• Users Online: 2525
  • Print this page
  • Email this page

 Table of Contents  
Year : 2021  |  Volume : 7  |  Issue : 1  |  Page : 67-69

Intradiaphragmatic extralobar pulmonary sequestration accompanying eventration of diaphragm

Department of Pediatric Surgery, IGIMS, Patna, Bihar, India

Date of Submission09-Jan-2021
Date of Decision14-Mar-2021
Date of Acceptance17-Mar-2021
Date of Web Publication12-Feb-2021

Correspondence Address:
Sandip Kumar Rahul
Department of Pediatric Surgery, IGIMS, Patna, Bihar
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jigims.jigims_7_21

Rights and Permissions

Pulmonary sequestrations (PSs) are rare anatomical aberrations of pulmonary development. We describe an unusual case of intradiaphragmatic extralobar PSs associated with eventration of the diaphragm on the left side. This association complicated the clinical presentation and intraoperative management of the patient.

Keywords: Diaphragm, eventration, pulmonary sequestration

How to cite this article:
Keshri R, Yadav R, Thakur VK, Chaubey D, Kumar V, Rahul SK. Intradiaphragmatic extralobar pulmonary sequestration accompanying eventration of diaphragm. J Indira Gandhi Inst Med Sci 2021;7:67-9

How to cite this URL:
Keshri R, Yadav R, Thakur VK, Chaubey D, Kumar V, Rahul SK. Intradiaphragmatic extralobar pulmonary sequestration accompanying eventration of diaphragm. J Indira Gandhi Inst Med Sci [serial online] 2021 [cited 2022 Aug 12];7:67-9. Available from: http://www.jigims.co.in/text.asp?2021/7/1/67/318928

  Introduction Top

Intradiaphragmatic extralobar pulmonary sequestration (IDEPS) represents a unique subset of pulmonary sequestrations (PSs), which often increases the complexity of presentation and adds to the diagnostic dilemma.[1] For definitive management, excision of a portion of diaphragm along with the lesion is necessary. Awareness about this unique anatomy is emphasized upon by presenting this unique case where it was also associated with a left diaphragmatic eventration.

  Case Report Top

A 9-month-old male child presented with a history of respiratory distress associated with chest retractions and fever to the outpatient clinic for the last 2 weeks. He had a history of similar episodes in the past 6 months. On examination, he was tachycardic and tachypneic with a respiratory rate of 54/min. He was pale, anicteric, and weighed 4750 g. Abdomen was scaphoid and chest auscultation revealed decreased breath sounds in the left lower chest. On chest X-ray, left dome of the diaphragm appeared elevated and there was evidence of compression of ipsilateral lung and contralateral mediastinal shift [Figure 1]. Contrast-enhanced computed tomography of the chest and abdomen revealed left-sided eventration of the diaphragm with location of abdominal viscera in the thorax. There was also an evidence of a supradiaphragmatic mass adherent to the diaphragm with separate blood supply from the descending aorta but without any communication with the tracheobronchial tree, suggesting a diagnosis of extralobar PS [Figure 2].
Figure 1: X-ray chest findings

Click here to view
Figure 2: Contrast-enhanced computed tomography of the chest and abdomen (coronal section)

Click here to view

Considering the nature of the lesion and because its blood supply was from the aorta just below the diaphragm and also due to the presence of lots of bowel loops in the left thorax, it was decided to perform surgery via the abdominal route to reduce the contents and have better control of the vascular pedicle before excising the mass. After counseling the parents about the nature of the deformity and the surgical procedure, the child underwent exploration through a left subcostal incision. Stomach, several small intestinal bowel loops, and transverse colon were found at an abnormally high location in the left chest. After reducing them, an intact thin-walled flabby diaphragm was found on the left side, but there was a well-defined vascular pedicle entering the chest through a small defect medially in the diaphragm. This necessitated increasing the size of the defect to palpate an intrathoracic mass adherent to the left diaphragm partially and free from the lung lobes on the left side [Figure 3]. After ligation of the vascular supply to this structure, it was excised en mass along with a portion of diaphragm. The resulting defect along with the rest of the redundant diaphragm was repaired with interrupted prolene sutures [Figure 4]. The child did well in the postoperative period and was discharged on full oral diet on the 7th postoperative day. Biopsy revealed features of bronchoalveolar tissue in the excised mass, confirming a diagnosis of extralobar PS. The child has been asymptomatic on follow-up visits and after 12 months of surgical repair.
Figure 3: Intraoperative picture showing intradiaphragmatic extralobar pulmonary sequestration and diaphragmatic eventration

Click here to view
Figure 4: Intraoperative picture after lesion excision and repair of the left diaphragm

Click here to view

  Discussion Top

Embryologically, lungs bud from the developing foregut.[2] Abnormal budding and separation of such abnormal buds from the normal tracheobronchial pathway, by definition, lead to a PS.[2] These anomalies have a separate systemic blood supply and can present with either an “intralobar” or “extralobar anatomy.”[3]

Extralobar PS merits complete excision because they can often be confused with malignant masses or have mass effect over adjacent tissue, leading to associated symptoms.[4] These lesions can also be the seat of repeated infections and lead to tissue adhesion, which may make it difficult to separate the sequestration tissue from the diaphragm. Hence, several investigators have recommended early surgery.[1]

Extralobar PS can occur in thoracic or in extrathoracic sites such as abdomen.[1],[4] Such lesions can be approached through the thorax or abdomen, depending upon its location. However, lesions that are adherent to the diaphragm may be approached either way. However, other than approach to surgery, IDEPS leads to diagnostic difficulty at presentation.[1],[5] Several investigators have addressed these issues.[1] Preoperative imaging may not always identify the location of PS as thoracic, abdominal, or intradiaphragmatic and therefore guide the approach to surgery.[3] Hence, the use of minimal invasive techniques often resolves this dilemma and is worth considering.

Literature suggests that a significant portion of diaphragmatic eventration is associated with PS.[6] However, such associations may have several anatomical variations and therefore need careful preoperative imaging to identify such variations in anatomy. Both intralobar and extralobar PSs have been described with eventration of diaphragm.[7],[8],[9]

In our case, preoperative imaging showed features of left diaphragmatic eventration with displaced abdominal viscera along with a supradiaphragmatic extralobar PS with a conspicuous blood supply. We, therefore, had to deal with abnormal anatomy on either side of the diaphragm and therefore decided to have an abdominal approach followed by thoracic exploration via the diaphragm because any way diaphragm would need repair for eventration. Intraoperatively, finding the lesion with vascular supply from beneath the diaphragm and lesion being partially adhered to it, both could be dealt with precisely by the abdominal approach.

  Conclusion Top

Diaphragmatic eventration and extralobar PS, both entities merit extensive radiological assessment to reveal the anatomic variations and guide the approach to surgery for appropriate management. IDEPS represents a unique subset where a portion of involved diaphragm needs to be excised along with PS.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Chun H, Gang Y, Xiaochun Z, Jin T, Bo X, Limin W, et al. Diagnosis and management of intradiaphragmatic extralobar pulmonary sequestration: A report of 11 cases. J Pediatr Surg 2015;50:1269-72.  Back to cited text no. 1
Sade RM, Clouse M, Ellis FH Jr. The spectrum of pulmonary sequestration. Ann Thorac Surg 1974;18:644-58.  Back to cited text no. 2
Nijagal A, Jelin E, Feldstein VA, Courtier J, Urisman A, Jones KD, et al. The diagnosis and management of intradiaphragmatic extralobar pulmonary sequestrations: A report of 4 cases. J Pediatr Surg 2012;47:1501-5.  Back to cited text no. 3
Franko J, Bell K, Pezzi CM. Intraabdominal pulmonary sequestration. Curr Surg 2006;63:35-8.  Back to cited text no. 4
Meier AH, Eggli KD, Cilley RE. Intradiaphragmatic extralobar sequestration-a rare pulmonary anomaly. J Pediatr Surg 2009;44:e27-9.  Back to cited text no. 5
Groff DB, Caprio A, Behrle F. Early repair of diaphragmatic hernia to correct associated anomalies. Am Surg 1977;43:610-2.  Back to cited text no. 6
Kuo HC, Chang CY, Leung JH. Pulmonary sequestration and diaphragmatic eventration in a 6-month-old infant. Pediatr Neonatol 2012;53:63-7.  Back to cited text no. 7
Dieltjens A, Naulaers G, Vandeven K, Coosemans W, Moerman P, Devlieger H. Right diaphragmatic eventration associated with intralobar pulmonary sequestration: A case report. Eur J Pediatr Surg 2000;10:140-1.  Back to cited text no. 8
Canty TG. Extralobar pulmonary sequestration. Unusual presentation and systemic vascular communication in association with a right-sided diaphragmatic hernia. J Thorac Cardiovasc Surg 1981;81:96-9.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

  In this article
Case Report
Article Figures

 Article Access Statistics
    PDF Downloaded50    
    Comments [Add]    

Recommend this journal