Introduction: Pulmonary sequestration (PS) is a rare pulmonary congenital malformation characterized by disconnection with the tracheobronchial tree or the pulmonary arterial blood supply thus impeding the connection to the arterial blood supply from systemic circulation, ultimately resulting in a non-functional lung.
Patient concerns: A 73-day-old boy with rhabdomyomatoid hyperplasia was hospitalized for cough and fever 2 months after birth.
Diagnoses: Routine B-ultrasound revealed a cystic malformation in the right lung. CT showed increased volume of the right lung accompanied with cystic low-density shadows of different sizes and a blood vessel leading from the abdominal aorta into the lesion lung. Thus, he was diagnosed with PS.
Interventions: The boy underwent an entire lobectomy of the lower lobe of the right lung. The operation field revealed several malformed blood vessels from the apposition to the right lower lobe. Most of the lung had cystic adenomatoid malformations. Other parts consisted of well-differentiated cystic dilated bronchus and striated muscles. Histopathological examination revealed rhabdomyus fibroblasts distributed throughout the bronchi. Nuclei were located at the margins of the cells. Striated muscle fibers observed as immunological markers (myogenin and desmin) of striated muscles were positively detected.
Outcomes: The boy recovered uneventfully after his operation and no abnormal clinical findings were found at the 10-month follow-up.
Conclusion: PS can not only accompany other developmental abnormalities, but can also associate with rhabdomyomatoid hyperplasia. This lesion is not a fatal congenital malformation and instead is a benign lesion affecting only a single lobe. It has a relatively good prognosis.