Ann Thorac Surg 2008;85:1809-1810. doi:10.1016/j.athoracsur.2007.11.040
© 2008 The Society of Thoracic Surgeons
Case Reports
Heterotopic Supradiaphragmatic Liver Combined With Intralobar Pulmonary Sequestration
Seon Uoo Choi, MD,
Hong Kwan Kim, MD,
Jhingook Kim, MD*
Department of Thoracic Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea
Accepted for publication November 14, 2007.
* Address correspondence to Dr Jhingook Kim, Department of Thoracic Surgery, Samsung Medical Center, 50 Ilwon-dong, Gangnam-gu, Seoul, 135-710, Korea (Email: jkimsmc{at}skku.edu).
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Abstract
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Heterotopic supradiaphragmatic liver is exceedingly rare. A total of 16 cases of supradiaphragmatic liver have been reported in the literature. Therefore, it is difficult to make a correct diagnosis preoperatively. Herein we report a case of supradiaphragmatic heterotopic liver coincidentally combined with intralobar pulmonary sequestration.
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Introduction
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Heterotopic supradiaphragmatic liver is exceedingly rare. Abnormally positioned liver tissue has been described, but it is more commonly observed in the abdominal cavity than in the thoracic cavity. A total of 16 cases of supradiaphragmatic liver have been reported in the literature since Hansbrough and Lipin described the first case in 1957 [1–5]. Herein we describe a case of supradiaphragmatic heterotopic liver coincidentally combined with intralobar pulmonary sequestration.
A 3-year-old boy was referred to our center from an outside clinic with a history of recurrent pneumonia refractory to antibiotics. Two years ago, he was diagnosed with pneumonia for the first time and then fully recovered after antibiotic therapy. At the time, he was incidentally diagnosed with dextrocardia, secundum-type atrial septal defect, and pectus excavatum. Since then, however, he frequently complained of cough, and then 3 months ago, he presented to the local clinic with a 24-hour history of cough and fever. Despite adequate treatment, he failed to respond to antibiotics and his symptoms worsened. A computed tomography of the chest revealed a masslike consolidation in the right lower lobe, which suggested an extralobar pulmonary sequestration (Fig 1). Additionally, multiple conglomerated cystic lesions were also detected in the subpleural portion of the right apical lung, which raised the possibility of combined congenital cystic adenomatoid malformation in the right upper lobe (Fig 1). We decided to surgically resect both the masslike consolidation in the right lower lobe and the cystic lesions in the right upper lobe, which were considered pulmonary sequestration and congenital cystic adenomatoid malformation, respectively. In addition to intractable pneumonia, right pulmonary hypoplasia was also suspected secondary to the sequestration and multiple cysts. Therefore, we performed a right pneumonectomy, because operative findings revealed that it was unavoidable owing to hypoplasia of the right lung.
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Fig 1. The preoperative computed tomography revealed a masslike consolidation (Box A) in the right lower lobe, which suggested an extralobar pulmonary sequestration. In addition, multiple conglomerated cystic lesions (Box B) were also detected in the subpleural portion of the right apical lung, which raised the possibility of combined congenital cystic adenomatoid malformation in the right upper lobe.
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Operative findings showed that there was a well-defined, green-brown, 4.7- x 3.5- x 3-cm solid mass in the right lower lobe. There were no abnormal connections in the right pulmonary hilar structures except that the pulmonary artery was relatively hypoplastic. Although a systemic artery, coming from the right hemidiaphragm and supplying the mass, was identified and then transected, there was no connection to the liver with an intact diaphragm. Contrary to the preoperative impression, the histopathologic findings revealed that the mass in the right lower lobe was hepatic parenchyma, microscopically consisting of cords of large polygonal hepatic cells and bile ducts (Fig 2). Multiple cystic lesions in the right upper lobe, which was considered congenital cystic adenomatoid malformation, turned out to be intralobar pulmonary sequestration (Fig 2). The postoperative course was uneventful, and he did well and remained free of symptoms.
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Fig 2. Histopathologic findings revealed that the mass in the right lower lobe (Box A) was hepatic parenchyma, microscopically consisting of cords of large polygonal hepatic cells and bile ducts. Multiple cystic lesions in the right upper lobe (Box B), which was considered congenital cystic adenomatoid malformation, turned out to be intralobar pulmonary sequestration. (Inset: Hematoxylin and Eosin stain, x200.)
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Comment
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The occurrence of heterotopic liver tissue is an uncommon finding. It is most often found in the vicinity of the liver such as in the gallbladder, spleen, pancreas, umbilicus, adrenal gland, or omentum, and it usually has some connective tissue or mesenteric attachment to the liver [1]. However, supradiaphragmatic, that is, intrathoracic, liver tissue is very rare. A total of 16 cases of supradiaphragmatic liver have been reported in the literature, and of those, several cases were diagnosed at autopsy [1–5]. Even in the thoracic cavity, accessory liver tissue is more likely to be attached to the liver through the diaphragmatic defect. Therefore, supradiaphragmatic liver tissue without connection to the liver is exceedingly rare. Furthermore, intralobar pulmonary sequestration was also combined with heterotopic liver in our case. In this case, both heterotopic supradiaphragmatic liver and intralobar pulmonary sequestration were simultaneously detected.
The pathogenesis of supradiaphragmatic liver, in the presence of an intact diaphragm, is unknown. Although heterotopic liver tissue may be acquired secondary to trauma or diaphragmatic hernia repair, previous reports have speculated that this condition mostly represents a developmental defect of the septum transversum [1]. The most probable explanation is the development of an accessory liver lobule, with atrophy or regression of the original connection to the abdominal liver [1]. Another possible explanation is the development of an entirely separate liver bud independent of the main hepatic diverticulum without any prior connection [1].
Because heterotopic supradiaphragmatic liver is extremely rare, it is difficult to make a correct diagnosis preoperatively. In our case, we also misdiagnosed it as the pulmonary sequestration. We did not suspect that the mass could be a heterotopic supradiaphragmatic liver at the time of operation, as we had concluded that it was the pulmonary sequestration. Definitive treatment of this anomaly is surgical resection, leading to successful outcomes. We presented a very interesting case of supradiaphragmatic heterotopic liver coincidentally combined with intralobar pulmonary sequestration.
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References
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- Mendoza A, Voland J, Wolf P, Benirschke K. Supradiaphragmatic liver in the lung Arch Pathol Lab Med 1986;110:1085-1086.[Medline]
- Hansbrough ET, Lipin RJ. Intrathoracic accessory lobes of the liver Ann Surg 1957;145:564-567.[Medline]
- Hudson TR, Brown HN. Ectopic (supradiaphragmatic) liver J Thorac Cardiovasc Surg 1962;43:552-555.[Medline]
- Sehdeva JS, Logan WB. Heterotopic (supradiaphragmatic) liver Ann Thorac Surg 1971;11:468-471.[Abstract/Free Full Text]
- Luoma R, Raboei E. Supradiaphragmatic accessory liver: a rare cause of respiratory distress in a neonate J Pediatr Surg 2003;38:1413-1414.[Medline]
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Abstract
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