Rotschild A, Ling E W, Wensley D F, Norman M G, Thurlbeck W M
Department of Pediatrics, British Columbia's Children's Hospital, Vancouver, Canada.
Pediatr Pulmonol. 1994 Jul;18(1):53-7. doi: 10.1002/ppul.1950180114.
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.
机械性压迫导致胸腔内空间减小,以及胎儿呼吸运动减少或缺失。我们报告了一个独特的病例,这两个因素共同导致了致死性肺发育不全。这名足月新生儿在颈髓有一个孤立的单侧病变,导致同侧膈肌神经源性萎缩、双侧肺发育不全和肺不成熟。萎缩的膈肌疝入胸腔减少了胸腔内空间,影响了胎儿肺的生长。由于膈肌萎缩导致胎儿呼吸受损,这使情况更加复杂,进一步加重了患侧的肺发育不全。另一侧肺也发育不全,尽管程度较轻,这表明其生长也受到了这些因素中的一个或两个的影响。