Pretzel syndrome

MeSH: C567020ORPHA: 500533

Overview

human disease

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 Pretzel syndrome, sourced from HPO and Orphanet clinical annotations.

MacrocephalySeizureHypotoniaGlobal developmental delayMegalencephalyPolyhydramniosAbnormal facial shapeInfantile spasmsVentriculomegalyCerebral white matter hypoplasiaPeriventricular white matter hyperintensitiesNephrocalcinosisWide mouthOpen mouthNarrow faceFacial hypotoniaHigh foreheadDiabetes insipidusAbsent speechJoint hypermobilityFailure to thriveAtrial septal defectCongestive heart failureStatus epilepticusDroolingFocal impaired awareness seizureHighly arched eyebrowDecreased muscle massSevere muscular hypotoniaTented upper lip vermilionInterictal epileptiform activitySevere global developmental delayFeeding difficultiesAbnormal cardiovascular system morphology

Classification & Codes

MeSH Code

C567020

Orphanet Code

ORPHA:500533
Pretzel syndrome
MeSHC567020
OrphanetORPHA:500533
Treatments0 drug(s)
Symptoms on record34 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO
Pretzel syndrome | OrphanDrug