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Year : 2020  |  Volume : 63  |  Issue : 1  |  Page : 116-118
Bronchogenic cyst presenting as content of omphalocele – A case report

Department of Pathology, Kempegowda Institute of Medical Sciences, Bengaluru, Karnataka, India

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Date of Web Publication31-Jan-2020


Bronchogenic cyst (BC) is a very rare congenital anomaly occurring due to budding of the primitive foregut, and its common location is the posterior mediastinum. BC when diagnosed prenatally can be treated if it is encroaching on the development of lungs. BC has been reported in other locations such as cervical, thoracic, abdominal sites and also as subcutaneous lesions. Omphalocele is a congenital malformation occurring due to a central defect in the abdominal wall with herniation of the viscera. The nonentity documented here was found in a female fetus with 20 weeks of gestational age. The mother was a primigravida who had antenatal ultrasound scan rendering diagnosis of a live fetus having abdominal wall defect with omphalocele. This case is exceptionally rare as the content of omphalocele was BC having a classical wall lined by pseudostratified ciliated columnar epithelium overlying band-like cartilage. The extensive search in the literature did not reveal another similar case.

Keywords: Bronchogenic cyst, congenital foregut anomalies, omphalocele

How to cite this article:
Venkatesh K, Pillarisetty K. Bronchogenic cyst presenting as content of omphalocele – A case report. Indian J Pathol Microbiol 2020;63:116-8

How to cite this URL:
Venkatesh K, Pillarisetty K. Bronchogenic cyst presenting as content of omphalocele – A case report. Indian J Pathol Microbiol [serial online] 2020 [cited 2023 Jan 30];63:116-8. Available from:

   Introduction Top

Omphalocele is a congenital malformation occurring due to a central defect in the abdominal wall with herniation of the viscera covered by the umbilical sheath. The incidence of omphalocele is 1 in 6000 births.[1] The etiology is unknown as most of these cases are sporadic and rarely hereditary.[2] Bronchogenic cyst (BC) is a rare developmental abnormality occurring due to budding of the primitive foregut between the third and sixth week of embryonic life, and the reported incidence is 1/42000.[3] Depending on the location of the budding, they are found in the cervical, thoracic, and abdominal locations.[4] The tracheobronchial tree is commonly affected, and BC presents as an extra pulmonary lesion mostly in the posterior mediastinum. It has been reported in other locations such as a thoracic cavity, in the neck, presternal, suprasternal, shoulder regions, and very rarely in the retroperitoneum. BC presenting as content of omphalocele in a baby documented here is the first of its kind, as extensive search in the literature did not reveal any similar case.

   Case Report Top

An unregistered case of a 22-year-old primigravida with a history of 20 weeks of gestation was admitted with labor pains in our hospital. As per the clinical history, she had got an ultrasound scan done twice, first at 10 weeks and the second in 18 weeks of gestation with an antenatal checkup. Scans reported a live fetus having an anterior abdominal wall defect in the umbilical region suggesting an omphalocele, and there were no other abnormalities or diaphragmatic defect. She was advised to undergo investigations to rule out genetic abnormalities; however, she had not yet done these tests. She had a spontaneous abortion; the baby was premature and did not survive. The baby had an omphalocele and was sent for an autopsy with the consent of the parents.

We received a dead female baby weighing 695 grams with crown-rump length measuring 18.0 cm. External examination revealed an anterior abdominal wall defect with an omphalocele in the umbilical region. On cutting open the herniated mass, a polypoidal structure was found within the omphalocele measuring 3.5 × 2.5 × 2.0 cm, encased in the umbilical sheath. The cut surface of this mass showed a closed cystic structure having a thick wall with a smooth glistening inner surface and thin mucoid material in the cavity [Figure 1]. It had a stalk-like structure with blood vessels in loose connective tissue attached to the peritoneum. The baby had an imperforate anus, and there were no other abnormalities. Multiple bits were processed, and Haematoxylin and Eosin stained sections were studied.
Figure 1: (a) Gross picture showing central abdominal defect with omphalocele, (b) polypoidal structure is seen within omphalocele encased by a sheath of an umbilical cord, and (c) cut surface showing thick walled cyst with glistening mucoid inner surface

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Histopathologically, the cystic mass showed wall typically resembling that of tracheo-bronchial tissue having pseudostratified ciliated columnar epithelial (PCCE) lining overlying connective tissue and a band-like cartilage [Figure 2]. Seromucinous glands in a loose connective tissue with a few smooth muscle cells were also seen. Multiple bits were studied, and no other types of tissues were seen. All the other organs, placenta and umbilical cord, were histologically normal for the gestational age.
Figure 2: Microphotographs of cystic mass showing (a) cyst wall lined by typical pseudostratified ciliated columnar epithelium with an underlying band-like cartilage resembling the bronchial wall (Haematoxylin and Eosin, ×10), (b) band-like cartilage, and (c) cyst wall lined by PCCE (Haematoxylin and Eosin stain, ×40)

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   Discussion Top

The abdominal wall defects include omphalocele (exomphalos) and gastroschisis, having an international incidence of 1.5 – 3/10000 births and 1/4000 – 6000 births, respectively. The abdominal wall develops by the fusion of fourfolds, namely, cephalic fold forming the thoracic and the epigastric part, caudal fold developing into the perineum, bladder and hypogastric portion, and the side walls formed by two lateral abdominal folds. The umbilical ring is formed at the center after the two lateral folds meet in the 4th week of embryonic life. Primitive gut increases in size by the 6th week causing herniation through an umbilical ring and then undergoes rotation and reintegration by the 10th week. The aetiology of abdominal wall defects, namely, “gastroschisis” and “omphalocele,” is not well understood, and it is thought to be due to an interruption in the process of mesenchymal differentiation.[2] Omphalocele is usually associated with trisomy 13, 18, and 21 in up to 30% of cases, increased maternal age more than 30 years, and there is no association with the socioeconomic conditions. Low maternal age of less than 20 years and poor socioeconomic conditions along with the use of aspirin, ibuprofen, pseudoephedrine, and other drug intakes in the first trimester, abuse of alcohol, and tobacco smoking have been attributed in the causation of gastroschisis.[5]

The parietal wall defect in omphalocele is central in a location with the diameter of the herniated viscera measuring from 2 cm to a large mass covering the entire abdominal surface. The viscera are covered by membranes comprising amniotic membrane, Wharton jelly, and peritoneum. Contents include small and large intestines, liver, stomach, and also spleen rarely. Gastroschisis is comparatively a smaller defect of 3 to 4 cm, paraumbilical in location, and commonly on the right side of the umbilical cord. Gastric foregut cystic developmental malformations lined by PCCE have been reported in adults as mass lesions in the wall of the stomach.[6]

The first case of BC was described by Meyer et al. in 1859. BC was defined by the following criteria: the cyst wall lined by PCCE together with the presence of at least one of the following: cartilage, smooth muscle or seromucinous glands.[3] Intra-abdominal BCs probably arise from an abnormal budding of the primitive foregut with migration into the abdomen before the fusion of pleuroperitoneal membranes that separate the thoracic and abdominal cavities.[4] It has been proposed that all developmental anomalies including gastric duplication cyst lined by PCCE and BC without cartilage and glandular tissue should be grouped under the heading of “foregut cystic malformations,” as they all share a common origin from the foregut and differ from each other in migration, location, and degree of differentiation.[7]

   Conclusion Top

BC is a very rare congenital anomaly compared to an omphalocele, and it should be considered in the differential diagnosis of abdominal masses. As per the available literature, BC presenting as the content of omphalocele is a nonentity and needs documentation.

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.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Singal R, Garg LN, Singal RP, Gupta S, Shahi SR, Singhal S, et al. Omphalocele and gastroschisis associated with multiple congenital abnormalities. J Med Life 2011;4:295-6.  Back to cited text no. 1
Klein MD. Congenital defects of the abdominal wall. In: Grosfeld JL, O'Neill JA Jr, Coran AG, Fonkalsrud EW, Caldamone AA, editors. Textbook of Paediatric Surgery. 6th ed. Philadelphia: Mosby Elsevier; 2006. p. 1157-71.  Back to cited text no. 2
Sumiyoshi K, Shimizu S, Enjoji M, Iwashita A, Kawakami K. Bronchogenic cyst in the abdomen. Virchows Arch A Pathol Anat Histopathol 1985;408:93-8.  Back to cited text no. 3
Coselli MP, de Ipolyi P, Bloss RS, Diaz RF, Fitzgerald JB. Bronchogenic cysts above and below the diaphragm: Report of eight cases. Ann Thorac Surg 1987;44:491-4.  Back to cited text no. 4
Curry JI, McKinney P, Thornton JG, Stringer MD. The aetiology of gastroschisis. BJOG 2000;107:1339-46.  Back to cited text no. 5
Geng YH, Wang CX, Li JT, Chen QY, Li XZ, Pan H. Gastric foregut cystic developmental malformation: Case series and literature review. World J Gastroenterol2015;21:432-8.  Back to cited text no. 6
Sharma S, Nezakatgoo N, Sreenivasan P, Vanatta J, Jabbour N. Foregut cystic developmental malformation: New taxonomy and classification--unifying embryopathological concepts. Indian J Pathol Microbiol 2009;52:461-72.  Back to cited text no. 7
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Correspondence Address:
Kusuma Venkatesh
No. 28, 2nd Cross, Maruthi Seva Nagar, Banasawadi Road, Bengaluru - 560 033, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_841_18

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