TY  - CHAP
M1  - Book, Section
TI  - Ectodermal Dysplasia, Ectrodactyly, and Macular Dystrophy (EEM) Syndrome
A1  - Bissonnette, Bruno
A1  - Luginbuehl, Igor
A1  - Engelhardt, Thomas
Y1  - 2019
N1  - 
T2  - Syndromes: Rapid Recognition and Perioperative Implications, 2e
AB  - EEM  is  an  acronym  that  stands  for  Ectodermal  dysplasia,  Ectrodactyly,  and  Macular  dystrophy  Syndrome.  This  genetic  disorder  affects  ectoderm  derivatives,  upper  and  lower  extremities,  and  retina.  The  clinical  features  include  hypotrichosis  with  sparse  and  short  hair  on  the  scalp,  sparse  and  short  eyebrows  and  eyelashes,  and  partial  anodontia.  The  hands  are  often  more  severely  affected  than  the  feet.  The  anomalies  vary  from  the  absence  of  digits  to  polydactyly,  syndactyly,  and  camptodactyly.  The  retinal  lesion  appears  as  a  central  geographic  atrophy  of  the  retinal  pigment  epithelium  and  choriocapillary  layer  of  the  macular  area  with  coarse  hyperpigmentation  and  sparing  of  the  larger  choroidal  vessels.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/18
UR  - accesspediatrics.mhmedical.com/content.aspx?aid=1164068675
ER  -