Surgical management of Gorham disease involving the upper cervical spine with occipito-cervical-thoracic fusion: a case report

Schell, Adam; Rhee, John M.; Allen, Abigail K; Andras, Lindsay M.; Zhou, Feifei

doi:10.1016/j.spinee.2016.02.020

Cited by 11 publications

(8 citation statements)

References 24 publications

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“…As reported both in our cases and in the literature (9,16,(23)(24)(25)(26)(27)(28)(29), and summarized in Table 5, spinal GSS can occur at any age and at any level from the cervical spine to the sacrum; however, the cervical and thoracic vertebrae are the most frequently involved regions. The early clinical manifestations of GSS are often subtle and nonspecific.…”

Section: Discussionsupporting

confidence: 72%

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Spinal Gorham-Stout syndrome: radiological changes and spinal deformities

Du¹,

Li²,

Xu³

et al. 2019

Quant. Imaging Med. Surg

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Background: Gorham-Stout syndrome (GSS) involving the spine is a rare clinical entity, and there is a paucity of comprehensive study on its radiological features. We aimed to present the radiological changes and spinal deformities in patients with spinal GSS. Methods: From January 2005 to December 2017, 11 consecutive GSS patients with spinal deformity were identified. Their medical records and imaging features were retrospectively reviewed. Computed tomography (CT) and magnetic resonance imaging (MRI) were used for a precise evaluation of spinal involvement. Posteroanterior and lateral standing radiographs were used to evaluate the spinal deformity. Results: CT showed multi-level generalized osteolytic lesions, with ill-defined fluid attenuation adjacent to the osseous changes. MRI demonstrated hyperintense signals on both T1-and T2-weighted images, while the unaffected segments showed normal signal intensity. Seven patients (63.6%) had a dominant feature of kyphosis, and 4 (36.4%) had scoliosis when spinal GSS was diagnosed. Kyphosis variably spanned from C7 to L1, averaged 94° (range, 53° to 158°), and was associated with sagittal imbalance in 4 cases. In our series, the apex of kyphosis and scoliosis coincided within the most seriously osteolytic segment. All patients received medication for GSS. Two adolescents taking Boston braces showed a relatively stable deformity. Four patients received long posterior spinal fusion, but two had fusion failure. Conclusions: CT and MRI investigations are important in the initial diagnosis and continued management for spinal GSS. A typical spinal deformity secondary to GSS presents as kyphosis or kyphoscoliosis, which is usually highly variable but highly concordant with osteolysis in terms of span and apex.

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Section: Discussionsupporting

confidence: 72%

“…Approximately 50 cases of GSS with involvements of the spine have been reported in the literature (9,16,17). However, the descriptions are mostly based on sporadic case reports.…”

Section: Introductionmentioning

confidence: 99%

Spinal Gorham-Stout syndrome: radiological changes and spinal deformities

Du¹,

Li²,

Xu³

et al. 2019

Quant. Imaging Med. Surg

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“…Aunque no existe un tratamiento efectivo y definitivo, la radioterapia, los medicamentos anti osteoclásticos (bifosfonatos) y el interferón α-2b, se han utilizado como opciones terapéuticas. Se ha descrito a la ripamicina como un nuevo posible agente terapéutico, actuando como inhibidor de la activación de las células T mediante el bloqueo de la transducción de señales intracelulares dependientes e independientes de calcio, el cual ha mostrado inhibir la linfangiogénesis (12,14,15).…”

Section: Discussionunclassified

Correlación clínico-radiológica como primera ruta en el diagnóstico de Síndrome de Gorham-Stout

Lozada-Martínez

Nieves

Bolaño-Romero

et al. 2020

CES Med

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El síndrome de Gorham-Stout constituye una rara enfermedad ósea cuya etiología sigue siendo desconocida, que se caracteriza por presentar osteólisis y proliferación anormal de canales vasculares. Se describe el caso de un paciente masculino de 47 años quien consultó por un cuadro de aproximadamente 15 días de evolución consistente en edema, dolor, eritema y secreción sero-hemática por herida quirúrgica en hombro derecho, con diagnóstico reciente de artritis séptica. Refería antecedente de síndrome de Gorham-Stout, evidenciándose al examen físico y radiografía comparativa de hombros. La presentación clínica de estos pacientes depende del área afectada y la extensión de la enfermedad, convirtiéndose su diagnóstico en un desafío en caso de que el deterioro se presente en zonas no expuestas. El principal síntoma consiste en dolor localizado, acompañado de hinchazón, debilidad y deterioro funcional de las extremidades afectadas. Estos pacientes pueden permanecer asintomáticos hasta sufrir una fractura ósea espontáneamente o después de un trauma menor.

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“…GSS may involve any part of the skeleton; however, the skull, shoulder and pelvis are the most commonly involved sites. Primary involvement of the spine is less common and has only been described in approximately 50 cases [8]. Chylothorax occurs when this disease invades the pleura or thoracic duct, and several reports describe an association between GSS and chylothorax [9, 10] Bilateral chylothorax is usually fatal and may lead to progressive respiratory failure, with a mortality rate increase to 53% [11].…”

Section: Discussionmentioning

confidence: 99%

A rare case of Gorham-stout syndrome involving the thoracic spine with progressive bilateral chylothorax: a case report

Wang

Liao

Cao

et al. 2019

BMC Musculoskelet Disord

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Background Gorham-Stout syndrome (GSS) with spinal involvement is extremely rare, and bilateral chylothorax as a complication is usually fatal. In our case, pleural effusion appeared in the left hemithorax after ligating the right thoracic duct. Case presentation A 14-year-old patient presented with GSS affecting the thoracic spine with bilateral chylothorax. The case was successfully managed using combined conservative and surgical treatments. At the 2-year follow-up visit, the amount of pleural fluid was reduced, the patient’s respiratory function had improved, and the deformity on the thoracic spine had gradually stabilized. Conclusions GSS is a rare disorder of the musculoskeletal system that responds poorly to therapies and exhibits very high morbidity and mortality. Chylothorax is a common complication when lesions involve the thoracic spine, and physicians should be vigilant for possible serious pulmonary complications.

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Surgical management of Gorham disease involving the upper cervical spine with occipito-cervical-thoracic fusion: a case report

Cited by 11 publications

References 24 publications

Spinal Gorham-Stout syndrome: radiological changes and spinal deformities

Spinal Gorham-Stout syndrome: radiological changes and spinal deformities

Correlación clínico-radiológica como primera ruta en el diagnóstico de Síndrome de Gorham-Stout

A rare case of Gorham-stout syndrome involving the thoracic spine with progressive bilateral chylothorax: a case report

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