Radiological Features of Scoliosis in Chiari I Malformation Without Syringomyelia

Zhu, Zezhang; Huang, Yan; Han, Xiao; Jin, Mengran; Xie, Dingding; Sha, Shifu; Liu, Zhen; Qian, Bangping; Zhu, Feng; Qiu, Yong

doi:10.1097/brs.0000000000001406

Cited by 18 publications

(8 citation statements)

References 20 publications

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“…As we know, scoliosis is commonly associated with Chiari-I-malformation with the concomitant rate from 15 to 50% [13]. Moreover, 10.5%~ 27.3% of Chiari-I-malformation patients with scoliosis do not have concurrent syringomyelia [14], just like in this case. The scoliosis may also be the result of KTS due to asymmetric growth of the spinal column.…”

Section: Discussionmentioning

confidence: 61%

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

Zhai

Zhong

Shen

et al. 2019

BMC Musculoskelet Disord

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BackgroundKlippel-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 presentationWe 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.ConclusionsKTS 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.

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Section: Discussionmentioning

confidence: 61%

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

Zhai

Zhong

Shen

et al. 2019

BMC Musculoskelet Disord

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“…With no KTS family history, they presented with hemihypertrophy, venous varicosities, and general malformations (two cases further shared hemangiomas, while one shared tonsil asymmetry and scoliosis) [1,7,13]. Scoliosis incidence with CIM ranges from 15 to 50% [12], and only 10.5% to 27.3% of those do not have syringomyelia [29]. It is not clear if our patient's scoliosis was caused by his Chiari I malformation, KTS itself or his leg length discrepancy and pelvic tilt.…”

Section: Discussionmentioning

confidence: 99%

Chiari I malformation with Klippel-Trenaunay syndrome: case report and review of the literature

Snee

Mazzola²,

Sikorskyj³

2021

Childs Nerv Syst

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We present a rare case of an 8-year-old male with Klippel-Trenaunay syndrome (KTS) and a Chiari I malformation (CIM). Magnetic resonance imaging (MRI) to investigate facial asymmetry and speech delay at age two revealed CIM with cerebellar tonsils 1.3 cm below the foramen magnum without syringomyelia. The patient underwent a craniectomy and posterior fossa decompression with C1 laminectomy. While gene sequencing determined the patient was negative for the PIK3CA gene mutation, the patient’s clinical history strongly suggests KTS. He has hemihypertrophy, leg length discrepancy, hemangiomas and pigmentary mosaicism along the upper and lower extremities, heart murmur, chronic low heart rate, recurrent hip pain, and mild scoliosis. Neurodevelopmental concerns include difficulty reading, attention deficit hyperactivity disorder (ADHD), anxiety, and difficulty running and going downstairs. His most recent MRI shows good decompression at the cervicomedullary junction, global cerebrospinal fluid (CSF) flow, and less peg-like cerebellar tonsils. Also noted were two intravertebral hemangiomas at T5 and T6. While the patient’s speech has improved, there is still difficulty with the expressive language. He still has mild delays, runs slowly, and does not alternate feet when climbing stairs. The patient is being followed by multiple specialists including neurology, hematology-oncology, genetics, orthopedic surgery, and developmental pediatrics.

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“…[24][25][26][27][28][29] The relationship between CM-I and scoliosis in the absence of syringomyelia is uncertain. Prior studies have found that scoliosis in the presence of CM-I without syringomyelia may still appear similar to CM-I with syringomyelia and respond similarly to PFD; 30,31 however, the opposite has also been observed. 10 It is therefore still important to assess the impact of treatment in patients with CM-I, scoliosis, and concurrent syringomyelia.…”

Section: Examination Of Cm-i Patients With Both Syrinx and Scoliosismentioning

confidence: 97%

Extradural decompression versus duraplasty in Chiari malformation type I with syrinx: outcomes on scoliosis from the Park-Reeves Syringomyelia Research Consortium

Sadler

Skidmore

Gewirtz

et al. 2021

Journal of Neurosurgery: Pediatrics

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OBJECTIVE Scoliosis is common in patients with Chiari malformation type I (CM-I)–associated syringomyelia. While it is known that treatment with posterior fossa decompression (PFD) may reduce the progression of scoliosis, it is unknown if decompression with duraplasty is superior to extradural decompression. METHODS A large multicenter retrospective and prospective registry of 1257 pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for patients with scoliosis who underwent PFD with or without duraplasty. RESULTS In total, 422 patients who underwent PFD had a clinical diagnosis of scoliosis. Of these patients, 346 underwent duraplasty, 51 received extradural decompression alone, and 25 were excluded because no data were available on the type of PFD. The mean clinical follow-up was 2.6 years. Overall, there was no difference in subsequent occurrence of fusion or proportion of patients with curve progression between those with and those without a duraplasty. However, after controlling for age, sex, preoperative curve magnitude, syrinx length, syrinx width, and holocord syrinx, extradural decompression was associated with curve progression > 10°, but not increased occurrence of fusion. Older age at PFD and larger preoperative curve magnitude were independently associated with subsequent occurrence of fusion. Greater syrinx reduction after PFD of either type was associated with decreased occurrence of fusion. CONCLUSIONS In patients with CM-I, syrinx, and scoliosis undergoing PFD, there was no difference in subsequent occurrence of surgical correction of scoliosis between those receiving a duraplasty and those with an extradural decompression. However, after controlling for preoperative factors including age, syrinx characteristics, and curve magnitude, patients treated with duraplasty were less likely to have curve progression than patients treated with extradural decompression. Further study is needed to evaluate the role of duraplasty in curve stabilization after PFD.

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Radiological Features of Scoliosis in Chiari I Malformation Without Syringomyelia

Abstract: 3.

Cited by 18 publications

References 20 publications

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

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

Chiari I malformation with Klippel-Trenaunay syndrome: case report and review of the literature

Extradural decompression versus duraplasty in Chiari malformation type I with syrinx: outcomes on scoliosis from the Park-Reeves Syringomyelia Research Consortium

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