arterial tortuosity syndrome

ICD-10: Q87.82MeSH: C565942ORPHA: 3342

Overview

connective tissue disease that is characterized by elongation and generalized tortuosity of the major arteries including the aorta

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with arterial tortuosity syndrome, sourced from HPO and Orphanet clinical annotations.

Congestive heart failureAortic root aneurysmVascular dilatationAortic aneurysmAbnormal carotid artery morphologyArterial stenosisTelangiectasia of the skinInguinal herniaLong faceHypertelorismMacrotiaThin skinHyperextensible skinCraniosynostosisAortic dissectionPulmonary artery stenosisAbnormal zygomatic bone morphologyFatigueFemoral herniaMacrocephalyMalar flatteningStrabismusMyopiaKeratoconusBlepharophimosisHypertensionKeratoglobusArachnodactylyIntellectual disabilityHypotoniaGlobal developmental delaySpecific learning disabilityHip dysplasiaRedundant skinAbnormal myocardium morphologyHypertrophic cardiomyopathyDilated cardiomyopathyMyocardial infarctionCardiac arrestRocker bottom footGastroesophageal refluxPyloric stenosisHiatus herniaDyspneaRespiratory distressScoliosisCoxa valgaCoxa varaHip dislocationRespiratory failureShort noseClinodactyly of the 5th fingerAvascular necrosis of the capital femoral epiphysisCardiorespiratory arrestPrematurely aged appearanceLong palmShort palpebral fissureMyocarditisEsophagitisJoint hypermobilityMedian cleft palate

Classification & Codes

ICD-10 Code

Q87.82

MeSH Code

C565942

Orphanet Code

ORPHA:3342
arterial tortuosity syndrome
ICD-10Q87.82
MeSHC565942
OrphanetORPHA:3342
Treatments0 drug(s)
Symptoms on record61 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO