Butterfly Vertebrae: A Systematic Review of the Literature and Analysis

Abstract

Study Design: Systematic review (Level 4) Objective: To summarize the demographics, clinical presentations, and conditions associated with butterfly vertebrae. Methods: A systematic search was performed of multiple databases. A total of 279 articles were identified for screening. Case series or case reports of butterfly vertebrae with adequate clinical detail were complied. Results: Eighty-two total articles (109 patients) were selected for final inclusion. Sixty-one percent of patients presented with a single butterfly vertebra, while 39% were multiple. The most common location for butterfly vertebrae was T1. Fifty-six percent of cases were associated with a syndrome, the most common being spondylocostal dysostosis. The presence of multiple butterfly vertebra was strongly associated with a syndrome or additional anomalies ( P < .001). Overall, the most common presenting complaint was low back pain. Seventy percent of patients had associated spinal disease. Other organ systems affected included musculoskeletal (43%), craniofacial (30%), neurologic (27%), cardiovascular (24%), genitourinary (23%), gastrointestinal (22%), laboratory abnormality (16%), and endocrine (9%). Conclusions: This study is the largest collection of butterfly vertebrae cases to date. Butterfly vertebrae are associated with spinal deformity and multiple butterfly vertebrae may indicate a syndromic illness. Low back pain or disc herniation may occur with lumbar butterfly vertebrae however the etiology of this phenomena has not been rigorously explained. Many diseases and syndromes are associated with butterfly vertebrae.

Transformed notochordal cells trigger chronic wounds in zebrafish, destabilizing the vertebral column and bone homeostasis

“…Clefts through the centra and hemicentrae were found in 70% of fish analysed. These resembled butterfly abnormalities as occasionally described in human vertebral columns ( Katsuura and Kim, 2019 ), and malformations involving notochordal remnants ( Fig. 6 G‴) ( Oner et al, 2006 ).…”

Anatomy and clinical significance of sacral variations: a systematic review

“…Butterfly vertebra or sagittal cleft vertebra is a rare congenital abnormality, which is characterised by flawed convergence of the chondrification centres of the vertebral body during embryogenesis [49]. Very few cases of butterfly vertebra have been reported throughout international literature and only two cases refer to sacral vertebrae alone; Boulet et al [16] were the first to report a novel case of S1 sagittal cleft vertebra occurring with space narrowing at L5-S1 level, while Kapetanakis et al [47] described a case of S1 butterfly vertebra with dorsal projection of the hemivertebrae within the sacral canal, causing stenosis of the left lateral recess and compression of left S1 nerve root.…”

A Concomitant Occurrence of the Atlantoaxial Subluxation with Rare Vertebral Formation and Segmentation Defects

“…Due to this efficient load transmission, they are usually asymptomatic and do not require treatment [ 11 ]. Nonetheless, multiple previous reports have linked butterfly vertebrae with low back pain which might be the most common presenting complaint occurring in 23% of all cases and 30% of non-syndromic cases [ 6 ]. However, still, the association between butterfly vertebrae and low back pain remains unproven and might be unrelated to the structural abnormality; thus the etiology of this phenomena has not been rigorously explained.…”

“…According to a systematic review from Katsuura and Kim [ 6 ], there was a high association between the presence of multiple butterfly vertebrae and a syndromic presentation (40/45 cases, 89%). The associated organ system that is most frequently vulnerable would be any structural abnormality inside the vertebral column that is not affected with the butterfly vertebrae, such as scoliosis (most common), failure of formation and segmentation, block vertebrae, kyphosis, and spina bifida.…”