Congenital lymphoedema of the genitalia
Identifieur interne : 00B687 ( Main/Exploration ); précédent : 00B686; suivant : 00B688Congenital lymphoedema of the genitalia
Auteurs : R. J. Bolt [Pays-Bas] ; W. Peelen [Pays-Bas] ; P. G. J. Nikkels [Pays-Bas] ; T. P. V. M. De Jong [Pays-Bas]Source :
- European journal of pediatrics [ 0340-6199 ] ; 1998.
Descripteurs français
- KwdFr :
- Humains, Lymphoedème (), Lymphoedème (anatomopathologie), Maladies de l'appareil génital mâle (), Maladies de l'appareil génital mâle (anatomopathologie), Maladies du prématuré (anatomopathologie), Maladies du pénis (), Maladies du pénis (anatomopathologie), Mâle, Nouveau-né, Prématuré, Scrotum (anatomopathologie).
- MESH :
- anatomopathologie : Lymphoedème, Maladies de l'appareil génital mâle, Maladies du prématuré, Maladies du pénis, Scrotum.
- Pascal (Inist)
- Wicri :
- topic : Enfant.
English descriptors
- KwdEn :
- Child, Corrective surgery, Exeresis, Genital Diseases, Male (congenital), Genital Diseases, Male (pathology), Histopathology, Humans, Infant, Newborn, Infant, Premature, Infant, Premature, Diseases (pathology), Lymphatic circulation, Lymphedema, Lymphedema (congenital), Lymphedema (pathology), Male, Male genital system, Malformation, Penile Diseases (congenital), Penile Diseases (pathology), Scrotum (pathology), Treatment.
- MESH :
- congenital : Genital Diseases, Male, Lymphedema, Penile Diseases.
- pathology : Genital Diseases, Male, Infant, Premature, Diseases, Lymphedema, Penile Diseases, Scrotum.
- Humans, Infant, Newborn, Infant, Premature, Male.
Abstract
Isolated congenital lymphoedema of the external genitalia in boys is extremely rare. It can have major physical and emotional consequences for the children. Three male patients with primary ly mphoedema of the penis and scrotum are described, The first case presented with lymphoedema of the prepuce and the phallic skin that persisted after the age of I year. Circumcision and excision of the subcutaneous tissues of the phallic skin were successful in producing the appearance of a normal circumcised penis, The second case presented with oedema of both phallic and scrotal skin. At age I year only a pastous thickening of the prepuce remained, scrotal and phallic skin normalised spontaneously. Routine circumcision was successful with a 5-year follow up. The third case presented with persistent oedema of both the scrotum and the phallus. He suffered leakage of lymph requiring incontinence pads, with complicating skin infections recurring every 4-6 weeks at age 8 years. After complete peeling of the scrotal and phallic skin from subcutaneous tissue some leakage persisted, infections subsided. The cause of this disorder remains unknown, although hypoplasia of the lymphatic vessels is reported in most cases. In the literature, several congenital malformations have been associated with primary lymphoedema. These were not noted in our patients. Rarely, the lymphoedema regresses spontaneously. The treatment of persistent lymphoedema is surgical and consists of meticulous excision of all subcutaneous layers of the affected skin, combined with reconstruction of the penis and or scrotum, Conclusion Primary lymphoedema of the male external genitalia is an extremely rare malformation of the lymphatic vessels of unknown origin. If persistent, surgical treatment is necessary.
Affiliations:
Links toward previous steps (curation, corpus...)
- to stream PascalFrancis, to step Corpus: 000A59
- to stream PascalFrancis, to step Curation: 001049
- to stream PascalFrancis, to step Checkpoint: 000A94
- to stream Main, to step Merge: 00BE18
- to stream PubMed, to step Corpus: 004D93
- to stream PubMed, to step Curation: 004D93
- to stream PubMed, to step Checkpoint: 004D93
- to stream Ncbi, to step Merge: 00BD26
- to stream Ncbi, to step Curation: 00BD26
- to stream Ncbi, to step Checkpoint: 00BD26
- to stream Main, to step Merge: 00B832
- to stream Main, to step Curation: 00B687
Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en" level="a">Congenital lymphoedema of the genitalia</title>
<author><name sortKey="Bolt, R J" sort="Bolt, R J" uniqKey="Bolt R" first="R. J." last="Bolt">R. J. Bolt</name>
<affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Paediatrics, Hospital Sint Jansdal, PO Box 138</s1>
<s2>3840 AC Harderwijk</s2>
<s3>NLD</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
</inist:fA14>
<country>Pays-Bas</country>
<wicri:noRegion>3840 AC Harderwijk</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Peelen, W" sort="Peelen, W" uniqKey="Peelen W" first="W." last="Peelen">W. Peelen</name>
<affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Paediatrics, Hospital Sint Jansdal, PO Box 138</s1>
<s2>3840 AC Harderwijk</s2>
<s3>NLD</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
</inist:fA14>
<country>Pays-Bas</country>
<wicri:noRegion>3840 AC Harderwijk</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Nikkels, P G J" sort="Nikkels, P G J" uniqKey="Nikkels P" first="P. G. J." last="Nikkels">P. G. J. Nikkels</name>
<affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Department of Pathology, University Hospital Utrecht, P.O. Box 85500</s1>
<s2>3508 GA Utrecht</s2>
<s3>NLD</s3>
<sZ>3 aut.</sZ>
</inist:fA14>
<country>Pays-Bas</country>
<wicri:noRegion>3508 GA Utrecht</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="De Jong, T P V M" sort="De Jong, T P V M" uniqKey="De Jong T" first="T. P. V. M." last="De Jong">T. P. V. M. De Jong</name>
<affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Department of Child Urology, Paediatric Renal Centre, University Children's Hospital, P.O. Box 18009</s1>
<s2>3501 CA Utrecht</s2>
<s3>NLD</s3>
<sZ>4 aut.</sZ>
</inist:fA14>
<country>Pays-Bas</country>
<wicri:noRegion>3501 CA Utrecht</wicri:noRegion>
</affiliation>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">INIST</idno>
<idno type="inist">99-0026049</idno>
<date when="1998">1998</date>
<idno type="stanalyst">PASCAL 99-0026049 INIST</idno>
<idno type="RBID">Pascal:99-0026049</idno>
<idno type="wicri:Area/PascalFrancis/Corpus">000A59</idno>
<idno type="wicri:Area/PascalFrancis/Curation">001049</idno>
<idno type="wicri:Area/PascalFrancis/Checkpoint">000A94</idno>
<idno type="wicri:explorRef" wicri:stream="PascalFrancis" wicri:step="Checkpoint">000A94</idno>
<idno type="wicri:doubleKey">0340-6199:1998:Bolt R:congenital:lymphoedema:of</idno>
<idno type="wicri:Area/Main/Merge">00BE18</idno>
<idno type="wicri:source">PubMed</idno>
<idno type="RBID">pubmed:9835443</idno>
<idno type="wicri:Area/PubMed/Corpus">004D93</idno>
<idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">004D93</idno>
<idno type="wicri:Area/PubMed/Curation">004D93</idno>
<idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Curation">004D93</idno>
<idno type="wicri:Area/PubMed/Checkpoint">004D93</idno>
<idno type="wicri:explorRef" wicri:stream="Checkpoint" wicri:step="PubMed">004D93</idno>
<idno type="wicri:Area/Ncbi/Merge">00BD26</idno>
<idno type="wicri:Area/Ncbi/Curation">00BD26</idno>
<idno type="wicri:Area/Ncbi/Checkpoint">00BD26</idno>
<idno type="wicri:doubleKey">0340-6199:1998:Bolt R:congenital:lymphoedema:of</idno>
<idno type="wicri:Area/Main/Merge">00B832</idno>
<idno type="wicri:Area/Main/Curation">00B687</idno>
<idno type="wicri:Area/Main/Exploration">00B687</idno>
</publicationStmt>
<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Congenital lymphoedema of the genitalia</title>
<author><name sortKey="Bolt, R J" sort="Bolt, R J" uniqKey="Bolt R" first="R. J." last="Bolt">R. J. Bolt</name>
<affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Paediatrics, Hospital Sint Jansdal, PO Box 138</s1>
<s2>3840 AC Harderwijk</s2>
<s3>NLD</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
</inist:fA14>
<country>Pays-Bas</country>
<wicri:noRegion>3840 AC Harderwijk</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Peelen, W" sort="Peelen, W" uniqKey="Peelen W" first="W." last="Peelen">W. Peelen</name>
<affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Department of Paediatrics, Hospital Sint Jansdal, PO Box 138</s1>
<s2>3840 AC Harderwijk</s2>
<s3>NLD</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
</inist:fA14>
<country>Pays-Bas</country>
<wicri:noRegion>3840 AC Harderwijk</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Nikkels, P G J" sort="Nikkels, P G J" uniqKey="Nikkels P" first="P. G. J." last="Nikkels">P. G. J. Nikkels</name>
<affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Department of Pathology, University Hospital Utrecht, P.O. Box 85500</s1>
<s2>3508 GA Utrecht</s2>
<s3>NLD</s3>
<sZ>3 aut.</sZ>
</inist:fA14>
<country>Pays-Bas</country>
<wicri:noRegion>3508 GA Utrecht</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="De Jong, T P V M" sort="De Jong, T P V M" uniqKey="De Jong T" first="T. P. V. M." last="De Jong">T. P. V. M. De Jong</name>
<affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Department of Child Urology, Paediatric Renal Centre, University Children's Hospital, P.O. Box 18009</s1>
<s2>3501 CA Utrecht</s2>
<s3>NLD</s3>
<sZ>4 aut.</sZ>
</inist:fA14>
<country>Pays-Bas</country>
<wicri:noRegion>3501 CA Utrecht</wicri:noRegion>
</affiliation>
</author>
</analytic>
<series><title level="j" type="main">European journal of pediatrics</title>
<title level="j" type="abbreviated">Eur. j. pediatr.</title>
<idno type="ISSN">0340-6199</idno>
<imprint><date when="1998">1998</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
<seriesStmt><title level="j" type="main">European journal of pediatrics</title>
<title level="j" type="abbreviated">Eur. j. pediatr.</title>
<idno type="ISSN">0340-6199</idno>
</seriesStmt>
</fileDesc>
<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Child</term>
<term>Corrective surgery</term>
<term>Exeresis</term>
<term>Genital Diseases, Male (congenital)</term>
<term>Genital Diseases, Male (pathology)</term>
<term>Histopathology</term>
<term>Humans</term>
<term>Infant, Newborn</term>
<term>Infant, Premature</term>
<term>Infant, Premature, Diseases (pathology)</term>
<term>Lymphatic circulation</term>
<term>Lymphedema</term>
<term>Lymphedema (congenital)</term>
<term>Lymphedema (pathology)</term>
<term>Male</term>
<term>Male genital system</term>
<term>Malformation</term>
<term>Penile Diseases (congenital)</term>
<term>Penile Diseases (pathology)</term>
<term>Scrotum (pathology)</term>
<term>Treatment</term>
</keywords>
<keywords scheme="KwdFr" xml:lang="fr"><term>Humains</term>
<term>Lymphoedème ()</term>
<term>Lymphoedème (anatomopathologie)</term>
<term>Maladies de l'appareil génital mâle ()</term>
<term>Maladies de l'appareil génital mâle (anatomopathologie)</term>
<term>Maladies du prématuré (anatomopathologie)</term>
<term>Maladies du pénis ()</term>
<term>Maladies du pénis (anatomopathologie)</term>
<term>Mâle</term>
<term>Nouveau-né</term>
<term>Prématuré</term>
<term>Scrotum (anatomopathologie)</term>
</keywords>
<keywords scheme="MESH" qualifier="anatomopathologie" xml:lang="fr"><term>Lymphoedème</term>
<term>Maladies de l'appareil génital mâle</term>
<term>Maladies du prématuré</term>
<term>Maladies du pénis</term>
<term>Scrotum</term>
</keywords>
<keywords scheme="MESH" qualifier="congenital" xml:lang="en"><term>Genital Diseases, Male</term>
<term>Lymphedema</term>
<term>Penile Diseases</term>
</keywords>
<keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Genital Diseases, Male</term>
<term>Infant, Premature, Diseases</term>
<term>Lymphedema</term>
<term>Penile Diseases</term>
<term>Scrotum</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Humans</term>
<term>Infant, Newborn</term>
<term>Infant, Premature</term>
<term>Male</term>
</keywords>
<keywords scheme="Pascal" xml:lang="fr"><term>Humains</term>
<term>Lymphoedème</term>
<term>Appareil génital mâle</term>
<term>Circulation lymphatique</term>
<term>Histopathologie</term>
<term>Maladies de l'appareil génital mâle</term>
<term>Maladies du pénis</term>
<term>Malformation</term>
<term>Exérèse</term>
<term>Chirurgie correctrice</term>
<term>Mâle</term>
<term>Nouveau-né</term>
<term>Prématuré</term>
<term>Traitement</term>
<term>Enfant</term>
</keywords>
<keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Enfant</term>
</keywords>
</textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en">Isolated congenital lymphoedema of the external genitalia in boys is extremely rare. It can have major physical and emotional consequences for the children. Three male patients with primary ly mphoedema of the penis and scrotum are described, The first case presented with lymphoedema of the prepuce and the phallic skin that persisted after the age of I year. Circumcision and excision of the subcutaneous tissues of the phallic skin were successful in producing the appearance of a normal circumcised penis, The second case presented with oedema of both phallic and scrotal skin. At age I year only a pastous thickening of the prepuce remained, scrotal and phallic skin normalised spontaneously. Routine circumcision was successful with a 5-year follow up. The third case presented with persistent oedema of both the scrotum and the phallus. He suffered leakage of lymph requiring incontinence pads, with complicating skin infections recurring every 4-6 weeks at age 8 years. After complete peeling of the scrotal and phallic skin from subcutaneous tissue some leakage persisted, infections subsided. The cause of this disorder remains unknown, although hypoplasia of the lymphatic vessels is reported in most cases. In the literature, several congenital malformations have been associated with primary lymphoedema. These were not noted in our patients. Rarely, the lymphoedema regresses spontaneously. The treatment of persistent lymphoedema is surgical and consists of meticulous excision of all subcutaneous layers of the affected skin, combined with reconstruction of the penis and or scrotum, Conclusion Primary lymphoedema of the male external genitalia is an extremely rare malformation of the lymphatic vessels of unknown origin. If persistent, surgical treatment is necessary.</div>
</front>
</TEI>
<affiliations><list><country><li>Pays-Bas</li>
</country>
</list>
<tree><country name="Pays-Bas"><noRegion><name sortKey="Bolt, R J" sort="Bolt, R J" uniqKey="Bolt R" first="R. J." last="Bolt">R. J. Bolt</name>
</noRegion>
<name sortKey="De Jong, T P V M" sort="De Jong, T P V M" uniqKey="De Jong T" first="T. P. V. M." last="De Jong">T. P. V. M. De Jong</name>
<name sortKey="Nikkels, P G J" sort="Nikkels, P G J" uniqKey="Nikkels P" first="P. G. J." last="Nikkels">P. G. J. Nikkels</name>
<name sortKey="Peelen, W" sort="Peelen, W" uniqKey="Peelen W" first="W." last="Peelen">W. Peelen</name>
</country>
</tree>
</affiliations>
</record>
Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Wicri/Sante/explor/LymphedemaV1/Data/Main/Exploration
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 00B687 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/Main/Exploration/biblio.hfd -nk 00B687 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien |wiki= Wicri/Sante |area= LymphedemaV1 |flux= Main |étape= Exploration |type= RBID |clé= Pascal:99-0026049 |texte= Congenital lymphoedema of the genitalia }}
![]() | This area was generated with Dilib version V0.6.31. | ![]() |