Case of the Week #646
Femicare, Center of prenatal ultrasonographic diagnostics, Martin, Slovakia
A 29-year-old primigravida with an unremarkable medical history underwent fetal ultrasound examinations at 22+3 and 31+4 weeks of gestation. The following images and video clips demonstrate the findings.
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Answer
We present a case of intrathoracic extralobar pulmonary sequestration (ELS).
During the course of pregnancy, the lesion showed progressive regression in size and became nearly indistinguishable from the adjacent lung parenchyma in the third trimester. No cardiac displacement was observed. The markedly reduced lesion remained visible on sagittal views, together with a persistent systemic feeding artery arising from the descending aorta. The female infant was delivered at term without complications. Postnatal chest radiography showed no apparent abnormality. However, follow-up ultrasound examination at one month of age confirmed the presence of a small residual lesion consistent with pulmonary sequestration. Given the absence of symptoms and the good clinical condition, elective surgical resection has been deferred and is planned at 5–6 years of age.
Discussion
Extralobar pulmonary sequestration (ELS) is a rare congenital bronchopulmonary malformation characterized by non functioning lung tissue that lacks communication with the tracheobronchial tree and receives its arterial supply from the systemic circulation, most commonly from the descending thoracic aorta [1,2]. In contrast to intralobar sequestration, ELS possesses its own pleural covering and typically presents as a well defined, triangular or ovoid echogenic mass adjacent to the lower lung lobes or within the posterior mediastinum [2]. Associated findings may include congenital diaphragmatic hernia, pulmonary hypoplasia, congenital cystic adenomatoid malformation, congenital lobar emphysema or bronchogenic cyst [3].
Prenatal sonography usually demonstrates a wedge-shaped, hyperechogenic intrathoracic mass, though it can be subdiaphragmatic in 30% of cases [3,4]. The identification of a systemic feeding artery on color Doppler is considered pathognomonic and remains the most reliable diagnostic feature, allowing differentiation from other echogenic lung lesions such as congenital pulmonary airway malformation (CPAM), hybrid lesions, bronchogenic cysts, or neuroblastoma [2,3]. In the present case, the persistent arterial vessel arising from the descending aorta confirmed the diagnosis despite near complete regression of the parenchymal component during late gestation.
The natural history of pulmonary sequestration diagnosed prenatally is variable [3-5]. Approximately 80% of lesions exhibit partial spontaneous regression around 26 weeks gestation [4], and cases of antenatal involution or disappearance have been documented [6]. While mediastinal shift occurs in 30% of cases, cardiac compression, polyhydramnios and hydrops are uncommon and usually limited to larger lesions [3-5]. Approximately 7% of patients with extralobar pulmonary sequestration will develop hydrops, though survival rates are high despite these findings [4,6]. In a literature review including 13 patients with pulmonary sequestration who developed hydrops, 7 patients underwent fetal intervention including fetal resection, paracentesis, thoracoamniotic shunting, and alcohol ablation of the vascular pedicle. All fetuses with hydrops survived whether or not they underwent intervention [6].
Postnatal management remains debated. While some centers advocate routine early surgical resection to prevent infection, torsion, or malignant transformation, others support conservative management in asymptomatic infants, with delayed or elective surgery [2,5,7]. Current trends favor individualized decision making. Lesion size measured by CCAM volume ratio (CVR) > 0.75 increases the odds of respiratory symptoms at birth by a ratio of 20 [4]. For small, asymptomatic ELS with stable imaging findings, delayed elective resection in early childhood is considered safe and avoids neonatal anesthesia risks [7]. The planned surgery at 5–6 years in this case aligns with this conservative strategy.
This case illustrates several teaching points relevant to prenatal diagnosis:
- Doppler identification of a systemic feeding artery is crucial for diagnosis.
- Significant third trimester regression does not exclude residual postnatal disease.
- Absence of mediastinal shift predicts an excellent perinatal outcome.
- Postnatal follow up imaging is mandatory even when prenatal “disappearance” is observed.
Overall, isolated intrathoracic ELS carries an excellent prognosis, and prenatal recognition allows appropriate counseling, avoidance of unnecessary intervention, and tailored postnatal planning.
References
- Savic B, Birtel FJ, Tholen W, et al. Lung sequestration: report of seven cases and review of 540 published cases. Thorax. 1979;34:96–101.
- Corbett HJ, Humphrey GME. Pulmonary sequestration. Paediatr Respir Rev. 2004;5:59–68.
- Yildirim G, Güngördük K, Aslan H, et al. Prenatal diagnosis of an extralobar pulmonary sequestration. Arch Gynecol Obstet. 2008;277:181–183.
- Riley JS, Urwin JW, Oliver ER, et al. Prenatal growth characteristics and pre/postnatal management of bronchopulmonary sequestrations. J Pediatr Surg. 2018;53(2):265–269.
- Langer B, Donato L, Riethmuller C, et al. Spontaneous regression of fetal pulmonary sequestration. Ultrasound Obstet Gynecol. 1995;6(1):33–39.
- Hung JH, Shen SH, Guo WY, et al. Prenatal diagnosis of pulmonary sequestration by ultrasound and magnetic resonance imaging. J Chin Med Assoc. 2008 Jan;71(1):53-7.
- Stanton M. The argument for a non‑operative approach to asymptomatic lung lesions. Semin Pediatr Surg. 2015;24:183–186.
Discussion Board
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