|Year : 2014 | Volume
| Issue : 1 | Page : 49-51
Management of adult intralobar pulmonary sequestration: A novel approach
Battal O Aldosary1, Yasser M Aljehani2, Nawal I Algubaisi1, Yahya A. A. Sabaah1, Khaled M Alkattan2
1 Department of Medicine, Pulmonary Medicine, Riyadh Armed Forces Hospital, Riyadh, Saudi Arabia
2 Department of Surgery, Thoracic Surgery Section, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
|Date of Web Publication||7-Mar-2014|
Battal O Aldosary
Department of Medicine, Consultant Adult Pulmonary, Riyadh Armed Forces Hospital, P.O. Box 7897, R-756, Riyadh 11159
Pulmonary sequestration is a known congenital pulmonary malformation. It has been classified into intralobar and extralobar types. We report a case of a symptomatic adult intralobar pulmonary sequestration which was managed in a rather unconventional approach. Such an approach, arterial embolization, has clearly shown to be safe with good outcome in infants and pediatric population. The patient made a full recovery with good outcome. Such case reports show feasibility and safety of embolization as a less invasive management option for adult pulmonary sequestration.
ملخص البحث :
تبين هذه الحالة كيفية العلاج لمريض بالغ يعاني من حبس داخل الفصيص . تعتبر هذه الحالة تشوه خلقي وقد قسمت إلى نوعين داخل وخارج الفصيص. وقد عولج المريض بطريقة غير تقليدية وذلك بالانصمام الشرياني والذي أثبتت فعاليته وسلامته لدى الأطفال. تعافى المريض بشكل جيد. وتبين هذه الحالة أن إستعمال هذه الطريقة العلاجية آمنة وسهلة ويمكن استخدامها لدى المرضى البالغين.
Keywords: Embolization, intralobar, sequestration
|How to cite this article:|
Aldosary BO, Aljehani YM, Algubaisi NI, Sabaah YA, Alkattan KM. Management of adult intralobar pulmonary sequestration: A novel approach. Saudi J Med Med Sci 2014;2:49-51
|How to cite this URL:|
Aldosary BO, Aljehani YM, Algubaisi NI, Sabaah YA, Alkattan KM. Management of adult intralobar pulmonary sequestration: A novel approach. Saudi J Med Med Sci [serial online] 2014 [cited 2017 Mar 29];2:49-51. Available from: http://www.sjmms.net/text.asp?2014/2/1/49/128454
| Introduction|| |
Pulmonary sequestration (PS) has been defined as a nonfunctional dysplastic lung tissue that embryologically does not connect to the tracheobronchial tree and fed by a systemic arterial circulation.  Pryce coined the term (PS) back in 1945. Many theories have been generated regarding the pathogenesis as well as the classification. The spectrum of clinical presentation is governed by the type of sequestration and several other variables.
| Case Report|| |
We report a case of a 32-year-old male, who is not known to have any medical illnesses. He presented with hemoptysis and shortness of breath (SOB) for 2 days duration. No constitutional symptoms, history of bruising or bleeding tendencies, and arthralgia or skin rashes were found. He reported two episodes of mild hemoptysis in the preceding 2 years. Other past, medical, surgical, drug, family, social, histories as well as review of systems were unremarkable. Upon examination, the patient had stable vital signs. His cardiac exam was within normal limits. His chest exam revealed normal expansion with equal air entry and vesicular breathing. No wheezes or added sounds could be detected. The rest of his systemic exam was unremarkable. Chest X-ray revealed no gross pathology initially. Laboratory investigations were normal. ECG was normal. His hemoptysis continued despite these measures. Computed tomography (CT) scan of the chest revealed right-sided lower lobe sequestration with feeding systemic artery from the descending thoracic aorta [Figure 1]. On further investigation, echocardiography was normal with no evidence of shunting. The treating team elected to embolize the feeding artery by Amplatzer vascular plugs and stainless steel coils through a radial artery approach [Figure 2]a]. The patient condition improved with resolution of his hemoptysis. His follow-up after 8 weeks also confirmed resolution of his hemoptysis with some occasional pleuritic chest pain. He remained free of hemoptysis for 6 months. The angiographic assessment demonstrated persistent occlusion of the feeding arterial supply [Figure 2]b.
|Figure 1: CT chest (mediastinal window) demonstrating the feeding arterial supply|
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| Discussion|| |
In 1777, Huber raised the possibility of systemic arterial supply to a sequestrated lung tissue. Then came the report of Rektorzik in 1861 describing a lung mass with no connection to the tracheobronchial tree.  The better understanding of this condition has lead Clements in 1987 to coin the term bronchopulmonary malinosculation as synonymous to pulmonary sequestration. It is considered a rare congenital pulmonary malformation of the lower respiratory tract. It accounts for 0.15-6% of all congenital pulmonary malformations. It is found in 1-1.8% of all pulmonary resections.  Several theories have been generated over the years since 1777: The fraction theory in 1861 by Rokitonski and Rektorzik, the excess theory in 1878 by Ruge, the traction theory in 1945 by Pryce, the obtained anomaly theory in 1959 by Gebaure and Mason. The idiopathic theory is believed by many authors. PS is classified anatomically into intralobar pulmonary sequestration (ILS) and extralobar pulmonary sequestration (ELS) types based on pleural coverage. Some variables have been added such as age, localization, origin of the arterial circulation, venous drainage, symptoms, and co-existing anomalies to aid in differentiating these types.  Pryce made the first classification; it included type 1; systemic arterial supply to a normal segment, type 2; systemic arterial supply to a sequestered segment as well as a normal segment, type 3; systemic arterial supply only to a sequestered segment.  Moreover, another rare variant has been reported; bronchopulmonary foregut malformation (BPFM) where the sequestered segment connects to the gastrointestinal tract. The ILS type constitutes 75-86% of all sequestration cases while ELS constitutes 14-25%.  They are more in the left side, with male predominance (4:1 ratio) is seen in the ELS type. ILS receives arterial supply through thoracic aorta (73%), abdominal aorta (21%) or intercostal arteries in 4% of cases. Venous drainage is mainly to pulmonary vein (95%) or azygous-hemiazygous veins (5%) of cases. Associated anomalies are seen more with ELS (65%) and (6-12%) with ILS.  In regard to presentation, ELS presents in early childhood while ILS in adulthood.  A total of 30% are incidentally found and the symptomatic type is mainly ILS. The diagnosis relies mainly on identifying the feeding systemic artery. The gold standard remains angiography ,, but with the improvement and development of CT and MRI, these noninvasive modalities reach accuracy of diagnosis in 90% of ILS cases. It has been reported that MRI may reach the same sensitivity as the CT scans. Some reports of radionuclides angiography in selected cases.  The treatment is considered to be surgical and it is recommended because of the risk of complications such as hemoptysis, pulmonary hypertension or even heart failure. Moreover, since the introduction of embolization by Park in 1998 in pediatric groups, this option got more popular.  The sequestration process leads to friable vessels due to inflammation and some develop atherosclerosis and even aneurysms. Embolization reduces the risk of hemorrhage especially intraoperatively. It makes dissection easier and safer according to some reports.  It is reported as a safe procedure in pediatrics but reports in the adult population are exceptional and mainly for type 1 while surgery is the gold standard for type 2 and 3.  Embolization is recommended 2-6 weeks before resection if planned.  Several reports demonstrated complete involution of the sequestrated segment in infants and pediatric population. The concern about incomplete occlusion of the feeding artery raises the possibility of recurrence which is reported in 25-47% of pediatric population. No figure is produced in the adult population. The reason for such occlusion failure is distal deployment to feeding artery. Other possibilities include the development of collaterals. For larger feeding arteries, coils might migrate adding failure to the outcome.  Amplatzer vascular plugs are recommended in such cases of large feeders. In conclusion, since the introduction of embolization as a management option for pulmonary sequestration in infants and pediatric population, and above all, the acceptable success rate, it is the time to consider such an approach in the adult population especially in the setting of surgery refusal or comorbidities.
| References|| |
|1.||Pekcolaklar A, Citak N, Metin M, Kok A, Celikten A, Sayar A, et al. The rare congenital anomaly of pulmonary sequestration experience and review of literature. Indian J Thorac Cardiovasc Surg 2010;26:251-4. |
|2.||Diks J, Schütte PR, Cheung D, Schnater JM. Treatment of pulmonary sequestrations by means of endovascular embolization: Future or fashion?. Case Rep Med 2011;2011:173918. |
|3.||Saxena P, Marshall M, Ng L, Sinha A, Edwards M. Preoperative embolization of aberrant systemic artery in sequestration of lung. Asian Cardiovasc Thorac Ann 2011;19:357-9. |
|4.||Leoncini G, Rossi UG, Ferro C, Chessa L. Endovascular treatment of pulmonary sequestration in adults using Amplatzer vascular plugs. Interact Cardio Vasc Thora Surg 2011;12:98-100. |
|5.||Ulys A, Samalavicius NE, Cicenas S, Petraitis T, Trakymas M, Sheinin D, et al. Extralobar pulmonary sequestration. Int Med Case Rep J 2011;4:21-3. |
[Figure 1], [Figure 2]