A Clinical Study of Sotos Syndrome Patients With Review of the Literature

Leventopoulos, George; Kitsiou-Tzeli, Sophia; Kritikos, K.; Psoni, Stavroula; Mavrou, Ariadni; Kanavakis, Emmanuel; Fryssira, Helen

doi:10.1016/j.pediatrneurol.2008.11.013

Cited by 73 publications

(61 citation statements)

References 36 publications

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“…This overgrowth stabilizes between the ages of 2 and 6 years with head circumference and height above the 97 th percentile. Subsequently, the height tends to normalize by adulthood [1,2]. Facial appearance typically includes a high anterior hairline, macrocephaly, frontal bossing, a long thin face, frontotemporal hair scarcity, and downward slanting palpebral fissure.…”

mentioning

confidence: 99%

Anesthetic considerations in a child with Sotos syndrome -A case report-

et al. 2017

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confidence: 99%

Anesthetic considerations in a child with Sotos syndrome -A case report-

et al. 2017

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“…Hastamızın 3 aylıkken PDA nedeni ile opere edildiği anamnezinden öğrenildi. Kriptorşidizm, mesane divertikülü, hipoplastik böbrek, düşük derece vezikoüreteral reflü, fimozis, inguinal herni, otozomal polikistik böbrek hastalı-ğı, kronik böbrek yetmezliği de Sotos sendromuyla birlikteliği olabilen genitoüriner sistem anormallikleridir [2]. Otozomal dominant polikistik böbrek hastalığı olan Sotos sendromu da bildirilmiştir [8].…”

Section: Discussionunclassified

A rare cause of tall stature: Sotos syndrome

Aslan¹,

Sesli²,

Pirgon³

2014

Diclemedj

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Sotos syndrome is an excessive growth syndrome and is characterized by macrocephaly, typical facial appearance and mental retardation. The majority of cases are sporadic, autosomal dominant inheritance pattern matching families have been reported. Syndrome responsible for gen encodes the nuclear receptor-binding SET domain1 (NSD1) protein. This rare genetic syndrome firstly described by Sotos et al. in 1964 at five cases with excessive height, acromegalic appearance and mild mental retardation. Hairline high forehead, macrocephaly, frontal bossing, long and thin face, frontotemporal hair sparseness, down slanting palpebral fissures and prominent mandible creating characteristic facial appearance and advanced bone age and varying degrees of mental retardation are other diagnostic criteria. Cardiovascular, central nervous system and genitourinary system anomalies may be associated with syndrome. In this case report we presenting a case who admitted to our clinic because of the rapid growth and mild mental retardation and diagnosed with Sotos syndrome for emphasize the importance of growth monitoring.

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“…Sotos syndrome, also known as cerebral gigantism, is characterized by increased birth weight, excessive growth during the first 4 years of life, advanced bone age, and distinctive facial features, including dolichocephaly, hypertelorism, and prominent mandible [1][2][3]8) . With respect to maxillofacial features, dolichocephaly is almost always present, and increased mandibular length with pointed chin, highlyarched palate, and premature eruption of the deciduous teeth are observed in over 50% of such cases.…”

Section: Discussionmentioning

confidence: 99%

“…Patients may have attention deficit hyperactivity disorder, and intelligence can range from normal to severe mental retardation 8) . Surgery for scoliosis and intensive dental treatment under general anesthesia in such patients has previously been reported.…”

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confidence: 99%

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A Case of Sotos Syndrome Treated with Distraction Osteogenesis in Maxilla and Mandible

Takano

Kasahara

Ogawa

et al. 2012

Bull. Tokyo Dent. Coll.

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Sotos syndrome is inherited in an autosomal-dominant manner and is characterized by increased birth weight, excessive growth, advanced bone age, and distinctive facial features, including dolichocephaly, hypertelorism, and a prominent mandible. We treated a jaw deformity due to Sotos syndrome consisting of malocclusion due to a narrow maxillary dental arch and mandibular retrusion from hypoplasia of the rami. The patient was a 17-year-old man. Malocclusion due to a narrow maxillary dental arch and mandibular retrusion was diagnosed. Rapid maxillary expansion with Lines corticotomy and mandibular advancement with distraction osteogenesis were performed. The maxilla was expanded laterally a total of 3 mm and the mandible prolonged 12 mm in the posterior area of the mandibular body. Subsequently, orthodontic treatment was continued. At present, 5 years after surgery, occlusion remains good and stable.

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A Clinical Study of Sotos Syndrome Patients With Review of the Literature

Cited by 73 publications

References 36 publications

Anesthetic considerations in a child with Sotos syndrome -A case report-

Anesthetic considerations in a child with Sotos syndrome -A case report-

A rare cause of tall stature: Sotos syndrome

A Case of Sotos Syndrome Treated with Distraction Osteogenesis in Maxilla and Mandible

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