Freeman-Sheldon syndrome: surgical correction of microstomia

Ferreira, Lydia Masako; Minami, Eliza; Andrews, Jorge de Moura

doi:10.1016/0007-1226(94)90056-6

Cited by 9 publications

(5 citation statements)

References 8 publications

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“…Patients must have early consultation with craniofacial and orthopedic surgeons, when craniofacial [21][22][23], clubfoot [24], or hand correction [25][26][27][28] is indicated to improve function or aesthetics. Operative measures should be pursued cautiously, with avoidance of radical measures and careful consideration of the abnormal muscle physiology in FSS.…”

Section: Discussionmentioning

confidence: 99%

Freeman-Sheldon Syndrome Presenting with Microstomia: A Case Report and Literature Review

Gurjar¹,

Parushetti²,

Gurjar³

2012

J. Maxillofac. Oral Surg.

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Freeman-Sheldon syndrome (FSS), as first described by Freeman and Sheldon in 1938, is a morphologically well-defined syndrome that results in a dysmorphic status combining bone anomalies and joint contractures with characteristic facies. It is part of the nosologic group of pathologies currently known as distal arthrogryposis as reported by Hall et al. (Am J Med Genet 11:185-239, 1982 [1]). It is a rare disorder and its exact prevalence is unknown. Our objective is to report a case of FSS presenting with microstomia and add a brief review of the literature for similar cases.

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Section: Discussionmentioning

confidence: 99%

Freeman-Sheldon Syndrome Presenting with Microstomia: A Case Report and Literature Review

Gurjar¹,

Parushetti²,

Gurjar³

2012

J. Maxillofac. Oral Surg.

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“…Because of the rarity of the condition, most of the information regarding treatment options for congenital microstomia has been obtained from treatment of facial burns and scleroderma patients (Hartford et al, 1975; Ortiz-Monasterio and Factor, 1980; Naylor et al, 1984; La Trenta et al, 1992). Congenital microstomia occurs in combination with syndromes such as Treacher Collins, Pierre Robin, or Freedman Sheldon (Guyuron and Winkler, 1988; Ferreira et al, 1994; Escoda-Francolí et al, 2009). Because of the complexity of the genetic abnormalities associated with these syndromes, the treatment of microstomia usually involves concomitant management with other genetic anomalies.…”

Section: Discussionmentioning

confidence: 99%

“…Most of the techniques that are used to correct microstomia involve a surgical release or some type of surgical flap to enlarge the stomal opening. Surgical correction has included a wide range of procedures such as commissuroplasties, commissurrotomies, z-plasties, and stair-step lengthening of the orbicularis oris (Pons and Bouhours, 1969; Guyuron and Winkler, 1988; Ferreira et al, 1994; Mehra et al, 1998; Langstein and Robb, 2005). Using the balloon technique to correct the microstomia will allow the native tissue to remain intact and possibly avoid the functional impairment and scarring associated with surgical treatments.…”

Section: Discussionmentioning

confidence: 99%

“…Because of the rarity of the condition, management of congenital microstomia stems mostly from experience in treating burn patients who develop microstomia. In the past, treatment options have included surgical methods such as commissurotomies and z-plasties as well as nonsurgical treatments using oral splints (Guyuron and Winkler, 1988; Ferreira et al, 1994). In severe microstomia, a surgical release may be required to permit splint placement (Heinle et al, 1988; Koymen et al, 2009).…”

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confidence: 99%

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A Novel Nonoperative Technique in the Initial Management and Treatment of Congenital Microstomia

Rezak

Bouvier

Olack

et al. 2012

The Cleft Palate Craniofacial Journal

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Congenital microstomia in the newborn can result in poor functional and aesthetic outcomes. In the past, treatment options have included surgical methods such as commissurotomies and z-plasties as well as nonsurgical treatments using oral splints. In severe microstomia, a surgical release may be required to permit splint placement. Because of the small diameter of the stoma, such surgical releases are not optimal and frequently must be repeated. We devised a nonoperative technique for the initial treatment of congenital microstomia that will slowly enlarge the stomal diameter without the need for surgical release. The patient is a newborn male born with multiple congenital anomalies including severe mandibular hypoplasia and retrusion, microstomia, and microglossia. The size of his oral cavity was less than 1 cm at birth with no clinical signs of a tongue. After initial dilation in the operating room with Hagar dilators, the oral stoma was serially dilated using Boston Scientific esophageal balloons. Once full dilation was achieved, we fabricated oral splints to continue the dilation process while maintaining oral competence with no surgical intervention.

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“…Previous authors reconstructed the vermillion deficit of the upper and lower lip using mucosal advancement flaps. 5 We felt that the upper lip is the aesthetically more important part of the mouth, and because of its projection is more visible, hence using lower lip vermillion in correction of upper lip vermillion deficit. With this procedure we widened the intercommissural distance from 18 to 35 mm.…”

mentioning

confidence: 98%