Copenhagen disease

ORPHA: 2062

Overview

Progressive non-infectious anterior vertebral fusion (PAVF) is an early childhood spinal disorder characterized by the gradual onset of thoracic and/or lumbar spine ankylosis often in conjunction with kyphosis with distinctive radiological features

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 Copenhagen disease, sourced from HPO and Orphanet clinical annotations.

Wide mouthThin vermilion borderBrachycephalyRound faceHypertelorismShort philtrumMicrognathiaWide nasal bridgeDownslanted palpebral fissuresAbnormality of the vertebral columnThickened skinLarge handsScoliosisKyphosisHemivertebraeAbnormal intervertebral disk morphologyDepressed nasal bridgeFlat faceTall statureJoint stiffnessAbnormal facial shapeMorphological central nervous system abnormalityBone painSpinal rigidityAbdominal situs inversusProximal radio-ulnar synostosisExostoses

Classification & Codes

Orphanet Code

ORPHA:2062
Copenhagen disease
OrphanetORPHA:2062
Treatments0 drug(s)
Symptoms on record27 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO