Lung hypoplasia and its associated major congenital abnormalities in perinatal death: an autopsy study of 850 cases.
OBJECTIVE
To determine the relative frequency of causes of lung hypoplasia (LH) and its associated congenital malformations among perinatal deaths.


METHODS
850 medical reports of perinatal autopsies, in a 25-year period, assessed for LH as a cause of death.


RESULTS
LH found in 96 (11.3%) cases, 89 (92.7%) were associated with major congenital malformation (secondary type) and primary type was seen in 7 cases (7.3%). Fourteen cases were associated with multiple congenital anomalies. 32 cases (33.3%) with Genito-urinary anomalies were the most common associated major malformations, followed by 19 cases (19.8%) of diaphragmatic impairment, 15 cases (15.6%) of musculoskeletal abnormalities and 11 cases (11.4%) of kidney agenesis. The most common musculoskletal abnormality was thanatophoric dwarfism in 10 cases (10.4%). Meckle-Gruber syndrome with 7 affected fetuses (7.3%) was the most common malformation syndrome associated with LH.


CONCLUSIONS
More than ninety percent of LH was secondary to pathology outside the respiratory tract. Renal agenesis is the most common association observed in LH, followed by diaphragmatic hernia and thanatophoric dysplasia.
Aghabiklooei
A
A
Department of Forensic medicine, Iran University of Medical Sciences, Shahrake Gharb, Sanat sq., Tehran, Iran.
Goodarzi
P
P
Kariminejad
Mohammad H
MH
D001344:Autopsy; D002423:Cause of Death; D000013:Congenital Abnormalities / Q000453:epidemiology*; D005260:Female; D006801:Humans; D007226:Infant Mortality / Q000639:trends*; D007231:Infant, Newborn; D008168:Lung / Q000002:abnormalities*; D008297:Male; D012189:Retrospective Studies