CASE 14485 Published on 29.03.2017

Spontaneous regression of intra-lobar pulmonary sequestration: a case report

Section

Paediatric radiology

Case Type

Clinical Cases

Authors

Sghedoni B, Fiocchi F, Di Pancrazio L, Ceccarelli L, Torricelli P

Policlinico di Modena,
Università di Modena e Reggio Emilia,
Istituto di Radiologia;
del Pozzo 71
41100 Modena, Italy;
Email:federica.fiocchi@gmail.com
Patient

2 years, male

Categories
Area of Interest Lung, Paediatric, Arteries / Aorta, Thorax ; Imaging Technique Digital radiography, CT
Clinical History
A 1-day-old baby boy was born with prenatal ultrasound feedback of intra-lobar pulmonary sequestration with feeding vessel originating from the descending thoracic aorta.
Imaging Findings
Chest X-ray showed an oval homogeneous opacity in the right lower lobe, close to the mediastinum. Computed tomography (CT) performed with intravenous contrast media detected a well-defined parenchymal consolidation of 1.6 x 1.2 cm. No pathological lymph nodes or pleural effusion were found. CT angiography revealed the presence of a small vessel arising from the mesenteric artery, and extending into the thorax and into the mass, passing close to the oesophagus.
The combination of well-defined lung mass and aberrant systemic vascular supply was pathognomonic for a diagnosis of intralobar pulmonary sequestration (ILS). The child remained asymptomatic during subsequent follow-up and no signs of infection were reported at serial clinical evaluations.
A follow-up CT angiogram at 2 years revealed the complete disappearance of the lesion. The aberrant vessel was hardly observable up to the thoracic-abdominal diaphragm.
Discussion
Pulmonary sequestration was first described by Rektorzik in 1861, as a segment part of non-functional lung that lacks normal connection to the tracheobronchial tree. The sequestered tissue usually receives the majority of its blood supply from systemic vessels, most often the distal thoracic or upper abdominal aorta, and venous drainage occurs most commonly via pulmonary veins followed by the azygous or hemiazygous system [1].
Investment of the dysplastic tissue mass by visceral pleura (intralobar) or by parietal pleura (extralobar) distinguishes the two types of sequestration. These lesions are rare pulmonary malformations accounting for 0.15 to 6.4% of all congenital anomalies [2]. Intralobar sequestration (ILS) is four times more common than the extralobar type (ELS) [3]. Overall, sequestration preferentially affects the lower lobes. They are almost always unilateral, the left side of the chest is affected twice as often as the right (respectively 90% versus 60%) and there is a male to female ratio of 3:1 in ELS and of 1:1 in ILS [4]. Associated congenital anomalies include diaphragmatic hernia, congenital cystic adenoid malformation, bronchogenic cysts, cardiovascular malformations, and pectus excavatum. Associated congenital anomalies are rare in ILS [3].
Clinical presentation depends on the type of sequestration [5]. ILS has been associated with chronic infections in infants and children due to connections with gastrointestinal tract, and resection is recommended regardless of the presence or absence of symptoms. ELS has not been associated with infection or malignant transformation and therefore expectant management is recommended with serial imaging in asymptomatic patients [6].
Prognosis is variable, ranging from lethal outcome to even spontaneous regression [1, 5, 7] of some lesions such us in our case. Survival has been reported after successful medical management, generally surgery, even in cases with a severe clinical presentation [5, 8].
We presented an atypical case of a baby boy with prenatal diagnosis of right ILS confirmed postnatally who remained asymptomatic in the first two years of life and which spontaneous regressed. Spontaneous regression is a rare but possible event that has been described [1, 9, 10, 11].
The radiological investigations such as CT angiography performed after birth allow accurate identification, localization and characterization of the pulmonary sequestration and demonstrate the aberrant arterial supply.
Differential Diagnosis List
Right intra-lobar pulmonary sequestration with spontaneous regression.
Congenital Cystic Adenomatoid Malformation (CCAM)
Microcystic (CCAM type III) solid form of CCAM
Final Diagnosis
Right intra-lobar pulmonary sequestration with spontaneous regression.
Case information
URL: https://www.eurorad.org/case/14485
DOI: 10.1594/EURORAD/CASE.14485
ISSN: 1563-4086
License