Cervical Kyphosis Associated With Anteroposterior Dissociation and Quadriparesis in Larsenʼs Syndrome

Katz, Danielle A.; Hall, John E.; Emans, John B.

doi:10.1097/01.bpo.0000161091.85350.54

Cited by 23 publications

(13 citation statements)

References 11 publications

(12 reference statements)

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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

Вариант Нестабильного Кифозогенного Порока Позвоночника

Рябых¹,

Ulrikh²,

Губин³

et al. 2014

Хирургия позвоночника 1-2014

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Section: Discussionunclassified

Вариант Нестабильного Кифозогенного Порока Позвоночника

Рябых¹,

Ulrikh²,

Губин³

et al. 2014

Хирургия позвоночника 1-2014

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“…In fact, only 2 reported cases of pediatric OCT stabilization could be located in the literature, 4,9 both of which were in the setting of Larsen syndrome, a rare autosomal dominant disorder of growth hormone receptors resulting in joint hypermobility as well as extremity and spinal deformities. Generally, OCT fusion is reserved for severe deformity or highly aberrant pathological conditions necessitating total craniocervical stabilization to prevent neurological injury.…”

Section: Discussionmentioning

confidence: 99%

Occipitocervicothoracic stabilization in pediatric patients

Fargen

Anderson

Harter

et al. 2011

PED

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Object Although rarely encountered, pediatric patients with severe cervical spine deformities and instability may occasionally require occipitocervicothoracic instrumentation and fusion. This case series reports the experience of 4 pediatric centers in managing this condition. Occipitocervical fixation is the treatment of choice for craniocervical instability that is symptomatic or threatens neurological function. In children, the most common distal fixation level with modern techniques is C-2. Treated patients maintain a significant amount of neck motion due to the flexibility of the subaxial cervical spine. Distal fixation to the thoracic spine has been reported in adult case series. This procedure is to be avoided due to the morbidity of complete loss of head and neck motion. Unfortunately, in rare cases, the pathological condition or highly aberrant anatomy may require occipitocervical constructs to include the thoracic spine. Methods The authors identified 13 patients who underwent occipitocervicothoracic fixation. Demographic, radiological, and clinical data were gathered through retrospective review of patient records from 4 institutions. Results Patients ranged from 1 to 14 years of age. There were 7 girls and 6 boys. Diagnoses included Klippel-Feil, Larsen, Morquio, and VATER syndromes as well as postlaminectomy kyphosis and severe skeletal dysplasia. Four patients were neurologically intact and 9 had myelopathy. Five children were treated with preoperative traction prior to instrumentation; 5 underwent both anterior and posterior spinal reconstruction. Two patients underwent instrumentation beyond the thoracic spine. Allograft was used anteriorly, and autologous rib grafts were used in the majority for posterior arthrodesis. Follow-up ranged from 0 to 43 months. Computed tomography confirmed fusion in 9 patients; the remaining patients were lost to follow-up or had not undergone repeat imaging at the time of writing. Patients with myelopathy either improved or stabilized. One child had mild postoperative unilateral upper-extremity weakness, and a second child died due to a tracheostomy infection. All patients had severe movement restriction as expected. Conclusions Occipitocervicothoracic stabilization may be employed to stabilize and reconstruct complex pediatric spinal deformities. Neurological function can be maintained or improved. The long-term morbidity of loss of cervical motion remains to be elucidated.

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“…In 1950, Larsen et al described the first series of cases with distinctive facial features, multiple joint dislocations, and spinal anomalies. [1][2][3][4][5][6][7][8][9][10][11][12] There is also a risk of dramatic cervical instability and sudden neurological deficit and death, as reported by Larsen himself. Since 1950, several authors have reported similar findings and yet a consensus regarding timing of correction, surveillance, pre-and postoperative bracing, and even the preferred surgical approach is missing.…”

Section: Introductionmentioning

confidence: 99%

A 360-Degree Surgical Approach for Correction of Cervical Kyphosis and Atlantoaxial Dislocation in the Case of Larsen Syndrome

Deora

Singh

Sardhara

et al. 2020

JNRP

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Larsen syndrome is chronic debilitating disease that presents with multiple joint dislocations and severely affects the cervical spine in the form of cervical kyphosis and atlantoaxial dislocation. Children usually present in early with a myriad of deficits, compressive myelopathy being the most common. In addition to a bony compression, there is sometimes a soft tissue component, which is seldom addressed. We present here a case of atlantoaxial dislocation with cervical kyphosis due to Larsen syndrome, and along with our previous experience on syndromic atlantoaxial dislocations, we try to define an algorithm for the treatment approach of these onerous challenges. The importance of early intervention is also emphasized with a literature review of similar cases. In addition to the obvious physical damage, early intervention can also avoid the more sinister socioeconomic face of this debilitating disease. AbstractKeywords ► cervical kyphosis ► atlantoaxial dislocation ► Larsen syndrome

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Cervical Kyphosis Associated With Anteroposterior Dissociation and Quadriparesis in Larsenʼs Syndrome

Cited by 23 publications

References 11 publications

Вариант Нестабильного Кифозогенного Порока Позвоночника

Вариант Нестабильного Кифозогенного Порока Позвоночника

Occipitocervicothoracic stabilization in pediatric patients

A 360-Degree Surgical Approach for Correction of Cervical Kyphosis and Atlantoaxial Dislocation in the Case of Larsen Syndrome

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