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Primary congenital lymphedema: Milroy disease: the first case observed in the Department of Pediatrics at the University Hospital Yalgado Ouedraogo, Ouagadougou.

Abstract Congenital lymphedema is the accumulation of lymphatic fluid in the child's interstitial spaces. Milroy disease is a rare, hereditary, autosomal dominant condition showing incomplete penetrance. We report the case of a 7-year old little girl with Milroy disease examined for erysipelas on congenital big right leg. A family history of large congenital member existed. Physical examination showed big oedematous right leg painful to palpation, with skin lichenification and erysipelas. Paraclinical assessment objectified cutaneous lymphedema with vascular involvement suggestive of ectasia of the right saphenous vein. Female karyotype showed no abnormalities, despite the small chromosomal rearrangements. Treatment was based on physiotherapy, bandages, compression stockings and psychotherapy. This first case in Burkina Faso testifies to the rarity of the pathology but especially to the diagnostic difficulties related to the inadequacy of paraclinical investigations.
PMID
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Authors

Mayor MeshTerms

Physical Therapy Modalities

Keywords

Milroy disease

Ouagadougou

Primary lymphedema

diagnosis

treatment

Journal Title the pan african medical journal
Publication Year Start




PMID- 28748022
OWN - NLM
STAT- MEDLINE
DA  - 20170727
DCOM- 20170804
LR  - 20170804
IS  - 1937-8688 (Electronic)
VI  - 27
DP  - 2017
TI  - [Primary congenital lymphedema: Milroy disease: the first case observed in the
      Department of Pediatrics at the University Hospital Yalgado Ouedraogo,
      Ouagadougou].
PG  - 21
LID - 10.11604/pamj.2017.27.21.11443 [doi]
AB  - Congenital lymphedema is the accumulation of lymphatic fluid in the child's
      interstitial spaces. Milroy disease is a rare, hereditary, autosomal dominant
      condition showing incomplete penetrance. We report the case of a 7-year old
      little girl with Milroy disease examined for erysipelas on congenital big right
      leg. A family history of large congenital member existed. Physical examination
      showed big oedematous right leg painful to palpation, with skin lichenification
      and erysipelas. Paraclinical assessment objectified cutaneous lymphedema with
      vascular involvement suggestive of ectasia of the right saphenous vein. Female
      karyotype showed no abnormalities, despite the small chromosomal rearrangements. 
      Treatment was based on physiotherapy, bandages, compression stockings and
      psychotherapy. This first case in Burkina Faso testifies to the rarity of the
      pathology but especially to the diagnostic difficulties related to the inadequacy
      of paraclinical investigations.
FAU - Ouattara, Chantal Zoungrana
AU  - Ouattara CZ
AD  - Departement de Pediatrie du CHU Yalgado Ouedraogo de Ouagadougou, Burkina-faso.
AD  - Unite de Formation et de Recherche en Sciences de la Sante (UFR/SDS), Universite 
      de Ouagadougou, Burkina Faso.
FAU - Kalmogho, Angele
AU  - Kalmogho A
AD  - Departement de Pediatrie du CHU Yalgado Ouedraogo de Ouagadougou, Burkina-faso.
AD  - Unite de Formation et de Recherche en Sciences de la Sante (UFR/SDS), Universite 
      de Ouagadougou, Burkina Faso.
FAU - Yonaba, Caroline
AU  - Yonaba C
AD  - Departement de Pediatrie du CHU Yalgado Ouedraogo de Ouagadougou, Burkina-faso.
AD  - Unite de Formation et de Recherche en Sciences de la Sante (UFR/SDS), Universite 
      de Ouagadougou, Burkina Faso.
FAU - Bouda, Chantal Gabrielle
AU  - Bouda CG
AD  - Departement de Pediatrie du CHU Yalgado Ouedraogo de Ouagadougou, Burkina-faso.
FAU - Yameogo, Ghislaine
AU  - Yameogo G
AD  - Service de Pediatrie Medicale du CHU Charles de Gaulle de Ouagadougou,
      Burkina-faso.
FAU - Kam, Ludovic
AU  - Kam L
AD  - Departement de Pediatrie du CHU Yalgado Ouedraogo de Ouagadougou, Burkina-faso.
AD  - Unite de Formation et de Recherche en Sciences de la Sante (UFR/SDS), Universite 
      de Ouagadougou, Burkina Faso.
LA  - fre
PT  - Case Reports
PT  - Journal Article
TT  - Le lymphoedeme congenital primaire: la maladie de Milroy: a propos du premier cas
      observe dans le Departement de Pediatrie du Centre Hospitalier Universitaire
      Yalgado Ouedraogo, Ouagadougou.
DEP - 20170509
PL  - Uganda
TA  - Pan Afr Med J
JT  - The Pan African medical journal
JID - 101517926
SB  - IM
MH  - Bandages
MH  - Burkina Faso
MH  - Child
MH  - Erysipelas/*etiology
MH  - Female
MH  - Hospitals, University
MH  - Humans
MH  - Lymphedema/congenital/*diagnosis/therapy
MH  - *Physical Therapy Modalities
MH  - Psychotherapy/*methods
MH  - Stockings, Compression
PMC - PMC5511709
OTO - NOTNLM
OT  - Milroy disease
OT  - Ouagadougou
OT  - Primary lymphedema
OT  - diagnosis
OT  - treatment
EDAT- 2017/07/28 06:00
MHDA- 2017/08/05 06:00
CRDT- 2017/07/28 06:00
PHST- 2016/12/18 [received]
PHST- 2017/04/10 [accepted]
AID - 10.11604/pamj.2017.27.21.11443 [doi]
AID - PAMJ-27-21 [pii]
PST - epublish
SO  - Pan Afr Med J. 2017 May 9;27:21. doi: 10.11604/pamj.2017.27.21.11443. eCollection
      2017.