Abstract Introduction Pulmonary sequestration is a rare congenital malformation characterized by nonfunctioning lung tissue with anomalous systemic blood supply. It typically presents in childhood with recurrent infections. However, it may also present in adulthood as a life-threatening haemorrhage due to rupture of high-pressure systemic arterial blood supply. Case Presentation A 37-year-old previously healthy male presented to the emergency department with acute hemoptysis and upper back pain following two days of worsening cough. Initial examination revealed an increasingly pale appearance, and decreased breath sounds over the left lower chest. Computed tomography demonstrated an intrapulmonary sequestration of the left lower lobe with an anomalous feeding vessel from the thoracic aorta showing active extravasation, resulting in both hemoptysis and a large left hemothorax. Emergency chest tube placement yielded two litres of blood. While in the emergency department, the patient developed hemodynamic instability with tachycardia and hypotension. Massive transfusion protocol was initiated, and the patient underwent emergent left thoracotomy in the operating room. Intraoperative findings confirmed significant haemorrhage from the anomalous systemic vessel, which was successfully controlled and divided. A left lower lobectomy was completed. The patient recovered well and was discharged on postoperative day six. Conclusions This case illustrates a unique presentation of pulmonary sequestration with concurrent intrabronchial and pleural haemorrhage, demonstrating the potential for catastrophic bleeding from anomalous systemic vasculature. The successful outcome highlights the importance of prompt recognition and immediate surgical intervention in managing this life-threatening complication.

BackgroundPulmonary sequestration is a rare pulmonary malformation, with intralobar pulmonary sequestration being the most common subtype. Lobectomy has generally been performed for its treatment, owing to unclear boundaries of the lesion. However, recent reports have introduced lung resection using intravenous indocyanine green (ICG) as a treatment for pulmonary sequestrations.Case descriptionA 34-year-old woman presented with chest pain, and enhanced chest computed tomography (CT) displayed a solid mass of 4.5 × 3.1 cm in the right S10 area. An aberrant artery was found running from the celiac artery through the diaphragm to the thoracic cavity. The patient was diagnosed as having pulmonary sequestration Pryce type III, and surgical resection was performed. Intrathoracic findings demonstrated that the precise area of the pulmonary sequestration could not be clearly identified, and a 5-mm aberrant artery was present in the pulmonary ligament. Following the separation of the aberrant artery, intravenous injection of ICG clearly delineated the border between the normal lung tissue and the pulmonary sequestration. Wedge resection was then performed without any postoperative events, and the pathological diagnosis was also pulmonary sequestration.ConclusionsWe herein reported a case of a patient who underwent sublobar resection for intrapulmonary sequestration using intravenous ICG injection, together with a literature review. Our case suggests that a comprehensive understanding of abnormal vessels and pulmonary vasculature in pulmonary resection for intrapulmonary sequestrations, complemented with the use of ICG, might potentially avoid unnecessary pulmonary resection and enable sublobar surgical resection.

BACKGROUND: Pulmonary sequestration is one of the most common malformations of the lungs in clinical practice. However, difficulties are possible in the interpretation of the radiological data, which leads to a violation of the examination technique in such patients and, accordingly, to the lack of success of the therapy and proper routing.
 AIM: To demonstrate the clinical case of a patient with intralobar pulmonary sequestration, describing the characteristic radiographic picture, and suggesting research methodology for the identified features of the aberrant vessel.
 METHODS: Clinical and radiological data of a 27-year-old patient with acute respiratory symptoms and suspected abscessed pneumonia based on X-ray and computed tomography (CT) findings were presented. Due to the absence of clinical and radiological dynamics, atypical CT semiotics of abscesses, CT angiography of the thoracic aorta was performed after intravenous contrast enhancement with 80 ml of iodine-containing preparation (350 mg/mL).
 RESULTS: A pulmonary tissue thickening was revealed in the lower left lobe with lumpy clear contours, which consisted of a system of cystic cavities with contents, particularly well differentiated after contrast enhancement. On the native study, an additional vascular structure following from the subdiaphragmatic space was traced paravertebrally, which was the reason for extending the CT angiography scanning area to the epigastric region of the abdomen. A large-caliber arterial vessel was detected, which departed as a branch from the celiac trunk and passed to the above-described thickening of the pulmonary tissue. The combination of these signs is typical for intrapulmonary sequestration.
 CONCLUSIONS: The correct interpretation of the identified changes allowed the patient to be routed to a hospital with a thoracic surgery department, since the sequestration must be removed in the vast majority of cases to prevent recurrent inflammatory episodes. Extension of the scanning area below the diaphragm may be useful, since part of the sequesters is supplied with blood from the abdominal aorta and its visceral branches. This will prevent the need for repeat CT angiography, and information about the source of the blood supply is extremely important for surgeons.

Pulmonary sequestration is a complex malformation of the lungs, which is based on a violation of the development of their bronchopulmonary and vascular components. The multifactorial nature of morphological and functional disorders is clinically manifested by respiratory distress syndrome and heart failure with a threat of a critical outcome for a newborn child, despite the sufficient development of the respiratory system as a whole. Purpose - optimization of diagnosis and treatment of lung sequestration in children based on the study of clinical and diagnostic data, as well as the results of surgical treatment. Materials and methods. The study included 18 patients aged from 8 days to 18 years, including newborns - 6, children from 1 to 12 months - 5, from 1 to 3 years - 3, from 7 to 9 years - 2 and from 15 to 18 years - 2. Median age was 4.5 months. Research methods included evaluation of clinical symptoms, chest X-ray, contrast-enhanced CT, and angiography. Prenatal diagnosis was carried out using ultrasonography and fetal MRI. In all cases, open surgical treatment was used. Results. Intrapulmonary sequestration was diagnosed in 13 (72.2%) patients, extrapulmonary - in 5 (27.8%), p=0.082. Left-sided localization in 11 (61.1%) patients, right-sided localization in 7 (38.9%) patients, р=0.3. Associated malformations had 9 (50%) patients: polycystic (n=4) or hypoplasia (n=2) of the lung, diaphragmatic hernia (n=3), pericardial defect (n=1), thoracic dystopia of the kidney (n=2), Waardenburg syndrome (n=1), ventricular septal defect (n=1), innominate artery tracheal compression (n=1). Symptomatic course was noted in 15 (83.3%) patients, asymptomatic - in 3 (16.7%) cases (p=0.021). Main symptoms were as follows: pulmonary bleeding, hemophthisis, respiratory and hemodynamic disorders, signs of inflammation. Lobectomy (n=8), atypical segmental lung resection (n=4) and transection of aberrant vessels (n=1) were performed for intrapulmonary sequestration, and sequestrectomy (n=4) for extrapulmonary sequestration. In cases of associated diaphragmatic hernia (n=3), simultaneous diaphragmatic plasty was performed, including using a non-free pericardial flap (n=1) or a PTFE patch (n=1), and in case of innominate artery compression of the trachea, aortopexy (n=1). In 17 (94.4%) cases, a positive result of surgical treatment was noted. Postoperative complications (intrathoracic bleeding) and lethality were observed in 1 (5.6%) case. Patients were examined in the long-term period from 2 months to 30 years after surgery. Conclusions. Surgical correction of pulmonary sequestration is appropriate as the diagnosis is made, mainly in the neonatal period. Given the complexity of the pathology, especially in the presence of associated malformations, and the expediency of early correction in the neonatal period, thoracotomy access should be preferred as safer for sequential division of arterial and then venous vessels, and for atypical segmental lung resection or lobectomy, depending on peculiarities of sequester. The presence of associated defects requires simultaneous correction. In the case of associated diaphragmatic hernia, autopericardial defect plastics or a synthetic patch are appropriate as an alternative to simple suturing. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of the participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors. Keywords: congenital malformations of the lungs, lung sequestration, surgical treatment, children.

BackgroundSeveral reports have documented that the pulmonary sequestration is in communication with the gastrointestinal tract and the concept of bronchopulmonary foregut malformation (BPFM) has become more widespread. However, there are few reports of the sequestration associated with the pancreas derived from the foregut. We describe the history and pathophysiology of BPFM including pancreatic tissue in a male infant with respiratory distress.Case presentationA male patient was born at 38 weeks of gestation and weighed 2752 g at birth. He developed pneumonia and was hospitalized at 3 months of age. Chest radiographs and CT scans led to the diagnosis of a lung abscess in the left lower intralobar pulmonary sequestration with aberrant arteries from the abdominal cavity. At 4 months of age, when the abscess had resolved, left lower lobectomy and the resection of the intralobar sequestration were performed. The pulmonary sequestration was conjoined with the esophagus. A fistula was found between the lower esophageal wall and the pulmonary sequestration. An additional small segment of the esophageal wall was excised. Histologically, the mediastinal surface of the sequestration tissue contained pancreatic tissue. Furthermore, esophageal and gastric tissue, cartilage tissue, and ciliated epithelium were confirmed. A definitive diagnosis of BPFM was made.ConclusionsWe postulated the rare case of a communicating BPFM with intrapulmonary sequestration on one end and the esophagus on the other forming a mass lesion, which included ectopic pancreatic tissue in a male infant.

Pulmonary saequestration is a rare congenital malformation characterized by a dysplastic portion of lung parenchyma supplied by an anomalous artery originating from the aorta or its branches. The worldwide incidence of pulmonary sequestration among all congenital lung malformations in children ranges from 1.5% to 6.4%. There are two main types of pulmonary sequestration according to the localization of the malformation, i.e., intrapulmonary sequestration (dysplastic tissue located inside a lobe of the normal lung) and extrapulmonary sequestration. Our case presentation aims to make physicians aware of this rare anomaly which may be difficult to diagnose because of its oligosymptomatic course prior to first presentation. We present the case of a 10-year-old girl who suffered from a second episode of prolonged pneumonia of the left lower lobe. Contrast-enhanced-computed-tomography (CT) scan of the thoraco-abdominal segment of the aorta and its branches revealed intrapulmonary sequestration localized at the left lower lobe of the lung. The intrapulmonary sequester was perfused by a large artery arising from the celiac trunk. The girl underwent open surgery with ligation of the anomalous feeding artery and atypical pulmonary resection of the affected area of the left lower lobe. Postoperatively, the child recovered without any complications.

INTRAPULMONARY SEQUESTRATION IN AN ADULT

Intralobar pulmonary sequestration is a rare congenital malformation, usually diagnosed later in childhood or adolescence. We report a case who presented with tachypnea and was diagnosed at 2 months of age. Pulmonary sequestration is usually managed by embolization or surgical resection. Recently, preoperative embolization of aberrant arteries to minimize the risk of serious intraoperative hemorrhage has also been described. Our case was successfully treated with embolization followed by a thoracoscopic resection.

BackgroundCongenital lung malformations exist along a spectrum of pathogenesis and disease severity. Extrapulmonary sequestration (EPS), in which nonfunctional lung tissue develops without connection to the tracheobronchial tree, is one rare manifestation of this disease. Atypical vascular anatomy with a systemic feeding vessel characterizes these lesions.Case presentationA 3 day old, 37 week gestation infant underwent chest X-ray for confirmation of umbilical catheter placement and was found to have an elevated left hemidiaphragm consistent with eventration versus congenital diaphragmatic hernia. He remained asymptomatic and was evaluated as an outpatient at the age of 9 months, where CT angiogram demonstrated extrapulmonary versus intrapulmonary sequestration with a systemic feeding vessel from the left internal mammary artery.ConclusionsIt is exceedingly rare for the feeding artery to arise from the internal mammary; two such cases have been reported to date, both in adult patients. Here we present a third case of EPS with arterial supply from the internal mammary successfully treated with video-assisted thoracoscopic resection in a 9 month old infant.

Thoracoscopic surgery for pediatric benign tumors is a common procedure. However, a large incision is needed to remove large tumors from the thoracic cavity. And, for intrapulmonary sequestration in lower lobe, it sometimes needs a large incision to ligate the aberrant vessels. A muscle-sparing axillar skin crease incision (MSASCI) has been introduced for thoracic open surgery, resulting in excellent aesthetic outcomes compared with a standard incision. We herein report the utility of this MSASCI technique in thoracoscopic surgery to remove large tumors from the thoracic cavity and to ligate the aberrant vessels in intrapulmonary sequestration in lower lobe. From 2014 April to 2016 March, we performed the MSASCI technique in thoracoscopic surgeries for 5 children. Five cases were diagnosed as mediastinal masses (mature teratoma for 1 case, ganglioneuroblastoma for 2 cases, and extrapulmonary sequestration and intrapulmonary sequestration for 1 case each). The age at surgery was 32.0 ± 25.0 months (range 5-58 months). The size of the mediastinal mass was 9 × 5 × 5 cm, 4 × 3 × 3 cm, 5 × 5 × 2.5 cm, and 3 × 2.5 × 2 cm. For 4 other cases, except for the intrapulmonary sequestration case, the mass was resected under thoracoscopic surgery using only three or four 5-mm trocars and the mass was removed from the thoracic cavity using the MSASCI technique. For the intrapulmonary sequestration case, the aberrant vessels were resected under thoracoscopic surgery using only two 5-mm and one 12-mm trocars and the left lower lobectomy was performed using the MSASCI technique. All lesions were resected completely. No cases had surgical complications, none showed recurrence, and all cases demonstrated good cosmetic outcomes. Performing thoracoscopic surgery using a MSASCI technique is associated with good cosmetic outcome.

Intrapulmonary sequestration is a rare congenital abnormality occurs as a result of tracheobronchial tree malformation and can be associated with pulmonary hypoplasia and hydrops fetalis, both are considered as life threatening conditions. We report two cases with intralobar sequestration. First patient is a 30-year-old pregnant who visited our outpatient clinic for routine follow up. Ultrasonography showed a hyperechogenic lesion in the right lung of 25 weeks male fetus. In the second 25 -year- old patient, we report a hyperechogenic lesion in left lower lobe in a 26 weeks fetus, who was also on a suspicion of ventricular septal defect that was later confirmed by fetal echocardiography. Our aim is to increase the awareness of pulmonary sequestration as a differential diagnosis of intrathoracic masses and to ensure the early diagnosis during pregnancy to avoid the hazardous complications.

We report the case of a fit and healthy 41-year-old man, who presented with significant haemoptysis without a history of recurrent infections. His computed tomography scan showed a dense lesion in the left lower lobe with a feeding vessel arising from the abdominal aorta, characteristic for an intra-pulmonary sequestration. To prevent possible further haemoptysis or infections, a left lower lobectomy was performed. The histological examination showed the typical features of a sequestration. However, within the sequestration, a carcinoid tumour without atypical features was found. There was no lymph node involvement. Sequestrations are congenital lesions without communication with the bronchial tree and with a systemic blood supply. They commonly cause recurrent infection. Fatal haemoptysis has also been described, but is rare. There are very few reports of neoplastic lesions in sequestrations. This case illustrates two unusual aspects of sequestrations. Surgery offers definitive treatment for both pathologies, as opposed to embolisation.

A 39-year old man, a smoker without significant medical history, presented with vague chest pain, cough, and hemoptysis. His symptoms had started 2 weeks earlier. Radiography showed an area of decreased lucency (ground glass appearance) in the right lower lobe. CT scan confirms the presence of a triangular area of inhomogeneous parenchymatous increased attenuation mainly of ground glass with inlying bronchocoeles and tree-in-bud appearance compatible with an intrapulmonary sequestration. The arterial supply is derived from the lower thoracic aorta. The venous drainage is to the left atrium. The hemoptysis is a result of a supratherapeutic International Normalized Ratio (INR) on Sintrom intake for atrial fibrillation (AF). Treatment of sequestration consisted of a thoracoscopic lobectomy.

To analyze the characteristics and technical difficulties of complete video-assisted thoracoscopic surgery (c-VATS) for treatment of pulmonary sequestration operation. 25 cases of c-VATS lobectomy for intrapulmonary sequestration performed between January 2009 and May 2012 were reviewed. The 25 patients included 13 (52%) males and 12 (48%) females, with a mean age of 34.7 years (range, 16-62 years). Preoperative imaging by CT scan and three-dimensional reconstruction of abnormal blood vessels diagnosed 19 cases as pulmonary sequestration, misdiagnosed 1 case as pulmonary cyst syndrome, 4 cases as bronchiectasis and 1 case as benign tumor. All the patients underwent c-VATS excision, 16 in the left lower lobe, 7 in the right lower lobe, 1 in right middle lobe and 1 extralobar pulmonary sequestration. Vascular abnormality was observed intraoperative including the thoracic aorta in 20 cases, abdominal aorta in 2 cases, phrenic arteries and intercostal artery in 1 cases and thoracic aorta combined with abdominal aorta in 1 case. No conversion to open was achieved in all cases. The mean operating time was 114.2 mins (range, 78-156 mins), the mean blood loss was 228 mL (range, 50-3,000 mL), the mean duration of chest drainage was 3.2 days (range, 2-7 days) and the mean length of post-operative hospital stay was 6.6 days (range, 3-13 days). There was no mortality, without significant postoperative complications, were cured and discharged. Patients were followed up for 2-32 months, mean 21.4 months, with no recurrence. c-VATS is feasible, effective, and safe in treatment of pulmonary sequestration. It is worthy of clinical application.

Thoracoscopic lobectomy in intrapulmonary lung sequestration

Pulmonary sequestration is a rare congenital malformation of the lower respiratory tract characterized by a non-functioning mass of lung tissue that is not communicating with the normal tracheo-bronchial tree and receives its vascular supply from a systemic artery. We report the first case of intrapulmonary sequestration in Qatar, of a 22 year-old Qatari male who presented with recurrent chest infection. This case study includes a literature review.

Fig. 2. (A) Three dimensional reconstructed computed tomography image showing the venous drainage of the right lower lobe directly in the inferior vena cava (white arrow). (B) Intra-operative view with aberrant artery of the intrapulmonary sequestration (Ar) and venous drainage of the right lower lobe (RLV) directly in the inferior vena cava (IVC) just above the diaphragm. The patient underwent right lower lobectomy with uneventful recovery.

Utility of preoperative visualization for intrapulmonary sequestration in video-assisted thoracoscopic surgery

There are still few reports on thoracoscopic lobectomy in neonates. The rate of prenatally diagnosed congenital cystic lung diseases is increasing, and such diseases appear to be a good indication for thoracoscopic lobectomy. We performed a prospective trial of thoracoscopic lobectomy for all congenital cystic lung diseases in children. Complete thoracoscopic lobectomy was performed by employing carbon dioxide insufflation without single lung ventilation. The results were analyzed, comparing patients with pre- and postnatal diagnoses. Seven patients were prenatally diagnosed with congenital pulmonary airway malformations (CPAM) between January 2008 and August 2009, and all but one underwent surgery during the neonatal period (prenatal group). Nine patients underwent surgery after infection subsided due to lesions (7 CPAM, 2 intrapulmonary sequestration) postnatally identified by infection presence (median 2 years; range 15 days to 14 years) (postnatal group). The prenatal group showed a significantly lower rate of adhesions, shorter operation time, and smaller volume of blood loss. There were two conversions in the postnatal group. There were two postoperative complications (persistent air leak and phrenic nerve paralysis) in the prenatal group. The esthetic results were good. Thoracoscopic lobectomy for congenital cystic lung diseases in neonates was practicable, with good esthetic results. Dissection was easier with significantly less blood loss in patients without adhesions, even in neonates. However, this procedure necessitated highly skilled endoscopic maneuvers within a small working space.

Objective:Most mechanistic studies of pancreatitis in mice employ the secretagogue-induced model. The currently reported studies were designed to develop an alternative, and possibly more clinically relevant, mouse model of pancreatitis.Design:Na-taurocholate...