Document Detail

Isolated congenital spleen agenesis: a rare cause of chronic thromboembolic pulmonary hypertension in an adult.
MedLine Citation:
PMID:  18657065     Owner:  NLM     Status:  MEDLINE    
This report describes a case of isolated congenital spleen agenesis complicated by chronic thromboembolic pulmonary hypertension (CTPH) in a 44-year-old female patient. The patient had increasing exertional dyspnoea and thrombocytosis. An echocardiogram showed severe pulmonary hypertension and right ventricular hypertrophy, and contrast-enhanced chest CT revealed multiple thromboemboli within both pulmonary arteries. A perfusion lung scan demonstrated multiple segmental defects and no spleen was detected by abdominal CT, ultrasonography or scintigraphy. Comprehensive clinical examinations disclosed no evidence of a thrombus elsewhere or of an associated malformation such as a cardiac anomaly. Anticoagulation therapy was started, and a perfusion lung scan revealed partial improvement of the hypoperfusion in the right lower lobe. However, repeat echocardiography showed the pulmonary hypertension persisting for 1 year. The multiple segmental defects in the perfusion lung scans were also persistent. Collectively, a diagnosis of CTPH with isolated congenital spleen agenesis was established. This is the first documented case of CTPH in an adult with isolated congenital asplenia. Although congenital spleen agenesis is a rare condition, this case report suggests that this possibility should be considered when a diagnosis of CTPH and thrombocytosis is made.
Fumiyuki Takahashi; Koji Uchida; Tetsutaro Nagaoka; Noriyuki Honma; Ri Cui; Masakata Yoshioka; Yoshiteru Morio; Tsutomu Suzuki; Shigeru Tominaga; Kazuhisa Takahashi; Yoshinosuke Fukuchi
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2008-07-24
Journal Detail:
Title:  Respirology (Carlton, Vic.)     Volume:  13     ISSN:  1440-1843     ISO Abbreviation:  Respirology     Publication Date:  2008 Nov 
Date Detail:
Created Date:  2008-09-24     Completed Date:  2009-02-03     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9616368     Medline TA:  Respirology     Country:  Australia    
Other Details:
Languages:  eng     Pagination:  913-5     Citation Subset:  IM    
Department of Respiratory Medicine, Juntendo University School of Medicine, Tokyo, Japan.
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MeSH Terms
Dyspnea / etiology
Hypertension, Pulmonary / etiology*
Pulmonary Artery / radiography
Pulmonary Embolism / complications*,  radiography
Spleen / abnormalities*
Thrombocytosis / etiology
Tomography, X-Ray Computed

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