Document Detail

Hypothalamic hamartoma associated with multiple congenital abnormalities. Two patients and a review of reported cases.
MedLine Citation:
PMID:  9973674     Owner:  NLM     Status:  MEDLINE    
We report 2 patients with hypothalamic hamartoma associated with multiple congenital abnormalities and analyze 42 (including our own) reported cases, including our 2 cases, of hypothalamic hamartoma or hypothalamic hamartoblastoma with multiple congenital abnormalities, to understand the timing of their occurrence and clarify the prognosis. To this end, we classified them into lethal and nonlethal cases. We found poly- and syndactyly, cleft or high-arched palate and nose abnormalities to be important manifestations of this syndrome. Major organ abnormalities and CNS and endocrine abnormalities occurred frequently among the lethal cases, very likely indicative of a disturbance of embryogenesis between gestational days 34-37 and thus implicated in a negative prognosis.
H Tsugu; T Fukushima; T Nagashima; H Utsunomiya; M Tomonaga; A Mitsudome
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Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  Pediatric neurosurgery     Volume:  29     ISSN:  1016-2291     ISO Abbreviation:  Pediatr Neurosurg     Publication Date:  1998 Dec 
Date Detail:
Created Date:  1999-07-14     Completed Date:  1999-07-14     Revised Date:  2005-11-16    
Medline Journal Info:
Nlm Unique ID:  9114967     Medline TA:  Pediatr Neurosurg     Country:  SWITZERLAND    
Other Details:
Languages:  eng     Pagination:  290-6     Citation Subset:  IM    
Department of Neurosurgery, School of Medicine, Fukuoka University, Fukuoka, Japan.
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MeSH Terms
Abnormalities, Multiple* / diagnosis
Child, Preschool
Hamartoma* / complications,  diagnosis
Hypothalamic Diseases* / complications,  diagnosis
Pierre Robin Syndrome / complications,  diagnosis

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