Kyphoscoliosis with Klippel-Trenaunay syndrome: a case report and literature review

Autor: Jiliang Zhai, Min-Er Zhong, Jianxiong Shen, Haining Tan, Zheng Li
Jazyk: angličtina
Rok vydání: 2019
Předmět:
Zdroj: BMC Musculoskeletal Disorders, Vol 20, Iss 1, Pp 1-7 (2019)
Druh dokumentu: article
ISSN: 1471-2474
DOI: 10.1186/s12891-018-2393-z
Popis: Abstract Background Klippel-Trenaunay syndrome (KTS) is a rare congenital syndrome characterized by the triad of venous varicosities, capillary malformations and limb hypertrophy. However, KTS may rarely occur in combination with kyphoscoliosis. Case presentation We presented an 18-year-old female with KTS and kyphoscoliosis. Hypertrophy of bone and soft tissue affected her left face, trunk and lower limb. Moreover, the patient is associated with subacute thyroiditis, vitamin D deficiency and iron deficiency anemia, high level of D-dimer, swollen tonsil, kyphoscoliosis and Chiari-I-malformation without syringomyelia. A posterior correction and spinal fusion from T10 to L5 levels were performed for this patient. The lumbar curve was corrected from 105° to 60° and the kyphosis improved from 58° to 26°. The distance of trunk shift decreased from 10 cm to 1.4 cm. There were no thrombotic events occurred. At the 8th month follow-up, there was no significantly change of the curve in the coronal and sagittal radiographs. During the 31-month follow-up, the patient did not experience any discomfort. And her general appearance did not have any change until the last follow-up. However, she refused to take radiograph for worrying about radiation. Conclusions KTS is a rare disease with classic clinical triad. However, it can also have other different features, including kyphoscoliosis, elevated D-Dimer, vitamin D deficiency and iron-deficiency anemia. These issues should be taken into consideration when planning treatment for kyphoscoliosis in KTS patients.
Databáze: Directory of Open Access Journals
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