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PubMed · 2083460

[Biodegradable osteosynthesis applied to craniofacial surgery].

Abstract

Since skin problems as decubitus and infections are well known risks in osteoplastic trepanations in congenital malformations in children, we searched for a reduction in size of the implants and the possibility to use biodegradable materials. After design of special constructions regarding dimension and stability purposes for children below one year, we performed osteoplastic trepanations in 27 minipigs. In 78% the healing was uneventful and the osteoplasty remained in place whereas in 22% (= 6 animals) we observed a local infection which lead to loss of implants as well as cranioplasty in 3 animals. Based on this research, we used our biodegradable implants in osteoplastic trepanations of congenital craniostenosis in children. The postoperative follow-up is now from 3 to 6 months in 4 children and the results are perfect in all. We did not observe any local infection, neither cutaneous problems, even not in bilateral frontal advancement and floating forehead procedures. The implants are presented as well as the results in animal research and the first follow-up in 4 children.

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BibTeXRIS

O E Illi, U G Stauffer, H Sailer, P Beck. 1990. [Biodegradable osteosynthesis applied to craniofacial surgery].. https://pubmed.ncbi.nlm.nih.gov/2083460/

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Pfeiffer syndrome.

Pfeiffer syndrome is a rare autosomal dominantly inherited disorder that associates craniosynostosis, broad and deviated thumbs and big toes, and partial syndactyly on hands and feet. Hydrocephaly may be found occasionally, along with severe ocular proptosis, ankylosed elbows, abnormal viscera, and slow development. Based on the severity of the phenotype, Pfeiffer syndrome is divided into three clinical subtypes. Type 1 "classic" Pfeiffer syndrome involves individuals with mild manifestations including brachycephaly, midface hypoplasia and finger and toe abnormalities; it is associated with normal intelligence and generally good outcome. Type 2 consists of cloverleaf skull, extreme proptosis, finger and toe abnormalities, elbow ankylosis or synostosis, developmental delay and neurological complications. Type 3 is similar to type 2 but without a cloverleaf skull. Clinical overlap between the three types may occur. Pfeiffer syndrome affects about 1 in 100,000 individuals. The disorder can be caused by mutations in the fibroblast growth factor receptor genes FGFR-1 or FGFR-2. Pfeiffer syndrome can be diagnosed prenatally by sonography showing craniosynostosis, hypertelorism with proptosis, and broad thumb, or molecularly if it concerns a recurrence and the causative mutation was found. Molecular genetic testing is important to confirm the diagnosis. Management includes multiple-staged surgery of craniosynostosis. Midfacial surgery is performed to reduce the exophthalmos and the midfacial hypoplasia.

Acrocephalosyndactylia↗

Defending eponyms.

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Acrocephalosyndactylia↗