Epigastric Heteropagus: A Case Report with Review of the Literature

Abstract

Incomplete conjoined twinning or heteropagus attached at the autosite's epigastrium is an extremely rare form of conjoined twinnings. We report a case of epigastric parasitic twinning in which the parasite has a well developed lower trunk and pelvis with rudimentary lower limbs, and well developed upper extremities without shoulder girdles and thoracic cage. The clinical features of this rare entity are discussed with a literature review. We emphasize that in spite of monstrous appearance, autosite component of epigastric heteropagus can be treated successfully with minor surgery. This fact should be kept in mind during the intrauterine evaluation of these type of anomalies in order to avoid needless terminations.

Bizarre Presentation of Epigastric Heteropagus: Report of a Case

“…Epigastric heteropagus refers to conjoined twins, in which the parasite is attached to the epigastrium of the autosite. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] After incomplete cleavage of the embryo, ischemic changes of varying degree occur and the remaining parasite usually has a rudimentary upper part of the body. Some reports describe a parasite connected at the chest wall as a result of a giant omphalocele, but they were still usually classified as epigastric heteropagus.…”

“…To our knowledge, only 27 cases have been reported in the English literature. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] In some cases, the parasite was attached to the chest wall of the autosite, but these cases were still categorized as epigastric heteropagus. 2,7,12,13 In epigastric heteropagus, the parasite usually has a rudimentary upper part of the body, a pelvic bone, visceral organs such as a bladder, kidney, genitourinary organs, gastrointestinal tract, and lower limbs, but the pattern of abnormalities differs from that in caudal duplication (dipygus).…”

Thoracopagus parasites in two sets of twins: evidence for the ‘fusion theory’

“…While conjoint twins have the highest incidence of being attached at the thorax (thoracopagus) [1], the literature has very few cases of thoracopagus parasitic twins [1,2]. In most of the reported cases, the parasitic twins were found to be attached at the epigastrium and frequently also had omphalocele [1][2][3][4][5][6][7][8][9][10][11][12]. We, herein, report two sets of thoracopagus parasitic twins.…”

“…The extent of the development in the parasitic twin has also varied in different case reports. Most parasitic twins had better developed lower limbs [2,7,8,11,12], while very few have well-developed upper limbs [6,10]. This may be either because that the lower half of the body in the parasite is more resistant to resorption or the resorption process takes place in a cranio-caudal direction.…”

“…Therefore, both sets of twins described by us are rare variants. Most cases described in the literature have quoted the attachment of the parasitic twin at the epigastric region [1][2][3][4][5][6][7][8][9][10][11][12]. The extent of the development in the parasitic twin has also varied in different case reports.…”