Unilateral cervical spinal cord lesion in a term newborn, associated with ipsilateral diaphragmatic atrophy and pulmonary hypoplasia.
Two major factors can interfere with normal development of the fetal lungs: mechanical constriction resulting in decreased intrathoracic space and reduced or absent fetal breathing movements. We report a unique case in which both factors contribute to the development of lethal pulmonary hypoplasia. This full-term neonate had an isolated unilateral lesion in the cervical spinal cord resulting in ipsilateral neurogenic atrophy of the diaphragm, bilateral pulmonary hypoplasia, and lung immaturity. Evenetration of the atrophied diaphragm decreased intrathoracic space, compromising fetal lung growth. This was compounded by impaired fetal respiration due to the diaphragmatic atrophy, further aggravating pulmonary hypoplasia on the affected side. The other lung was also hypoplastic, although to a lesser degree, suggesting that its growth was also affected by either or both of these factors.
Department of Pediatrics, British Columbia's Children's Hospital, Vancouver, Canada.
A
A
Rotschild
E W
EW
Ling
D F
DF
Wensley
M G
MG
Norman
W M
WM
Thurlbeck
D000015:Abnormalities, Multiple / Q000196:embryology*; D001344:Autopsy; D003964:Diaphragm / Q000002:abnormalities*; D017809:Fatal Outcome; D005260:Female; D005325:Fetal Organ Maturity; D006801:Humans; D007231:Infant, Newborn; D008168:Lung / Q000002:abnormalities*; D013116:Spinal Cord / Q000002:abnormalities*