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Year : 2017  |  Volume : 60  |  Issue : 3  |  Page : 399-401
Intrapulmonary ectopic liver associated with scimitar syndrome

1 Department of Pathology, Aster Malabar Institute of Medical Sciences, Kozhikode, Kerala, India
2 Department of Pathology, Aster Malabar Institute of Medical Sciences, Kozhikode, Kerala; Department of Cardiac Sciences, Aster Medcity, Kochi, Kerala, India

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Date of Web Publication22-Sep-2017


We report a case of intrapulmonary ectopic liver associated with Scimitar syndrome. A two month old male child who presented with features of congestive cardiac failure underwent extensive cardiac and radiological investigations and was diagnosed with Scimitar syndrome. He was also found to have a mass in the lower lobe of the right lung, the possibilities being either a pulmonary sequestration or a diaphragmatic hernia. As his dyspnea progressively worsened, a right lower lobectomy was performed at the age of two years. Intraoperatively, no connecting pedicle or hernia sac was seen. The resected lung specimen showed a light brown mass enclosed by the lung parenchyma and microscopy showed cords of normal hepatocytes with portal tracts. The hepatocytes were positive for Hep Par1, and bile ducts were CK 7 positive. To the best of our knowledge, this is the first reported case of an intrapulmonary ectopic liver associated with Scimitar syndrome.

Keywords: Ectopic liver, intrapulmonary, Scimitar syndrome

How to cite this article:
Kutty SV, Lilly M, Kuruvila S, Koshy S. Intrapulmonary ectopic liver associated with scimitar syndrome. Indian J Pathol Microbiol 2017;60:399-401

How to cite this URL:
Kutty SV, Lilly M, Kuruvila S, Koshy S. Intrapulmonary ectopic liver associated with scimitar syndrome. Indian J Pathol Microbiol [serial online] 2017 [cited 2021 Jul 23];60:399-401. Available from: https://www.ijpmonline.org/text.asp?2017/60/3/399/215382

   Introduction Top

Supradiaphragmatic heterotopic liver is usually an incidental finding on the chest radiographs. Imaging techniques may help in the accurate diagnosis of this condition, thus avoiding surgical resection where it is not indicated.[1] However, resection is sometimes performed when either radiological investigations are inconclusive or as a result of misdiagnosis.[2] The preoperative differential diagnoses in our patient were pulmonary sequestration and diaphragmatic hernia. Lobectomy was done as the patient had worsening of dyspnea.

   Case Report Top

This male child was admitted at two months of age at our institution with congestive heart failure. He was evaluated extensively with radiological and cardiac investigations which included computed tomography (CT) and magnetic resonance imaging (MRI) studies of the thorax, cardiac catheterization, aortogram, and echocardiography, and was ultimately diagnosed with Scimitar syndrome. He had levocardia, right lung hypoplasia, dilated right atrium and right ventricle, moderate-sized fossa ovalis, atrial septal defect, anomalous obstructed drainage of right lower lobe pulmonary vein, interrupted inferior vena cava with hemiazygos continuation, and pulmonary artery hypertension. Aortogram revealed three blood vessels supplying the right lower lobe of the lung - one from the thoracic aorta and two from abdominal aorta. CT of the thorax showed an oval well-defined soft-tissue lesion of 4.1 cm × 3.3 cm occupying the major part of the right lower lobe. A diagnosis of pulmonary sequestration was made. However, MRI of the thorax revealed extension of the posterior segment of the right lobe of liver to the right lower chest and suggested the possibility of a diaphragmatic hernia. The patient was under regular follow-up. As he had progressive worsening of dyspnea and saturation fall, a right lower lobectomy was performed at 2 years of age. Operation notes described a solid mass situated within the lower lobe of the right lung with two major blood vessels, one medial and one lateral, seen entering the mass from below the diaphragm. These vessels were ligated and the mass was dissected off the diaphragm. We received a right lower lobectomy specimen of 7 cm × 6.5 cm × 4 cm, cut section showing a well-circumscribed pale brown mass of 4 cm × 3 cm × 2.3 cm which was completely surrounded by lung parenchyma. The mass was around 5 mm away from the diaphragmatic surface of the lobectomy specimen and 1 cm away from the hilum. Microscopy of sections from the mass showed cords of normal hepatocytes with portal tracts and sinusoids. This hepatic tissue was well delineated from the surrounding lung parenchyma. The hepatocytes were uniformly and strongly positive for Hep Par1 [Figure 1]. Based on these findings, we made a diagnosis of intrapulmonary ectopic liver.
Figure 1: (a) Lobectomy specimen showing a pale brown mass enclosed by the lung parenchyma. (b) Nodule of liver tissue with adjacent lung alveoli and a bronchiole (H and E, ×10). (c) Cords of normal hepatocytes with sinusoids and portal tract (×40). (d) The hepatocytes are strongly positive for Hep Par1 (×10)

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   Discussion Top

The liver and the diaphragm develop in close relation to each other. The central tendon of the diaphragm develops from the septum transversum, and the paired pleuroperitoneal membranes with the dorsal mesentery of esophagus between them form the dorsal portion of the diaphragm. The liver develops from the endoderm of the hepatic diverticulum, and the growth of the dorsal portion of the liver facilitates the obliteration of the pleuroperitoneal canals, resulting in the closure of these canals at the dome of the diaphragm. Hence, if thoracoabdominal connections remain open, a bud of proliferating liver tissue could migrate through the pleuroperitoneal canals into the developing pleural space above.[3],[4] The margins of the developing diaphragm could compress and separate the migrated liver tissue so that it grows above the completed diaphragm as an ectopic mass.[4] There could also be regression or atrophy of the pedicle connecting it with the abdominal liver. Another possible explanation is the development of an entirely separate liver bud independent of the main hepatic diverticulum.[5] In our case, although MRI of the chest suggested the possibility of a diaphragmatic hernia, operation notes did not specify the presence of a defect in the diaphragm. There was also no hernia sac or membrane surrounding the ectopic liver. Some authors classify heterotopic livers as ectopic or accessory based on the absence or presence of a connecting pedicle to the abdominal liver.[6] In our case, the ectopic liver did not have a pedicle and had a separate blood supply. Moreover, both macroscopic and microscopic examination revealed a liver nodule that was entirely surrounded by the lung parenchyma with a 5 mm margin from the diaphragmatic aspect of the lobectomy specimen. Based on all these findings, we diagnosed it as a case of intrapulmonary ectopic liver.

The most common location for liver heterotopia is intra-abdominal, usually in the vicinity of the abdominal liver. As they are composed of normal hepatocytes, they are prone to similar complications including development of hepatocellular carcinoma.[7] Supradiaphragmatic heterotopic livers may be associated with cardiopulmonary abnormalities. The associated cardiac anomaly in our case was Scimitar syndrome which, in its classical form, comprises a spectrum of cardiovascular and bronchopulmonary abnormalities represented by partial or total anomalous pulmonary venous drainage of the right lung to the inferior vena cava, varying degrees of right lung and right pulmonary artery hypoplasia that results in dextroversion of the heart and an anomalous systemic blood supply from the abdominal aorta to the inferior segments of the right lung.[8] Choi et al. have reported a case of right supradiaphragmatic heterotopic liver associated with intralobular sequestration of the right lung.[9] Another case report by Shapiro and Metlay shows an association of a right supradiaphragmatic heterotopic liver with Goldenhar syndrome, that is, hemifacial microsomia with cardiac and conotruncal abnormalities such as double-outlet right ventricle, hypoplastic left ventricle, ventricular septal defect, mitral valve atresia, absent ductus arteriosus, right-sided aortic arch, and infundibular pulmonary stenosis.[10] Mendoza et al. have documented an association with almost identical cardiovascular anomalies with differences being in the presence of a patent ductus arteriosus, preductal coarctation of aorta, and anomalous right pulmonary venous return to the inferior vena cava. These authors suspect a common pathogenetic pathway for the development of these cardiac anomalies and supradiaphragmatic liver heterotopia.[5],[10]

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There are no conflicts of interest.

   References Top

Cho YJ, Kim HJ, Bae YA, Jang SK, Yeon JW. Supradiaphragmatic liver confirmed by a hepatocyte specific contrast agent (Gd-EOB-DTPA): A case report. Invest Magn Reson Imaging 2015;19:52-5.  Back to cited text no. 1
Wang Y, Junlin L, Zhang WG, Chen JH, He Y, Chen JM. Accessory lobe of right liver mimicking a pulmonary tumor in an adult male. Ann Thorac Surg 2010;89:e9-10.  Back to cited text no. 2
LeRoux BT. Heterotopic intrathoracic liver. Thorax 1961;16:68-9.  Back to cited text no. 3
Bedii Salman A. Left-sided congenital diaphragmatic hernia associated with intrathoracic ectopic liver lobule. Eur J Cardiothorac Surg 2002;21:558-60.  Back to cited text no. 4
Mendoza A, Voland J, Wolf P, Benirschke K. Supradiaphragmatic liver in the lung. Arch Pathol Lab Med 1986;110:1085-6.  Back to cited text no. 5
Collan Y, Hakkiluoto A, Hästbacka J. Ectopic liver. Ann Chir Gynaecol 1978;67:27-9.  Back to cited text no. 6
Arakawa M, Kimura Y, Sakata K, Kubo Y, Fukushima T, Okuda K. Propensity of ectopic liver to hepatocarcinogenesis: Case reports and a review of the literature. Hepatology 1999;29:57-61.  Back to cited text no. 7
Bo I, Daubeney PE, Rigby ML. Uncommon variants of the scimitar syndrome in two siblings. Ann Pediatr Cardiol 2015;8:56-8.  Back to cited text no. 8
Choi SU, Kim HK, Kim J. Heterotopic supradiaphragmatic liver combined with intralobar pulmonary sequestration. Ann Thorac Surg 2008;85:1809-10.  Back to cited text no. 9
Shapiro JL, Metlay LA. Heterotopic supradiaphragmatic liver formation in association with congenital cardiac anomalies. Arch Pathol Lab Med 1991;115:238-40.  Back to cited text no. 10

Correspondence Address:
Sajna V. M. Kutty
Department of Pathology, Old Block, 2nd Floor, Aster Malabar Institute of Medical Sciences, P. O. Govindapuram, Kozhikode - 673 016, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_416_16

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