Perlman syndrome

MeSH: C536399ORPHA: 2849

Overview

syndrome characterized by polyhydramnios with neonatal macrosomia, nephromegaly, distinctive facial appearance, renal dysplasia, nephroblastomatosis, and predisposition to Wilms tumor. It shows similarities to Beckwith-Wiedemann syndrome

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 Perlman syndrome, sourced from HPO and Orphanet clinical annotations.

Tall statureAbnormal upper lip morphologyOpen mouthMacrocephalyRetrognathiaRound faceSmooth philtrumMicrognathiaHigh foreheadWide nasal bridgeDeeply set eyeIntellectual disabilityHypotoniaGlobal developmental delaySpecific learning disabilityHepatomegalyShort noseCryptorchidismBroad alveolar ridgesEpicanthusPosteriorly rotated earsLow-set earsThickened helicesAnteverted naresHyperinsulinemiaNephroblastomaHigh, narrow palateHypoplasia of penisAbnormal pancreas morphologyInguinal herniaDolichocephalyPtosisSeizureStatus epilepticusCapillary hemangiomaBilateral single transverse palmar creasesNaevus flammeus of the eyelidFemoral hernia

Classification & Codes

MeSH Code

C536399

Orphanet Code

ORPHA:2849
Perlman syndrome
MeSHC536399
OrphanetORPHA:2849
Treatments0 drug(s)
Symptoms on record38 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO