Binder syndrome: Clinical findings and surgical treatment of 18 patients at the Department of Plastic Surgery in Polanica Zdrój

Drozdowski, Piotr; Łątkowski, Ireneusz; Zachara, Mateusz; Wójcicki, Piotr

doi:10.17219/acem/62123

Cited by 7 publications

(8 citation statements)

References 23 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Though hand anomalies are rare, the incidence of brachydactyly and polydactyly has been reported 50 . Association of cleft lip and palate have been observed in a few cases 51 . Although no brain abnormalities have been associated with BS, the patients may present with mental retardation 52 .…”

Section: Resultsmentioning

confidence: 99%

See 1 more Smart Citation

Binder's syndrome: A narrative review

Siddiqui

Sennimalai

Samrit

et al. 2022

Special Care in Dentistry

View full text Add to dashboard Cite

The classical features of Binder's syndrome (BS) have been widely reported, yet there is a lack of information on diagnostic and treatment challenges. Therefore, we aimed to review the literature on various aspects, including the role of a multidisciplinary approach in the management of BS. Methods: A thorough literature search was performed on PubMed, Scopus, Embase, Ovid, Web of Science, EBSCOhost, and Google Scholar using the keywords"Maxillonasal dysplasia", "Maxillonasal dysostosis", "nasomaxillary hypoplasia", "Binder type", "Binder syndrome", "Binder phenotype", and "OMIM 155050". In addition, the articles published in the English language from the inception of the database until June 2021 were considered. Results:The search of different databases yielded 958 publications, out of which 145 relevant articles were reviewed. The studies were categorised by year of publication, study design, and theme. Most of the studies were case reports (42%) followed by case series (19%). The most reported topic was based on different surgical techniques (42%). Finally, the results were synthesised into a narrative review. Conclusions:The need for a multidisciplinary approach involving clinicians from different specialities is highlighted. Future research is warranted to develop concrete clinical guidelines for the management of this syndrome.

show abstract

Section: Resultsmentioning

confidence: 99%

“…50 Association of cleft lip and palate have been observed in a few cases. 51 Although no brain abnormalities have been associated with BS, the patients may present with mental retardation. 52 The sense of smell is usually not affected.…”

Section: Associated Anomaliesmentioning

confidence: 99%

Binder's syndrome: A narrative review

Siddiqui

Sennimalai

Samrit

et al. 2022

Special Care in Dentistry

View full text Add to dashboard Cite

show abstract

“…3,[14][15][16][17][18] Regarding the results of surgical treatment, 4 studies had a risk of bias; no objective method was used to evaluate treatment outcome. [18][19][20][21]…”

Section: Quality Assessment and Risk Of Biasmentioning

confidence: 99%

“…Most of the studies (12/14 studies) performed rhinoplasties to treat nasomaxillary dysplasia. Different surgical techniques were used depending on the authors: the rhinoplasty was associated with either a chondrocostal graft, 8,18,19,[22][23][24][25] a bony graft, 9,20,25,26 or an allogenic graft (silicone in 2 articles 19,23 and expanded polytetrafluoroethylene (ePTFE) implants in 1 article). 27 The 2 other articles evaluated maxillary osteotomy techniques (a modified LeFort II osteotomy 14 and LeFort I osteotomy).…”

Section: Surgical Options To Treat Nasomaxillary Dysplasiamentioning

confidence: 99%

Surgical Treatment for Patients With Binder Syndrome, Clinical Features and Associated Symptoms: A Systematic Review

Kerbrat

Ferri

2021

Journal of Craniofacial Surgery

View full text Add to dashboard Cite

Background: Binder syndrome is a rare congenital malformation of the midface. Since 1990, no systematic review has been conducted on this condition. Our study aims to review and collect the surgical treatment options available for correcting nasomaxillary dysplasia and the information available in the international literature on Binder syndrome and associated symptoms. Methods: This systematic literature review was registered in the PROSPERO (International Prospective Register of Systematic Reviews) database. Systematic literature searches were performed in Medline, Scopus, the Cochrane Library, and Web of Science databases. Study quality assessment was performed according to recommendations from the Cochrane Handbook for Systematic Reviews of Diagnostic Test Accuracy. Results: The authors identified 200 articles from our database research. One hundred sixty-nine articles were excluded after the title and abstract screening. Seven articles were excluded after screening the full text. In total, 24 clinical studies were included: 14 studies aim to describe a surgical option and 10 articles treat information about Binder syndrome or associated features. Most of the studies (12/14 studies) performed rhinoplasties to treat nasomaxillary dysplasia. The 2 other articles evaluated maxillary osteotomy techniques. Only 3 articles compared the results obtained from different surgical techniques. Conclusions: No optimal surgical treatment plan has been developed, even if numerous surgical options are available. Etiology is still uncertain.

show abstract

“…Severe intellectual disability has been reported with acrodysostosis but brain malformations, ataxia, and skin abnormalities are not reported features (Sezer, Sutbeyaz, Koseoglu, Aras, & Akin, 2009). There is also a limited group of syndromic entities, such the Sjögren-Larsson syndrome (OMIM #270200), that encompass ichthyosis, intellectual disability, short stature, spastic paraparesis, and dysmorphic features including midface hypoplasia (Drozdowski, Latkowski, Zachara, & Wojcicki, 2017;Dutra, de Aquino, & Barsottini, 2009).…”

mentioning

confidence: 99%

Novel progressive acrodysostosis‐like skeletal dysplasia, cerebellar atrophy, and ichthyosis

Velasco

Ullah

Martin

et al. 2020

American J of Med Genetics Pt A

View full text Add to dashboard Cite

Acrodysostosis refers to a rare heterogeneous group of bone dysplasias that share skeletal features, hormone resistance, and intellectual disability. Two genes have been associated with acrodysostosis with or without hormone resistance (PRKAR1A and PDE4D). Severe intellectual disability has been reported with acrodysostosis but brain malformations and ichthyosis have not been reported in these syndromes. Here we describe a female patient with acrodysostosis, intellectual disability, cerebellar hypoplasia, and lamellar ichthyosis. The patient has an evolving distinctive facial phenotype and childhood onset ataxia. X‐rays showed generalized osteopenia, shortening of middle and distal phalanges, and abnormal distal epiphysis of the ulna and radius. Brain magnetic resonance imaging showed cerebellar atrophy without other brainstem abnormalities. Genetic workup included nondiagnostic chromosomal microarray and skeletal dysplasia molecular panels. These clinical findings are different from any recognized form of acrodysostosis syndrome. Whole exome sequencing did not identify rare or predicted pathogenic variants in genes associated with known acrodysostosis, lamellar ichthyosis, and other overlapping disorders. A broader search for rare alleles absent in healthy population databases and controls identified two heterozygous truncating alleles in FBNL7 and PPM1M genes, and one missense allele in the NPEPPS gene. Identification of additional patients is required to delineate the mechanism of this unique disorder.

show abstract

Binder syndrome: Clinical findings and surgical treatment of 18 patients at the Department of Plastic Surgery in Polanica Zdrój

Cited by 7 publications

References 23 publications

Binder's syndrome: A narrative review

Binder's syndrome: A narrative review

Surgical Treatment for Patients With Binder Syndrome, Clinical Features and Associated Symptoms: A Systematic Review

Novel progressive acrodysostosis‐like skeletal dysplasia, cerebellar atrophy, and ichthyosis

Contact Info

Product

Resources

About