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Progressive congenital scoliosis

MedGen UID:
347356
•Concept ID:
C1857025
•
Congenital Abnormality; Finding
Synonym: Congenital scoliosis, progressive
 
HPO: HP:0008458

Definition

A progressive form of scoliosis with congenital onset. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Progressive congenital scoliosis

Conditions with this feature

Ehlers-Danlos syndrome, kyphoscoliotic type 1
MedGen UID:
75672
•Concept ID:
C0268342
•
Disease or Syndrome
PLOD1-related kyphoscoliotic Ehlers-Danlos syndrome (PLOD1-kEDS) is characterized by hypotonia, generalized joint hypermobility, early-onset kyphoscoliosis, skin fragility, and ocular abnormality. Intelligence is normal. Life span may be normal, but affected individuals are at risk of life-threatening arterial ruptures and spontaneous dissections of medium-sized arteries. Adults with severe kyphoscoliosis are at risk for complications from restrictive lung disease, recurrent pneumonia, and cardiac failure.
See: Condition Record
Ehlers-Danlos syndrome, kyphoscoliotic type 1

Professional guidelines

PubMed

Treatment of children scoliosis by corrective brace with regulated force effect.
Culik J, Marik I, Cerny P
J Musculoskelet Neuronal Interact 2011 Jun;11(2):203-7. PMID: 21625057
Fusionless instrumentation systems for congenital scoliosis: expandable spinal rods and vertical expandable prosthetic titanium rib in the management of congenital spine deformities in the growing child.
Yazici M, Emans J
Spine (Phila Pa 1976) 2009 Aug 1;34(17):1800-7. doi: 10.1097/BRS.0b013e3181978ec9. PMID: 19644331

Recent clinical studies

Etiology

Safety and Efficacy of One-stage Spinal Osteotomy for Severe and Rigid Congenital Scoliosis Associated with Split Spinal Cord Malformation.
Chen B, Yuan Z, Chang MS, Huang JH, Li H, Yang WZ, Luo ZJ, Tao HR
Spine (Phila Pa 1976) 2015 Sep 15;40(18):E1005-13. doi: 10.1097/BRS.0000000000001039. PMID: 26165211
Serial derotational casting in congenital scoliosis as a time-buying strategy.
Demirkiran HG, Bekmez S, Celilov R, Ayvaz M, Dede O, Yazici M
J Pediatr Orthop 2015 Jan;35(1):43-9. doi: 10.1097/BPO.0000000000000229. PMID: 24887080
A retrospective cohort study of pulmonary function, radiographic measures, and quality of life in children with congenital scoliosis: an evaluation of patient outcomes after early spinal fusion.
Vitale MG, Matsumoto H, Bye MR, Gomez JA, Booker WA, Hyman JE, Roye DP Jr
Spine (Phila Pa 1976) 2008 May 15;33(11):1242-9. doi: 10.1097/BRS.0b013e3181714536. PMID: 18469699
The effect of opening wedge thoracostomy on thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis.
Campbell RM Jr, Smith MD, Mayes TC, Mangos JA, Willey-Courand DB, Kose N, Pinero RF, Alder ME, Duong HL, Surber JL
J Bone Joint Surg Am 2004 Aug;86(8):1659-74. doi: 10.2106/00004623-200408000-00009. PMID: 15292413
Progressive congenital scoliosis treatment using a transpedicular anterior and posterior convex hemiepiphysiodesis and hemiarthrodesis. A preliminary report.
Keller PM, Lindseth RE, DeRosa GP
Spine (Phila Pa 1976) 1994 Sep 1;19(17):1933-9. doi: 10.1097/00007632-199409000-00013. PMID: 7997926

Diagnosis

Thoracic spinal cord impingement by an arachnoid web at the level of a hemivertebra: case report.
Hubbard ME, Hunt MA, Jones KE, Polly DW
J Neurosurg Spine 2017 Dec;27(6):638-642. Epub 2017 Sep 8 doi: 10.3171/2017.5.SPINE161243. PMID: 28885124
Safety and Efficacy of One-stage Spinal Osteotomy for Severe and Rigid Congenital Scoliosis Associated with Split Spinal Cord Malformation.
Chen B, Yuan Z, Chang MS, Huang JH, Li H, Yang WZ, Luo ZJ, Tao HR
Spine (Phila Pa 1976) 2015 Sep 15;40(18):E1005-13. doi: 10.1097/BRS.0000000000001039. PMID: 26165211
Kyphoscoliotic type of Ehlers-Danlos Syndrome (EDS VIA) in six Egyptian patients presenting with a homogeneous clinical phenotype.
Abdalla EM, Rohrbach M, Bürer C, Kraenzlin M, El-Tayeby H, Elbelbesy MF, Nabil A, Giunta C
Eur J Pediatr 2015 Jan;174(1):105-12. Epub 2014 Oct 3 doi: 10.1007/s00431-014-2429-9. PMID: 25277362
Serial derotational casting in congenital scoliosis as a time-buying strategy.
Demirkiran HG, Bekmez S, Celilov R, Ayvaz M, Dede O, Yazici M
J Pediatr Orthop 2015 Jan;35(1):43-9. doi: 10.1097/BPO.0000000000000229. PMID: 24887080
Short anterior instrumented fusion and posterior convex non-instrumented fusion of hemivertebra for congenital scoliosis in very young children.
Garrido E, Tome-Bermejo F, Tucker SK, Noordeen HN, Morley TR
Eur Spine J 2008 Nov;17(11):1507-14. Epub 2008 Sep 27 doi: 10.1007/s00586-008-0760-4. PMID: 18820956Free PMC Article

Therapy

Safety and Efficacy of One-stage Spinal Osteotomy for Severe and Rigid Congenital Scoliosis Associated with Split Spinal Cord Malformation.
Chen B, Yuan Z, Chang MS, Huang JH, Li H, Yang WZ, Luo ZJ, Tao HR
Spine (Phila Pa 1976) 2015 Sep 15;40(18):E1005-13. doi: 10.1097/BRS.0000000000001039. PMID: 26165211
Fusionless instrumentation systems for congenital scoliosis: expandable spinal rods and vertical expandable prosthetic titanium rib in the management of congenital spine deformities in the growing child.
Yazici M, Emans J
Spine (Phila Pa 1976) 2009 Aug 1;34(17):1800-7. doi: 10.1097/BRS.0b013e3181978ec9. PMID: 19644331
The effect of mid-thoracic VEPTR opening wedge thoracostomy on cervical tilt associated with congenital thoracic scoliosis in patients with thoracic insufficiency syndrome.
Campbell RM Jr, Adcox BM, Smith MD, Simmons JW 3rd, Cofer BR, Inscore SC, Grohman C
Spine (Phila Pa 1976) 2007 Sep 15;32(20):2171-7. doi: 10.1097/BRS.0b013e31814b2d6c. PMID: 17873807
The effect of opening wedge thoracostomy on thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis.
Campbell RM Jr, Smith MD, Mayes TC, Mangos JA, Willey-Courand DB, Kose N, Pinero RF, Alder ME, Duong HL, Surber JL
J Bone Joint Surg Am 2004 Aug;86(8):1659-74. doi: 10.2106/00004623-200408000-00009. PMID: 15292413
The efficacy of convex hemiepiphysiodesis in patients with iatrogenic posterior element deficiency resulting from diastematomyelia excision.
Uzumcugil A, Cil A, Yazici M, Acaroglu E, Alanay A, Akalan N, Ozisik P, Surat A
Spine (Phila Pa 1976) 2003 Apr 15;28(8):799-805. PMID: 12698124

Prognosis

A retrospective cohort study of pulmonary function, radiographic measures, and quality of life in children with congenital scoliosis: an evaluation of patient outcomes after early spinal fusion.
Vitale MG, Matsumoto H, Bye MR, Gomez JA, Booker WA, Hyman JE, Roye DP Jr
Spine (Phila Pa 1976) 2008 May 15;33(11):1242-9. doi: 10.1097/BRS.0b013e3181714536. PMID: 18469699
Transpedicular hemiepiphysiodesis and posterior instrumentation as a treatment for congenital scoliosis.
Ginsburg G, Mulconrey DS, Browdy J
J Pediatr Orthop 2007 Jun;27(4):387-91. doi: 10.1097/01.bpb.0000271312.95621.b0. PMID: 17513957
The effect of opening wedge thoracostomy on thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis.
Campbell RM Jr, Smith MD, Mayes TC, Mangos JA, Willey-Courand DB, Kose N, Pinero RF, Alder ME, Duong HL, Surber JL
J Bone Joint Surg Am 2004 Aug;86(8):1659-74. doi: 10.2106/00004623-200408000-00009. PMID: 15292413
Congenital scoliosis with posterior spinal arthrodesis T2-L3 at age 3 years with 41-year follow-up. A case report.
Winter RB, Lonstein JE
Spine (Phila Pa 1976) 1999 Jan 15;24(2):194-7. doi: 10.1097/00007632-199901150-00023. PMID: 9926393
Progressive congenital scoliosis treatment using a transpedicular anterior and posterior convex hemiepiphysiodesis and hemiarthrodesis. A preliminary report.
Keller PM, Lindseth RE, DeRosa GP
Spine (Phila Pa 1976) 1994 Sep 1;19(17):1933-9. doi: 10.1097/00007632-199409000-00013. PMID: 7997926

Clinical prediction guides

Safety and Efficacy of One-stage Spinal Osteotomy for Severe and Rigid Congenital Scoliosis Associated with Split Spinal Cord Malformation.
Chen B, Yuan Z, Chang MS, Huang JH, Li H, Yang WZ, Luo ZJ, Tao HR
Spine (Phila Pa 1976) 2015 Sep 15;40(18):E1005-13. doi: 10.1097/BRS.0000000000001039. PMID: 26165211
A retrospective cohort study of pulmonary function, radiographic measures, and quality of life in children with congenital scoliosis: an evaluation of patient outcomes after early spinal fusion.
Vitale MG, Matsumoto H, Bye MR, Gomez JA, Booker WA, Hyman JE, Roye DP Jr
Spine (Phila Pa 1976) 2008 May 15;33(11):1242-9. doi: 10.1097/BRS.0b013e3181714536. PMID: 18469699
The effect of opening wedge thoracostomy on thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis.
Campbell RM Jr, Smith MD, Mayes TC, Mangos JA, Willey-Courand DB, Kose N, Pinero RF, Alder ME, Duong HL, Surber JL
J Bone Joint Surg Am 2004 Aug;86(8):1659-74. doi: 10.2106/00004623-200408000-00009. PMID: 15292413
Progressive congenital scoliosis treatment using a transpedicular anterior and posterior convex hemiepiphysiodesis and hemiarthrodesis. A preliminary report.
Keller PM, Lindseth RE, DeRosa GP
Spine (Phila Pa 1976) 1994 Sep 1;19(17):1933-9. doi: 10.1097/00007632-199409000-00013. PMID: 7997926
Convex anterior and posterior hemiarthrodesis and hemiepiphyseodesis in young children with progressive congenital scoliosis.
Winter RB
J Pediatr Orthop 1981;1(4):361-6. doi: 10.1097/01241398-198112000-00001. PMID: 7334113

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