León-Ureña Zahira A De, Sadowinski-Pine Stanislaw, Jamaica-Balderas Lourdes, Penchyna-Grub Jaime
Servicio de Neumología. Hospital Infantil de México Federico Gómez, Ciudad de México, México.
Servicio de Patología. Hospital Infantil de México Federico Gómez, Ciudad de México, México.
Bol Med Hosp Infant Mex. 2018;75(2):119-126. doi: 10.24875/BMHIM.M18000007.
Congenital pulmonary malformations are a rare cause of neonatal morbidity. Some of them have a common origin, which allows the identification of combined lesions. Its diagnosis can be made prenatally by ultrasound, with the limitation that this study is performed in specialized centers and depends on the expertise of the operator. The association of pulmonary sequestration and congenital malformation of the airway has been described in approximately 40-60 cases since its first description in 1949. Many lesions are not perceptible in intrauterine life and in the neonatal period there are recurrent respiratory symptoms that in some cases are associated with a congenital pulmonary malformation.
We report the case of a young infant, who was diagnosed with pulmonary sequestration at 24 weeks of gestational age, undergoing intrauterine surgical treatment with a report of complete resolution of the malformation in posterior ultrasounds. She was valued by pediatric pneumology at 4 months of age, where angiotomography was performed and the presence of pulmonary sequestration was confirmed by lobectomy. The histopathological study reported extralobar pulmonary sequestration with congenital malformation of the pulmonary airway type 2. These combined lesions were identified by histopathological study. The treatment of choice was surgical.
Upon the confirmation of a malformation, we emphasize the importance of performing a screening in order to search for other that could be associated.
先天性肺畸形是新生儿发病的罕见原因。其中一些有共同起源，这有助于识别合并病变。其诊断可在产前通过超声进行，但局限性在于该检查需在专业中心进行，且取决于操作者的专业水平。自1949年首次描述以来，肺隔离症与先天性气道畸形的关联已在约40 - 60例病例中被描述。许多病变在宫内生活时不易察觉，在新生儿期会出现反复呼吸道症状，某些情况下与先天性肺畸形有关。
我们报告一例年轻婴儿病例，其在孕24周时被诊断为肺隔离症，接受了宫内手术治疗，后续超声检查报告畸形完全消退。该婴儿在4个月大时由儿科肺病科进行评估，接受了血管造影检查，并通过肺叶切除术证实存在肺隔离症。组织病理学研究报告为叶外型肺隔离症合并2型先天性肺气道畸形。这些合并病变通过组织病理学研究得以识别。首选治疗方法为手术。
在确诊畸形后，我们强调进行筛查以寻找可能相关的其他畸形的重要性。
