First Successful Le Fort III Distraction in a Patient With Beare-Stevenson Cutis Gyrata Syndrome.

StudyThe Journal of craniofacial surgery· Jul 2026

First Successful Le Fort III Distraction in a Patient With Beare-Stevenson Cutis Gyrata Syndrome.

Nobuhiro Ando, Nobuyuki Mitsukawa, Minami Sasaki, Ryo Ando

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Pediatric NeurosurgeryStudyTreatmentQ2IF 1.1

Le Fort III distraction achieved about 25 mm midface advancement in Beare-Stevenson syndrome.

First Successful Le Fort III Distraction in a Patient With Beare-Stevenson Cutis Gyrata Syndrome.

Nobuhiro Ando … Ryo Ando

The Journal of craniofacial surgery · Jul 2026 · Q2 in Surgery

Purpose

Beare-Stevenson cutis gyrata syndrome (BSS) is a genetic disorder caused by mutations in the FGFR2 gene.

Methods

The patient was a 5-year-old girl who was diagnosed with BSS shortly after birth and underwent a tracheostomy because of respiratory distress.

~25 mm

Results

~25 mm

midface moved forward, easing eye bulge and improving bite

More results

Two uneventful years after surgery, the patient showed significant improvements in proptosis, occlusion, and facial profile, with an excellent clinical course.

DISCUSSION: BSS is an extremely rare condition presenting with severe midface hypoplasia, with only around 30 reported cases.

More results

Given the high incidence of Chiari malformations along with the characteristic craniofacial abnormalities, early intervention by both pediatric neurosurgeons and plastic surgeons is crucial.

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Conclusion

Given the high incidence of Chiari malformations along with the characteristic craniofacial abnormalities, early intervention by both pediatric neurosurgeons and plastic surgeons is crucial.

First Successful Le Fort III Distraction in a Patient With Beare-Stevenson Cutis Gyrata Syndrome.

First Successful Le Fort III Distraction in a Patient With Beare-Stevenson Cutis Gyrata Syndrome.

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