The rare congenital anomaly of pulmonary sequestration experience and review of literature (original) (raw)

Pulmonary sequestration: a review of 26 cases

European Journal of Cardio-Thoracic Surgery, 1998

Objectives: Pulmonary sequestration is a continuum of lung anomalies for which no single embryonic hypothesis is yet available. The aim of this study was to assess the diagnostic tools and treatment for the rare condition, pulmonary sequestration, in an unspecialised centre. Methods: We performed an analysis of 26 cases of pulmonary sequestration (paediatric and adult) operated at the Centre Hospitalier Universitaire Vaudois between May 1959 and May 1997. A review of the extralobar and intralobar types of sequestrations is discussed. Angiography is compared to other diagnostic tools in this condition, and treatment is discussed. Results: Twenty-six cases of pulmonary sequestrations, a rare congenital pulmonary malformation, were operated on in the defined time period. Seventy-three percent (19) of the cases were intralobar and 27% (seven) extralobar. Extralobar localisation was basal in 71% and situated between the upper and the lower lobe in 29%. In six cases, the diagnosis was made by exploratory thoracotomy. In the other 20 cases, diagnosis was evoked on chest X-ray and confirmed by angiography. Lobectomy (46%) was the most common treatment procedure. Segmental resection was performed in 30% of the cases and bilobectomy in 4%. Post-operative morbidity was low. The most significant complications were pleural empyema, haemothorax and haemopneumoperitoneum in case of extralobar sequestration. There was no evidence of metaplasia or pre-neoplastic changes. Conclusions: Despite its rarity, some radiological features are sufficiently suggestive of diagnosis of pulmonary sequestration. Investigations are necessary in order to avoid unexpected pathology at the time of operation. Resection of the involved lung leads to excellent results and the long-term outcome is highly favourable.

Thoracoscopic treatment of a rare bilateral extralobar lung sequestration in a 3-years old girl

La Pediatria Medica e Chirurgica

Majority of sequestrations fall into two categories: Intra-Lobar (ILS) and Extra-Lobar (ELS). Rarely the abnormal lung could be attached to the gastrointestinal tract, Bronchopulmonary Foregut Malformation (BPFM). We described a case of a girl of 3-years-old with antenatal diagnosis of left intrathoracic mass of the inferior lobe. Postnatal Computed-Tomography (CT) revealed a bilateral ELS with an isthmic bridge crossing the vertebral spine. She follows a MRI follow-up at 18months/30months confirming the lesion. Before surgery, a three-dimensional-CT-angiography was performed to study the mass, its blood supply and to plan surgery. She underwent to thoracoscopic resection. Two aberrant blood vessels were dissected from the thoracic aorta and ligated. The postoperative course was uneventful. She was discharged after 3 days. The rarity of our case is due to the bilateral extension. An appropriate preoperatory imaging study is necessary for the success of surgery while thoracoscopy is ...

Thoracoscopic Excision of Prenatal Diagnosed Extralobar Pulmonary Sequestration – Case Report

Jurnalul Pediatrului, 2020

Background: Pulmonary sequestrations are rare congenital bronchopulmonary malformation. Two types of bronchopulmonary sequestration have been described: intralobar and, extralobar sequestration. Case report: We present the case of a male patient with extralobar sequestration diagnosed prenatally and successfully treated by means of video-assisted surgical excision at the age of 7 months. Prenatal diagnostic of pulmonary malformation was suspected at routine ultrasound and confirmed on fetal MRI. The baby was delivered at 39 weeks of gestation by C-section. The diagnostic of extralobar sequestration was confirmed by CT scan performed at the age of 1 month. The child was asymptomatic so the surgical excision of the mass was performed after the age of 6 months by means of video-assisted thoracoscopy. The procedure was technical challenging but underwent flawless. The postoperative course was uneventful. Conclusions: Prenatal diagnostics is a very useful tool in planning and making appropriate therapeutic decision for patients with pulmonary sequestration. Video-assisted thoracoscopic excision is the optimal treatment option for extralobar sequestration.

A Rare Case of Congenital Pulmonary Airway Malformation Associated with Intralobar Pulmonary Sequestration in an Adult - Case Report

ARS Medica Tomitana, 2018

The congenital pulmonary airway malformations (CPAM) are extremely rare malformations which are most frequently diagnosed before birth or during childhood and are extremely rarely diagnosed in adults. Sometimes, type II CPAM can be associated with pulmonary sequestrations (PS), especially those extralobar. We present the case of a 39 years old female without any known previous respiratory symptoms until this age, who has having for approximatively 2 months repeated episodes of haemoptysis. The angio-CT exam showed the existence of multiple cyst-like formations in the left inferior pulmonary lobe (LLL) and 2 arteries starting from thoracic aorta that participated at the vascularization of the LLL. We performed a left inferior lobectomy and the postoperative evolution was favorable. The histopathological exam confirmed the diagnosis of type I CPAM associated with intralobar PS. The particularity of the presented case is given by the fact that this patient of 39 years old had no previo...

Uniportal thoracoscopic resection of intralobar and extralobar pulmonary sequestration

Journal of visualized surgery, 2018

Pulmonary sequestration (PS) is a rare congenital malformation of the respiratory tract. Two main variants are described, the intralobar and the extralobar PS. Clinical manifestations vary from accidental findings to life threatening complications. Surgical resection is the definitive and indicated treatment of PS. The operation could be performed through an open thoracotomy or video-assisted thoracic surgery approach. We report the management of two patients with diagnosis of extralobar PS in the first case and intralobar PS in the second case. Both patients underwent uniportal video-assisted thoracic surgery resection of PS with success. In our experience, we confirm that uniportal video-assisted thoracic surgery is a safe and feasible approach for extralobar and intralobar PS.

Pulmonary sequestrations: experience of 31 patients

GULHANE MEDICAL JOURNAL

Aims:Pulmonary sequestration (PS) is a rare congenital lung malformation. It is characterized by a nonfunctional pulmonary tissue that have no relation to the bronchial system. Case series are rare due to low incidence of pulmonary sequestration. In this study, we aim to analyze the presentation, demographics, diagnostic procedures, location, type and treatment of PS in our institution over 12-year period. Methods:We retrospectively evaluated medical records of pathologically proved PS from January 2003 through December 2015 in our institution. Thirty-one patients were included in the study. Demographics, clinical presentation, diagnostic imaging, location, type of sequestration, type of surgery were collected. Results:Of 31 patients, 3 patients (9.6%) were female and 28 patients were (90.3%) male. Average age was 24.6 years. Twenty-four (77%) patients had described symptoms. Chest X-ray scanning was performed to all patients as the first imaging modality. Of the 15 patients (48.4%) had intralobar sequestration and 16 patients (51.6%) had extralobar sequestration. Extralobar sequestrations were more common (55.1%). Lower lobectomy in 13 patients, segmentectomy in 2 intralobar sequestration patients, and simple mass excision in all extralobar sequestration patients (16 patients) were performed as surgical procedure. Six patients had postoperative complications: prolonged air leak in 4 patients, pneumonia in 2 patients. Conclusions:It has similar appearance with many diseases of lung with traditional imaging methods and cause difficulties in diagnosis. Proving the presence of a feeding systemic artery with angiographic imaging is essential for definitive diagnosis. The recommended optimal treatment for pulmonary sequestration is resection of the sequestered tissue by segment or lobar resection.

Computed tomography findings of atypical intralobar pulmonary sequestration: A case series

2021

Introduction: Bronchopulmonary sequestration is a rare congenital abnormality of the lung that has two different types as intralobar and extralobar. In this study, we aimed to present six cases of intralobar sequestration with atypical findings in terms of feeding, drainage, and localization. Methods: Patients diagnosed with intralobar pulmonary sequestration in our clinic between 2015 and 2019 were evaluated retrospectively. Demographical features and atypical computed tomography (CT) findings of the patients were presented by literature. Results: Among 45 patients with intralobar sequestration, 6 (13.3%) (5 males and 1 female) with a mean age of 43.5±25.4(0-78) years old had atypical pulmonary findings on CT images. Atypical features regarding arterial supply was detected in 8.9%, venous drainage in 2.2%, location in 4.4%, radiological appearance in 4.4% and co-existing lesion in 2.2% of the patient with intralobar sequestration. Conclusion: Typical and atypical features of pulmon...

A Case Of Intrapulmonary Sequestration Seeming As An Intrathoracic Mass

Sakarya Medical Journal, 2014

As a rare congenital thoracic malformation, pulmonary sequestration is composed of nonfunctioning primitive lung tissue. It has anomalous systemic supply rather than the pulmonary circulation and no connection with the tracheobronchial tree. It may present as respiratory infection or asymptomatic mass on chest imaging studies. Surgical removal is the treatment of choice. This paper presents a case of intralobar pulmonary sequestration that presented during childhood.

A rare case of coexistent intralobar and extralobar pulmonary sequestration

Central European Journal of Medicine, 2012

In the pathology of respiratory tract, sequestration presents as a non-functional lung tissue with no communication with tracheobronchial tree. It represents a rare congenital bronchopulmonary and vascular malformation, which occurs at a frequency of 0.1 to 6%. Intralobar and extralobar sequestrations are extremely rare congenital anomalies, especially if they occur at the same time in a patient. Proper diagnosis and appropriate surgical therapy, in the absence of associated anomalies, provide an excellent prognosis. In this paper, we are describing the simultaneous presence of intralobar sequestration (ILS) in the lower lobe of the left lung and extralobar sequestration (ELS) which is positioned on the aortic arch, in a 53 years old man. Two years post surgery, there is no recurrence or any patomorphological and functional disorders in the respiratory tract.