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Neonatal pulmonary sequestration (PS) with rhabdomyomas-like hyperplasia: A case report.

ABSTRACT: 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.

SUBMITTER: Liu XM

PROVIDER: S-EPMC7313550 | biostudies-literature | 2020 May

REPOSITORIES: biostudies-literature

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Neonatal pulmonary sequestration (PS) with rhabdomyomas-like hyperplasia: A case report.

Liu Xiu-Mei XM Yuan Li-Mei LM Wu Yu-Rui YR Zhou Chun-Ju CJ

Medicine 20200501 20

<h4>Introduction</h4>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.<h4>Patient concerns</h4>A 73-day-old boy with rhabdomyomatoid hyperplasia was hospitalized for cough and fever 2 months after birth.<h4>Diagnoses</h4>Routine B-ultrasound re ...[more]

PMID: 32443310

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Project description:BACKGROUND:Pulmonary sequestration is a congenital lung disease characterized by nonfunctioning pulmonary tissue that lacks normal communication with the bronchial tree and is supplied by a nonpulmonary systemic artery. Symptomatic bronchopulmonary sequestration is uncommon, seen more frequently in the pediatric population than in adults. It has traditionally been treated with surgical resection; however, a limited but growing number of cases have been treated with angiographic embolization. Given the inherent risks of cardiothoracic surgery, embolization of the anomalous vessel is an enticing alternative treatment. We present a case of a 56-year-old woman with known, symptomatic, intralobar pulmonary sequestration that was successfully treated with coil embolization. CASE PRESENTATION:A 56-year-old Pacific Islander woman with a history of chronic myeloid leukemia was admitted to the hospital with an episode of hemoptysis. Computed tomography of the chest demonstrated left lower lobe intralobar pulmonary sequestration fed by a large tortuous vessel branching off of the descending thoracic aorta. Surgical resection of the sequestration is the current standard treatment strategy of symptomatic intralobar pulmonary sequestration. The cardiothoracic surgeon noted that given the size and location of arterial blood supply, intervention would involve thoracotomy and lobectomy. The interventional radiologist offered embolization of the lesion as an alternative to surgery. Multiple coils, 6-13 mm in size, were used to embolize the sequestration. No considerable flow distal to the coils was noted postembolization. CONCLUSIONS:Intralobar pulmonary sequestration is a rare condition that typically requires surgical management. This case demonstrates the efficacy of coil embolization as an alternative management strategy. To date, limited case reports of adults treated with endovascular embolization exist. Treatment of symptomatic pulmonary sequestration with embolization can be considered as an alternative to surgical resection.

Dataset Information

Neonatal pulmonary sequestration (PS) with rhabdomyomas-like hyperplasia: A case report.

Publications

Neonatal pulmonary sequestration (PS) with rhabdomyomas-like hyperplasia: A case report.

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