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Case Report |
1 Department of Radiology, Asan Medical Center, University of Ulsan College of
Medicine, 388-1 Poongnap-dong, Songpa-gu, Seoul 138-040, Korea.
2 Department of Neonatology, Asan Medical Center, University of Ulsan College of
Medicine, Seoul 138-040, Korea.
3 Department of Pediatric Surgery, Asan Medical Center, University of Ulsan
College of Medicine, Seoul 138-040, Korea.
4 Department of Radiology, Hôpital Jeanne d'Arc, B.P. 303, 54201 Toul,
France.
Received February 21, 2002;
accepted after revision May 23, 2002.
Address correspondence to C. H. Yoon.
Introduction
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To the best of our knowledge, three-dimensional (3D) imaging findings have not been reported for fetus in fetu. In our case report of fetus in fetu, multidetector 3D CT was helpful in delineating the complicated anatomy of this entity and for defining the blood supply before surgery.
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At physical examination, the mass was firm, smooth, and not tender.
Laboratory findings, including serum
-fetoprotein and ß-human
chorionic gonadotropin levels, were within normal limits. On radiography of
the abdomen, we identified a craniocaudal arrangement of what appeared to be
two spines and two long bones in the right upper quadrant of the abdomen
(Fig. 1A) and thus suspected
fetus in fetu.
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CT was performed before surgery to more clearly define the relationship between the mass and the adjacent organs. We used a multidetector CT scanner (QX/i LightSpeed; General Electric Medical Systems, Milwaukee, WI). Contrast-enhanced CT was performed after IV injection of 4 mL/kg of a mixture (1:1) of saline and iopromide (Ultravist 370; Schering, Berlin, Germany) through a 24-gauge angiographic catheter inserted in an antecubital vein via a power injector at a flow rate of 0.8 mL/sec. The technical parameters included 1.25 mm of collimation, table speed of 3.75 mm, gantry rotation speed of 0.8 sec, 120 kVp, and 40 mA. Postprocessing was performed on a commercially available workstation (Advantage Windows 3.1; General Electric Medical Systems). The techniques we used for 3D reconstruction were shaded surface display, maximum intensity projection, and volume rendering.
Three-dimensional CT scans showed a large well-defined mass enveloped by a sac in the retroperitoneum that displaced the right kidney posteriorly. The mass contained both cystic and solid portions. The bony components resembled vertebral bodies, ribs, two femurs, two humeri, and cartilaginous structures. The fetuslike structure was anencephalic and had two short upper extremities and fused lower extremities containing two long bones with toelike structures (Figs. 1B and 1C). An anomalous blood supply to the mass arose directly from the right renal artery (Fig. 1D). Another ovoid lobulating mass supplied from the right renal artery was present on the inner surface of the cyst (Fig. 1E).
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This boy underwent surgical exploration 3 days after birth, and a large
retroperitoneal mass that displaced the right kidney posteriorly was revealed.
As suggested by the 3D CT findings, surgeons found an anomalous blood supply
from the right renal artery to the fetuslike structure. The mass was resected
en bloc. Gross examination revealed the mass to be an anencephalic fetus in
fetu that weighed approximately 185 g and was 7 cm in length. The separate
ovoid mass (
3 cm) contained grossly symmetric pieces of cartilage.
Histopathologic findings showed such extensive autolysis that it was
impossible to determine whether the ovoid mass was another fetus in fetu or a
teratoma. Neither a definite vertebral axis nor well-organized structures were
identified in this second mass, but the cartilaginous tissues were
symmetrically located within it, suggesting early formation of both upper and
lower extremities. A small gutlike structure with mucosal and smooth muscle
wall was also noted. These findings suggested that the small solid mass could
in fact be another fetus in fetu.
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Some authors believe that teratoma and fetus in fetu are part of a spectrum of the same disorder, but others have suggested, for several reasons, that fetus in fetu is a pathologic entity that is distinct from teratoma. An important feature that has been used to distinguish fetus in fetu from teratoma is the presence of a vertebral column. Identification of the vertebral column indicates that fetal development of the included twin must have advanced at least beyond the primitive streak stage (12-15 days' gestation) for a notochord, which is the precursor of the vertebral column, to have developed [1,2,3,4,5,6,7]. Fetus in fetu generally occurs singly; multiple masses have been found in only a few instances. In our patient, a mass enveloped by a sac contained a second mass that was suggestive of a second fetus in fetu.
Imaging plays an important role in the correct diagnosis of fetus in fetu. This diagnosis can be made on abdominal radiography by identifying a vertebral column, specific bony structures, or both. Occasional cases have also been reported in which the spinal column could not be identified on imaging [1], likely because the vertebral bodies could not be seen in an underdeveloped and markedly dysplastic spinal column. Vascular connections to the fetus in fetu have been reported. The predominant blood supply appears to be derived from the plexus, where the fetus in fetu and the sac are attached to the abdominal wall. Other sources of blood supply include the superior mesenteric artery, inferior mesenteric artery, and middle sacral artery [1]. In our patient, the fetus in fetu derived its blood supply from the right renal artery.
The preoperative diagnosis of fetus in fetu was made in only 16.7% of the cases reported before 1980, which may be explained by the fact that CT, although performed to some degree before 1980, was not widely available. Currently, CT is extremely helpful, both for making the diagnosis and in preoperative planning [1]. Multidetector CT provides high quality images with high spatial resolution and fewer motion artifacts than single-detector CT. The 3D CT scans obtained in our patient are preferable to axial CT images for depicting a complicated lesion with high fidelity. We think that 3D CT provides better images than those of previously developed techniques. Therefore, 3D CT was helpful in preoperative planning because the information it provided enabled evaluation of the relationship of the fetuslike structure to the other abdominal structures and to its blood supply. To the best of our knowledge, this case is the first in which the blood supply of a fetus in fetu was identified before surgery.
In conclusion, 3D CT accurately revealed the detailed anatomy of a fetuslike structure and depicted its blood supply preoperatively; therefore, this imaging technique is useful for planning surgical treatment of patients with fetus in fetu.
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This article has been cited by other articles:
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H. Ghazle and K. Dolbow Fetus in Fetu Journal of Diagnostic Medical Sonography, September 1, 2009; 25(5): 272 - 276. [Abstract] [PDF] |
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