 11/09/2020] [Accepted: 11/19/2020] [Indexed: 11/06/2022]
Abstract
BACKGROUNDSquamosal sutures are minor sutures of the human skull. Early isolated fusion of the sutures (squamosal synostosis) is rarely found.OBSERVATIONSThe authors report a case of a girl who presented with an abnormal head shape and bilateral squamosal synostosis. Genetic testing revealed a chromosome 1p12–1p13.3 deletion. She has been managed with conservative treatment of the synostosis. She has global developmental delay and multiple anomalies due to the chromosome abnormality.LESSONSIsolated squamosal suture synostosis could be an uncommon feature of chromosome 1p12–1p13.3 deletion.
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Affiliation(s)
- Sarut Chaisrisawadisuk
- Division of Plastic Surgery, Department of Surgery, and
- Cleft and Craniofacial South Australia, Women’s and Children’s Hospital, North Adelaide, South Australia, Australia; and
| | - Nithiwat Vatanavicharn
- Division of Medical Genetics, Department of Paediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand
| | | | - Peter J. Anderson
- Cleft and Craniofacial South Australia, Women’s and Children’s Hospital, North Adelaide, South Australia, Australia; and
- Faculty of Health and Medical Sciences, University of Adelaide, Adelaide, South Australia, Australia
| | - Mark H. Moore
- Cleft and Craniofacial South Australia, Women’s and Children’s Hospital, North Adelaide, South Australia, Australia; and
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Diab J, Anderson PJ, Moore MH. Late presenting bilateral squamosal synostosis. Arch Craniofac Surg 2020; 21:106-108. [PMID: 32380810 PMCID: PMC7206455 DOI: 10.7181/acfs.2019.00073] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/10/2020] [Accepted: 03/21/2020] [Indexed: 11/11/2022] Open
Abstract
Premature fusion of one or other of the minor sutures can subtly influence the shape of the human skull. Although infrequently reported or not clinically recognized, it can such contribute to a variety of craniofacial dysmorphisms. We herein report a case of late presenting, isolated bilateral synostosis of the squamosal suture dysmorphologies whose presentation mimics aspects of sagittal synostosis.
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Affiliation(s)
- Jason Diab
- Australian Craniofacial Unit, Adelaide, Australia
| | | | - Mark H Moore
- Australian Craniofacial Unit, Adelaide, Australia
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Cho DY, Evans KN, Weed MC, Lee A, Susarla SM. Bilateral Squamosal Suture Craniosynostosis Presenting with Abducens Nerve Palsy and Severe Papilledema. World Neurosurg 2020; 138:344-348. [PMID: 32217173 DOI: 10.1016/j.wneu.2020.03.079] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/17/2020] [Revised: 03/13/2020] [Accepted: 03/13/2020] [Indexed: 10/24/2022]
Abstract
BACKGROUND Patients with single-suture or minor suture craniosynostosis are typically asymptomatic at early presentation; intervention is aimed at reducing the risk of elevated intracranial pressure and associated developmental sequelae. Patients may be symptomatic in cases of major multisuture syndromic synostoses or delayed diagnosis. Clinical presentation in this context may include headaches, papilledema, cognitive delay, or behavioral issues. Cranial nerve palsies are atypical symptoms of intracranial hypertension in this patient population. CASE DESCRIPTION An 11-month-old, otherwise healthy girl presented with bilateral severe papilledema and left abducens nerve palsy owing to nonsyndromic near-complete bilateral squamosal suture synostosis with associated incomplete sagittal and right lambdoid synostoses. The patient underwent urgent open cranial expansion, with resolution of her papilledema and improvement in eye position and motility. CONCLUSIONS Cranial nerve palsies may be presenting symptoms of intracranial hypertension in patients with craniosynostosis. Multidisciplinary evaluation and treatment is paramount for appropriate management.
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Affiliation(s)
- Daniel Y Cho
- Division of Plastic Surgery, Department of Surgery, University of Washington School of Medicine, Seattle, USA
| | - Kelly N Evans
- Department of Pediatrics, University of Washington School of Medicine, Seattle, USA
| | | | - Amy Lee
- Department of Neurological Surgery, University of Washington School of Medicine, Seattle, USA
| | - Srinivas M Susarla
- Division of Plastic Surgery, Department of Surgery, University of Washington School of Medicine, Seattle, USA; Department of Oral and Maxillofacial Surgery, University of Washington School of Dentistry, Seattle, USA.
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What Is the Skull Structure Influence of Squamosal Suture Synostosis in Nonsyndromic and Syndromic Crouzon Craniosynostosis? J Craniofac Surg 2019; 30:1671-1675. [DOI: 10.1097/scs.0000000000005396] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
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Abstract
The squamosal suture is one of the lateral minor skull sutures, separating the parietal and squamous temporal bones. While the phenotypic appearances and sequelae of synostosis of the major cranial vault sutures are well documented, little is reported concerning synostosis of the squamosal suture (SQS). The aim of this study was to determine the frequency of squamosal suture synostosis, and to document the significance of this entity.A retrospective review of the diagnostic imaging for all new pediatric patients (aged ≤16 years) referred to the Oxford Craniofacial Unit between January 2008 and February 2013 was completed to identify patients with SQS. Computed tomography (CT) imaging was available in 422 patients and the axial and three-dimensional reconstructed images reviewed.Squamosal suture synostosis was confirmed in 38 patients (9%). It was present in conjunction with major suture synostosis in 33 patients and in isolation in 5. The incidence increased with age. It was more common in patients with syndromic craniosynostosis (18%) and associated syndromic conditions (36%) than in those with isolated major suture synostosis (6%). It was found to occur with coronal, lambdoid, and sagittal synostosis, but was most frequent with multisuture fusion patterns. Squamosal suture synostosis was not associated with a consistent calvarial deformity either in isolation or when associated with a major suture fusion. No patient underwent surgery specifically to correct SQS.In conclusion, contrary to previous reports, squamosal suture synostosis is a relatively frequent finding in the general case mix of a typical craniofacial unit, but is of limited clinical significance.
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Squamosal Craniosynostosis: Defining the Phenotype and Indications for Surgical Management. Ann Plast Surg 2017; 79:458-466. [PMID: 28953518 DOI: 10.1097/sap.0000000000001170] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
BACKGROUND Squamosal craniosynostosis is seldom reported in the craniofacial literature. Given that this is an uncommon diagnosis, phenotype and management remain unclear. The authors present a case series and review the literature to define the phenotype and management of these patients. METHODS We retrospectively reviewed 7 patients from our institution and systematically reviewed all published cases of squamosal craniosynostosis. Demographics, medical history, imaging, clinical presentation, subsequent workup, and treatment were examined and analyzed. RESULTS A comprehensive review of the literature yielded a total of 31 cases (including our new series) of squamosal craniosynostosis. Average age of presentation was 25.3 months, 52% of female patients, 74% of cases with bilateral squamosal involvement, 44% syndromic, 39% isolated squamosal (vs 61% multisutural). Overall, 56% of cases were handled surgically, whereas 44% were managed conservatively. Thirty-three percent of surgical cases required multiple operations. One patient with isolated bilateral squamosal craniosynostosis developed elevated intracranial pressure, requiring cranial vault remodeling. CONCLUSIONS Squamosal craniosynostosis frequently presents in a delayed fashion with nonsyndromic, bilateral involvement. In isolated bilateral squamosal cases, the associated phenotype is frontal prominence, occipital flattening, scaphocephalic tendency (low-end normocephalic cranial index), and superior parietal cornering. Evaluation of clinical signs and computed tomography imaging guides management, as evidence of increased intracranial pressure may indicate need for cranial vault expansion. Although previous literature suggests that nonsyndromic cases are nonsurgical, the majority of cases reviewed required surgical intervention, including our case of isolated bilateral squamosal craniosynostosis. We recommend vigilant management in patients with squamosal craniosynostosis, even those with isolated squamosal involvement.
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Squamosal Suture Synostosis: Incidence, Associations, and Implications for Treatment. J Craniofac Surg 2017; 28:1179-1184. [PMID: 28538065 DOI: 10.1097/scs.0000000000003603] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Abstract
Squamosal suture craniosynostosis is thought to be a relatively rare entity. In the authors' experience, it is underreported in imaging examinations and the existing literature. The authors sought to determine the incidence of squamosal synostosis, whether it is increasing in frequency, and its relationship with synostosis of the major calvarial sutures.Patients undergoing computed tomography imaging for suspected craniosynostosis over a 15-year period were reviewed by a plastic surgeon and pediatric neuroradiologist. Patients with synostosis of the squamosal sutures were identified and involvement of additional sutures, gender, and the presence of a known syndromic diagnosis were recorded. Patients greater than 4 years of age or those with prior craniofacial surgery were excluded.One hundred twenty-five patients met inclusion criteria, 26 of whom had squamosal suture synostosis (26/125, 20.8%). Squamosal synostosis was found in isolation in 3 patients (3/26, 11.5%), with 1 additional major suture in 10 patients (10/26, 38.5%), and ≥2 major sutures in 13 patients (13/26, 50%). Squamosal synostosis was more common in patients with a syndromic diagnosis (11/26 syndromic, 15/99 nonsyndromic, P < 0.001). Eleven of 26 patients with squamosal synostosis were identified in the radiology report (42.3%).Craniosynostosis of the squamosal suture is much more common than previously reported and can contribute to abnormal head shape in isolation, or in combination with major sutures. Squamosal suture synostosis is underdiagnosed clinically and radiologically, although insufficient evidence exists to determine if its true incidence is increasing.
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Unilateral Pterional Polycraniosynostosis Treated with Craniectomy and Helmet Therapy. PLASTIC AND RECONSTRUCTIVE SURGERY-GLOBAL OPEN 2017; 5:e1245. [PMID: 28280680 PMCID: PMC5340495 DOI: 10.1097/gox.0000000000001245] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/05/2016] [Accepted: 01/04/2017] [Indexed: 11/18/2022]
Abstract
Supplemental Digital Content is available in the text. Craniosynostosis is a condition in which one or more of the cranial sutures have fused prematurely, affecting the growth pattern and contours of the infant skull. The pterion is the junction of temporal, frontal, parietal, and sphenoid bones of the skull. We present a case of unilateral pterional craniosynostosis, which was treated with strip craniectomy and helmet therapy.
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Squamous Suture Synostosis: A Review With Emphasis on Cranial Morphology and Involvement of Other Cranial Sutures. J Craniofac Surg 2017; 28:51-55. [PMID: 27831981 DOI: 10.1097/scs.0000000000003184] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Abstract
Squamosal suture synostosis has received little attention, potentially due to its rare nature. The authors present here a clinical report of isolated unilateral squamosal suture synostosis and a literature review, which produced 6 articles describing 33 patients of squamosal synostosis.Of the reported patients, 15 were associated with a craniofacial syndrome, 10 were nonsyndromic, and 8 were not specified. The cranial morphology varied greatly and only 1 patient was consistent with the morphology predicted by Virchow law-decreased vertical growth with compensatory ipsilateral longitudinal growth (manifesting as occipital and possibly frontal zygomatic bulging). Additional suture synostoses were observed in 36.3% of nonsyndromic and 80% of syndromic patients, suggesting that either squamosal synostosis may have an effect on other sutures, or more likely, only the most severe patients are recognized and reported.Surgical and nonsurgical interventions have found limited utility due to the subtle nature of the cranial defects and a lack of increased intracranial pressure, with a conservative follow-up course being the preferred treatment.
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Chawla R, Alden TD, Bizhanova A, Kadakia R, Brickman W, Kopp PA. Squamosal Suture Craniosynostosis Due to Hyperthyroidism Caused by an Activating Thyrotropin Receptor Mutation (T632I). Thyroid 2015; 25:1167-72. [PMID: 26114856 DOI: 10.1089/thy.2014.0503] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/23/2022]
Abstract
BACKGROUND Congenital hyperthyroidism can be a cause of failure to thrive, hyperactivity, developmental delay, and craniosynostosis during infancy. Most commonly, the condition occurs in the setting of maternal autoimmune thyroid disease. Rarely, congenital hyperthyroidism can also occur secondary to activating mutations within the thyrotropin (TSH) receptor. PATIENT FINDINGS A Hispanic male infant presented at age 6 months with severe thyrotoxicosis. At the time of presentation he was being evaluated for squamosal suture synostosis and he was noted to have significant developmental delays. SUMMARY The patient's thyrotoxicosis was initially treated with antithyroid medication, and he subsequently underwent craniosynostosis repair leading to neurodevelopmental improvement. DNA from the patient and his parents was submitted for mutational analysis of exons 9 and 10 of the TSH receptor. He was found to carry a monoallelic transition 1895C>T in exon 10 that resulted in the substitution of threonine at position 632 by isoleucine (T32I). This mutation resulted in constitutive activation of the TSH receptor. Neither parent carried this mutation indicating that the child has acquired a de novo germline mutation. CONCLUSIONS We report the first case of squamosal suture craniosynostosis in a patient with non-autoimmune hyperthyroidism. Squamosal suture craniosynotosis is rare, often has a subtle presentation, and should be considered in all patients with this condition because prompt treatment of hyperthyroidism and craniosynotosis repair can lead to normalization of neurodevelopment.
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Affiliation(s)
- Reeti Chawla
- 1 Division of Pediatric Endocrinology, Phoenix Children's Hospital , Phoenix, Arizona
| | - Tord D Alden
- 2 Division of Neurosurgery, Ann and Robert H. Lurie Children's Hospital of Chicago , Northwestern University Feinberg School of Medicine, Chicago, Illinois
| | - Aigerim Bizhanova
- 3 Department of Biology, Harold Washington College , Chicago, Illinois
| | - Rachel Kadakia
- 4 Division of Pediatric Endocrinology, Ann and Robert H. Lurie Children's Hospital of Chicago , Northwestern University Feinberg School of Medicine, Chicago, Illinois
| | - Wendy Brickman
- 4 Division of Pediatric Endocrinology, Ann and Robert H. Lurie Children's Hospital of Chicago , Northwestern University Feinberg School of Medicine, Chicago, Illinois
| | - Peter A Kopp
- 5 Division of Endocrinology Metabolism and Molecular Medicine, Northwestern University Feinberg School of Medicine , Chicago, Illinois
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Tandon YK, Rubin M, Kahlifa M, Doumit G, Naffaa L. Bilateral squamosal suture synostosis: A rare form of isolated craniosynostosis in Crouzon syndrome. World J Radiol 2014; 6:507-510. [PMID: 25071892 PMCID: PMC4109103 DOI: 10.4329/wjr.v6.i7.507] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/05/2014] [Revised: 06/01/2014] [Accepted: 06/16/2014] [Indexed: 02/06/2023] Open
Abstract
Craniosynostosis is a pathologic condition which is characterized by the premature fusion of cranial sutures. It may occur alone or in association with other anomalies making up various syndromes. Crouzon syndrome is the most common craniosynostosis syndrome. Bicoronal sutures fusion is most commonly involved in Crouzon syndrome. There have only been a handful of cases of squamosal suture synostosis described in the surgery literature with the few ones described in Crouzon syndrome associated with other types of craniosynostosis. To the best of our knowledge, we are presenting the first case of isolated bilateral squamosal suture synostosis in a patient with Crouzon syndrome in a radiology journal with emphasis on its radiological appearance.
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Sauerhammer TM, Oh AK, Boyajian M, Magge SN, Myseros JS, Keating RF, Rogers GF. Isolated frontosphenoidal synostosis: a rare cause of synostotic frontal plagiocephaly. J Neurosurg Pediatr 2014; 13:553-8. [PMID: 24606403 DOI: 10.3171/2014.1.peds1378] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
OBJECT Unilateral fusion of the frontoparietal suture is the most common cause of synostotic frontal plagiocephaly. Localized fusion of the frontosphenoidal suture is rare but can lead to a similar, but subtly distinct, phenotype. METHODS A retrospective chart review of the authors' craniofacial database was performed. Patients with isolated frontosphenoidal synostosis on CT imaging were included. Demographic data, as well as the clinical and radiographic findings, were recorded. RESULTS Three patients were identified. All patients were female and none had an identifiable syndrome. Head circumference was normal in each patient. The mean age at presentation was 4.8 months (range 2.0-9.8 months); 2 fusions were on the right side. Frontal flattening and recession of the supraorbital rim on the fused side were consistent physical findings. No patient had appreciable facial angulation or orbital dystopia, and 2 patients had anterior displacement of the ipsilateral ear. All 3 patients were initially misdiagnosed with unilateral coronal synostosis, and CT imaging at a mean age of 5.4 months (range 2.1-10.8 months) was required to secure the correct diagnosis. Computed tomography findings included patency of the frontoparietal suture, minor to no anterior cranial base angulation, and vertical flattening of the orbit without sphenoid wing elevation on the fused side. One patient underwent CT scanning at 2.1 months of age, which demonstrated a narrow, but patent, frontosphenoidal suture. The patient's condition was assumed to be a deformational process, and she underwent 6 months of unsuccessful helmet therapy. A repeat CT scan obtained at 10.7 months of age demonstrated the synostosis. All 3 patients underwent fronto-orbital correction at mean age of 12.1 months (range 7.8-16.1 months). The mean duration of postoperative follow-up was 11.7 months (range 1.9-23.9 months). CONCLUSIONS Isolated frontosphenoidal synostosis should be considered in the differential diagnosis of atypical frontal plagiocephaly.
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Affiliation(s)
- Tina M Sauerhammer
- Division of Plastic and Reconstructive Surgery, Children's National Medical Center, Washington, DC
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Smartt JM, Gerety P, Taylor JA. Robotic Approaches to Palatoplasty and the Treatment of Velopharyngeal Dysfunction. Semin Plast Surg 2014; 28:32-4. [PMID: 26417207 DOI: 10.1055/s-0034-1368165] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/25/2022]
Abstract
The operative techniques used to address palatoplasty and velopharyngeal dysfunction rely on traditional methods of surgical exposure and tissue handling. As the role of robotic surgical systems has expanded, emphasis has shifted from extirpative to reconstructive applications. We discuss the possible role of surgical telemanipulation systems in the treatment of these diagnoses. Furthermore, we present a feasibility study that addresses a commonly performed treatment of velopharyngeal dysfunction-posterior pharyngeal flap (PPF). In brief, PPFs were successfully performed on a small series of cadaveric human specimens. The technical aspects of the procedure, including telemanipulator set-up, positioning, surgical instrumentation, and timing are described in detail. All cadavers underwent successful performance of PPFs. Operative times were within an acceptable range and use of the robotic system demonstrated a steep learning curve. Many of the potential advantages and costs associated with robotic surgical systems are discussed.
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Affiliation(s)
- James M Smartt
- Department of Plastic Surgery, University of Texas Southwestern and Children's Medical Center, Dallas, Texas
| | - Patrick Gerety
- Division of Plastic Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
| | - Jesse A Taylor
- Division of Plastic Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
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