Surgical Treatment of Cervical Kyphosis in Larsen Syndrome

Sakaura, Hironobu; Matsuoka, Takashi; Iwasaki, Motoki; Yonenobu, Kazuo; Yoshikawa, Hiroyuki

doi:10.1097/01.brs.0000250103.88392.8e

Cited by 44 publications

(25 citation statements)

References 10 publications

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“…However none of our patients had the “dish face” appearance, nor the typical congenital femora and tibiae anterior dislocation, or congenital hip dislocation. Cervical kyphosis and thoracolumbar scoliosis were not found in our series either [Sakaura et al, 1976]. Furthermore, patients with Larsen syndrome do not have marked learning disabilities.…”

Section: Discussionmentioning

confidence: 69%

A newly recognized syndrome with characteristic facial features, skeletal dysplasia, and developmental delay

Baratela

Bober

Tiller

et al. 2012

American J of Med Genetics Pt A

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We describe a series of seven male patients from six different families with skeletal dysplasia, characteristic facial features, and developmental delay. Skeletal findings include patellar dislocation, short tubular bones, mild metaphyseal changes, brachymetacarpalia with stub thumbs, short femoral necks, shallow acetabular roofs, and platyspondyly. Facial features include: a flattened midface with broad nasal bridge, cleft palate or bifid uvula and synophrys. All of the patients demonstrated pre-school onset of a cognitive developmental delay with a shortened attention span. Some of the cognitive delay was masked by a warm and engaging personality. We posit that these individuals have a newly recognized syndrome characterized by the described features. There is some phenotypic overlap between these patients and Desbuquois dysplasia; however molecular testing demonstrated that this is a distinct disorder. Given the family information available for each patient, we are suspicious that the constellation of findings reported herein could be an X-linked recessive syndrome.

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Section: Discussionmentioning

confidence: 69%

A newly recognized syndrome with characteristic facial features, skeletal dysplasia, and developmental delay

Baratela

Bober

Tiller

et al. 2012

American J of Med Genetics Pt A

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“…It has been indicated that posterior spinal fusion provides spinal stability through permitting the growth of anterior spinal elements to spontaneously correct the kyphosis after surgery (4). However, the success rate of posterior spinal fusion is 66% in patients younger than 2 years old and 50% in those older (7,8). As a result, recent studies recommend the posterior spinal fusion surgery along with anterior correction in patient older than 2-year-old (4, 9).…”

Section: Discussionmentioning

confidence: 99%

Management of Cervical Kyphosis in Larsen Syndrome: A Case Report

et al. 2016

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Introduction: Larsen syndrome is a congenital skeletal disorder manifested by several facial, ligamentous and spinal complications. Cervical kyphosis is one of the serious manifestations of the Larsen syndrome. However, there is no consensus regarding the best procedure of cervical kyphosis management in these patients. Case Presentation: A 1-year-old boy with the diagnosis of the Larsen syndrome was admitted to our hospital and undergone several corrective surgeries for knee, hip and foot deformities. At the age of 2 years, scoliosis was diagnosed and surgically managed. At the same time, cervical kyphosis was observed and monitored until the symptoms of neurological deficit due to cord compression led to the correction of cervical Kyphosis at the age of 4.5 years. Accordingly, an anterior/posterior (360 degree) cervical spinal fusion surgery was performed. Subsequently, cervicothoracic fusion was performed to correct cervicothoracic instability. No neurological complications were reported afterward. Conclusions: In spite of existing controversy around the best method of cervical kyphosis management in Larsen syndrome's patients older than 2-year old, anterior release and posterior fixation followed by anterior spinal fusion and strut grafting led to the satisfactory result in our case.

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“…Помимо изменений основания черепа, головного и спинного мозга, авторы описали комплекс вертебральных ано-малий, полностью соответствующий отмеченным и нами, -нарушения сегментации тел С 6 -С 7 и полное сме-щение С 7 кзади при блокированных остистых отростках шейных и груд-ных позвонков. Подобные анатоми-ческие варианты дислокации описаны при синдроме Ларсена у единичных пациентов [1][2][3].…”

Section: Discussionunclassified