Congenital Scoliosis
Congenital scoliosis (CS) is scoliosis already formed by birth. It is a developmental anomaly most likely caused by an insult to an embryo (Mackel et al., 2018). It occurs in 1/1000 infants and in 10 percent of all people with scoliosis (Pahys and Guille, 2018). The deformities of CS are most likely due to environmental insults and result from failure of segmentation or failure of formation in developing embryos (Burnei et al, 2015; Mackel et al, 2018; Pahys and Guille, 2018). CS patients frequently have cardiac, neurological, urinogenital and musculoskeletal co-morbidities. (Burnei et al, 2015; Mackel et al, 2018; Pahys and Guille, 2018). Conservative treatments include serial casting and bracing (Mackel et al., 2016; Pahys and Guille, 2018; Wang, et al. 2019; Zhang Y, and Zhang, 2020). They focus on preventing secondary curve progression and post-surgical stability (Mackel et al., 2016; Pahys and Guille, 2018; Wang, et al. 2019; Zhang Y, and Zhang, 2020). Recent systematic review studies show that Schroth therapy effectively decreases Cobb angles in subjects with adolescent idiopathic scoliosis (AIS) (Parent, 2020). I didn’t find studies on the efficacy of Schroth for CS. However, Schroth was effective for CS patients I have seen in decreasing pain and by reversing a trunk shift.
There are two reasons for scoliosis formation in the developing embryo that result in scoliosis; failure of segmentation and failure of formation. Failure of segmentation occurs when two or more developing vertebrae don’t separate. They can remain remained fused only one side (unilateral bar) or completely fused (block vertebrae). Failure of formation occurs when one or both sides of the developing vertebrae don’t form correctly. This can result in the full development of the vertebrae but in a wedged shape or in a hemi vertebrae where one side of the vertebrae remains completely undeveloped (Pahys & Guille, 2018). Twenty percent of infants born with scoliosis have both segmental and formation failure abnormalities.
The most significant cause of these failures is thought to be damage to an embryo from external agents (Burnei et al, 2015; Mackel et al, 2018). Macke et al. and Burei et al. wrote that that some of these agents include carbon monoxide, maternal diabetes, hyperthermia, arsenic, alcohol, Vitamin A deficiency, I (Kr) blockers, and Valproic acid. Consequently, alcohol consumption, smoking, low intake of vitamin A and diabetes during pregnancy are risk factors for infants born with congenital scoliosis.
Congenital scoliosis patients commonly have cardiac, neurological, urinogenital and musculoskeletal co-morbidities (Burnei et al, 2015; Mackel et al, 2018; Pahys and Guille, 2018). According to Burnei and Pahys, they include VACTERL syndrome, ventricular septal defects, Fallot tetralogy, diastematomyelia, syringomyelia, Chiari malformation, tethered chord, intradural lipoma, horseshoe kidney, vesicoureteral reflux, hypospadias, and inguinal hernias, Sprengel’s deformity, Klippel-Feil syndrome, femoral hypoplasia, and acetabular dysplasia. Mackel et al. also reported that rib defects in CS patients that impede lung growth or normal respiration.
According to Burnei et al. (2015) 75 percent of infants with congenital scoliosis will need surgery and surgery is indicated in CS patients between 1 and 4 years of age.
Mackel et. al (2018) reported that children with curve Cobb angles more than 40-50 degrees, with rapidly increasing curves, or with unilateral bar defects require surgery.
Conservative treatment for most CS patients focuses on delaying spinal corrective surgery until lung development is complete and to avoid the need for multiple surgeries (Pahys and Guille, 2018; Wang, et al. 2019; Zhang Y, and Zhang, 2020). It can also prevent the progression of secondary curves and stabilize surgically corrected curves (Mackel et al., 2016). One conservative intervention for CS is serial casting (Zhang Y, and Zhang, 2020; Pahys and Guille, 2018). Zhang, Y. and Zhang wrote that although serial casting was the most successful conservative intervention for early onset scoliosis (EOS) there was little evidence that it was efficacious for CS (a subgroup of EO). Pahys and Guille, however, cited studies that demonstrated surgeries for CS subjects were delayed for over 2 years following serial casting.
Another conservative treatment is bracing (Wang, et al. 2019; Mackel et al., 2016). Mackel et al. wrote that bracing can prevent secondary curve progression and help to stabilize surgical corrections. Wang et al. demonstrated scoliosis curve Cobb angle curve decreases following bracing of at least 5 degrees in 51 percent of 39 subjects under 8 years of age who had CS, Risser skeletal maturity stage 0, and Cobb angles of 50 degrees or less.
Schroth therapy is a conservative treatment that may also stabilize post-surgical curves and prevent secondary curve progression. Although I was unable to find any research studies on the effect of Schroth therapy for IS, Parent (2020), cited 9 recent systematic review studies that demonstrated fair to good evidence that exercise decreased scoliosis curve Cobb angles in adolescents with idiopathic scoliosis (AIS). Of these studies, the US Preventative Task Force (2018), Day et al. (2019), and Fan et al.(2020) were specific to scoliosis specific exercises. Studies by Burger et al. (2019) and Park, J. Jeon and Park (2017) were specific to Schroth exercises. The ability of Schroth exercises to decrease curves Cobb angles in AIS may also apply to flexible secondary curves in patients with IS. My own experience in treating IS patients with Schroth therapy is a pain relief or resolution in adolescent and adult patients and the reversal of a trunk shift in a 15- year-old male patient.
CS scoliosis primarily results from environmental toxins that result in segmental or formation abnormalities in developing embryos. Surgery is required for many of these patients. Serial casting or bracing can delay surgery until the lungs are fully developed, stabilize surgical corrections, and prevent the progression of secondary curves. Schroth therapy effectively reduces scoliosis curve Cobb angles in many patients with AIS and may be useful to reduce secondary curve Cobb angles, stabilize surgical corrections and relieve pain in CS patients. Cardiac, neurological, urinogenital, and musculoskeletal co-morbidities are common in these patients and must be considered when treating them.
References
Burnei, G., Gavriliu, S., Vlad, C., Georgescu, I., Ghita, R. A., Dughilă, C., Japie, E. M., & Onilă, A. (2015). Congenital scoliosis: an up-to-date. Journal of medicine and life, 8(3), 388–397.
Burger, M., Coetzee, W., du Plessis, L. Z., Geldenhuys, L., Joubert, F., Myburgh, E., van Rooyen, C., & Vermeulen, N. (2019). The effectiveness of Schroth exercises in adolescents with idiopathic scoliosis: A systematic review and meta-analysis. The South African journal of physiotherapy, 75(1), 904. https://doi.org/10.4102/sajp.v75i1.904
Day, J. M., Fletcher, J., Coghlan, M., & Ravine, T. (2019). Review of scoliosis-specific exercise methods used to correct adolescent idiopathic scoliosis. Archives of physiotherapy, 9, 8. https://doi.org/10.1186/s40945-019-0060-9
Dunn, J., Henrikson, N. B., Morrison, C. C., Blasi, P. R., Nguyen, M., & Lin, J. S. (2018). Screening for Adolescent Idiopathic Scoliosis: Evidence Report and Systematic Review for the US Preventive Services Task Force. JAMA, 319(2), 173–187. https://doi.org/10.1001/jama.2017.11669
Fan, Y., Ren, Q., To, M., & Cheung, J. (2020). Effectiveness of scoliosis-specific exercises for alleviating adolescent idiopathic scoliosis: a systematic review. BMC musculoskeletal disorders, 21(1), 495. https://doi.org/10.1186/s12891-020-03517-6
Mackel, C. E., Jada, A., Samdani, A. F., Stephen, J. H., Bennett, J. T., Baaj, A. A., & Hwang, S. W. (2018). A comprehensive review of the diagnosis and management of congenital scoliosis. Child’s nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 34(11), 2155–2171. https://doi.org/10.1007/s00381-018-3915-6
Pahys, J. M., & Guille, J. T. (2018). What’s New in Congenital Scoliosis?. Journal of pediatric orthopedics, 38(3), e172–e179. https://doi.org/10.1097/BPO.0000000000000922
Parent, E. (2020, November 7) in Scoliosis Research Society and SOSORT Bracing and exercise therapy for all ages [Webinar]. SRS and SOSORT. https://www.srs.org/professionals/online-education-and-resources/webinars
Park, J. H., Jeon, H. S., & Park, H. W. (2018). Effects of the Schroth exercise on idiopathic scoliosis: a meta-analysis. European journal of physical and rehabilitation medicine, 54(3), 440–449. https://doi.org/10.23736/S1973-9087.17.04461-6
Wang Y, Feng Z, Wu Z, Qiu Y, Zhu Z, Xu L. Brace treatment can serve as a time-buying tactic for patients with congenital scoliosis. J Orthop Surg Res. 2019 Jun 27;14(1):194. doi: 10.1186/s13018-019-1244-4. PMID: 31248440; PMCID: PMC6598228.
Zhang, Y. B., & Zhang, J. G. (2020). Treatment of early-onset scoliosis: techniques, indications, and complications. Chinese medical journal, 133(3), 351–357. https://doi.org/10.1097/CM9.0000000000000614