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Fetus in Fetu By George V., Meera Khanna, and Tripta Dutta New Delhi, India 9 This is the report of a case of fetus in fetu. It corresponds to the generally recognized criteria for this diagnosis. INDEX WORDS: Fetus in fetu. M ECKEL (CA 1800) coined the term fetus in fetu to describe a condition wherein a parasitic twin was found included within the abdomen of its partner. A fetus in fetu is a diamniotic, monochorionic, monozygotic twin of its bearer, included with the bearer due to anas- tomosis of the vitelline circulation. 1 Most often the fetus in fetu is intraabdominal and situated retroperitoneally, although other sites have also been reported. It is surrounded by a membrane that is analogous to the amniotic sac and characterized by a single feeding vessel and the absence of a true placenta. The included twin may be grossly deformed due to compression by the host's abdominal organs. In 1935, Willis, 2 adopted the presence of a vertebral axis (having passed through a primitive streak stage) and an appropriate arrangement of other organs or limbs with respect to the axis as the criterion to distinguish fetus in fetu from teratoma. A teratoma is a true neoplasm arising from embryonic totipotential cells with benign or malignant properties. Teratomas lack a vertebral column (not having passed through a primitive streak stage) and organogenesis but do manifest some degree of progressive uncoordinated growth. CASE REPORT Diagnosis A 3-month-old male child was admitted to the pediatric surgery unit of Safdarjang Hospital, New Delhi, with pro- gressive abdominal distention since birth. The mother had noticed a lump on the left side of the abdomen. A family history of twins was present. The infant was a normal looking baby. Examination of the abdomen revealed a firm, well- circumscribed, smooth-surfaced mass, 15 x 20 cm in size, in the left side of the abdomen mainly in the lumbar region and extending across the midline in the region of the umbilicus. Plain x-ray of the abdomen showed a soft tissue mass in the left side of the abdomen. Intravenous pyelography revealed a nonfunctioning left kidney. A clinical diagnosis of Wilms' tumor on the left side was made. Operative Findings In the left lateral position, through a subcostal incision, the abdomen was opened in layers. A retroperitoneal irregular mass was seen. Part of the mass was cystic and drained 300 to 400 cc of serous fluid. The rest of the mass was solid; part of it was within the cystic cavity. We exteriorized the sac wall completely and removed the mass. The entire specimen was covered with fine-hair-bearing skin (Fig. 1). A cephalic prominence was present. There was no separation of the abdomen and thorax. A gluteal cleft was present caudally but no external genitalia or anal dimple was seen. Two hands and one foot with well-defined fingers and toes and well-formed nails were present (Fig. 2). Radiologic examination of the specimen revealed small bones in the foot. A longitudinal, midline section of the specimen showed vertebral bodies. Another section, through the lower extrem- ity showed long bones. Results Postoperative recovery of the patient was uneventful. At follow-up after 3 months, he was perfectly well. Postoperative intravenous pyelography showed normal function of both kidneys. DISCUSSION In 1961 in his comparative study of the fetus in fetu and retroperitoneal teratoma, Lewis From the Pediatric Surgical Department, Safdarjang Hospital, New Delhi. Address reprint requests to Dr. T. Dutta, D-II/30, EAST KIDWAI NAGAR, New Delhi, 110023, India. 9 1983 by Grune & Stratton, Inc. 0022-3468/83/1803~)016501.00/0 Fig. 1. Specimen covered with fine-hair-bearing skin. 288 Journal of Pediatric Surgery, Vol. 18, No. 3 (June), 1983

Fetus in fetu

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Fetus in Fetu

By George V., Meera Khanna, and Tripta Dutta N e w Delhi, India

�9 This is the report of a case of fetus in fetu. It corresponds to the general ly recognized cr i ter ia for this diagnosis.

INDEX W O R D S : Fetus in fetu.

M ECKEL (CA 1800) coined the term fetus in fetu to describe a condition wherein a

parasitic twin was found included within the abdomen of its partner. A fetus in fetu is a diamniotic, monochorionic, monozygotic twin of its bearer, included with the bearer due to anas- tomosis of the vitelline circulation. 1

Most often the fetus in fetu is intraabdominal and situated retroperitoneally, although other sites have also been reported. It is surrounded by a membrane that is analogous to the amniotic sac and characterized by a single feeding vessel and the absence of a true placenta. The included twin may be grossly deformed due to compression by the host's abdominal organs.

In 1935, Willis, 2 adopted the presence of a vertebral axis (having passed through a primitive streak stage) and an appropriate arrangement of other organs or limbs with respect to the axis as the criterion to distinguish fetus in fetu from teratoma. A teratoma is a true neoplasm arising from embryonic totipotential cells with benign or malignant properties. Teratomas lack a vertebral column (not having passed through a primitive streak stage) and organogenesis but do manifest some degree of progressive uncoordinated growth.

CASE REPORT

Diagnosis A 3-month-old male child was admitted to the pediatric

surgery unit of Safdarjang Hospital, New Delhi, with pro- gressive abdominal distention since birth. The mother had noticed a lump on the left side of the abdomen. A family history of twins was present. The infant was a normal looking baby. Examination of the abdomen revealed a firm, well-

circumscribed, smooth-surfaced mass, 15 x 20 cm in size, in the left side of the abdomen mainly in the lumbar region and extending across the midline in the region of the umbilicus. Plain x-ray of the abdomen showed a soft tissue mass in the left side of the abdomen. Intravenous pyelography revealed a nonfunctioning left kidney. A clinical diagnosis of Wilms' tumor on the left side was made.

Operative Findings In the left lateral position, through a subcostal incision, the

abdomen was opened in layers. A retroperitoneal irregular mass was seen. Part of the mass was cystic and drained 300 to 400 cc of serous fluid. The rest of the mass was solid; part of it was within the cystic cavity. We exteriorized the sac wall completely and removed the mass.

The entire specimen was covered with fine-hair-bearing skin (Fig. 1). A cephalic prominence was present. There was no separation of the abdomen and thorax. A gluteal cleft was present caudally but no external genitalia or anal dimple was seen. Two hands and one foot with well-defined fingers and toes and well-formed nails were present (Fig. 2). Radiologic examination of the specimen revealed small bones in the foot. A longitudinal, midline section of the specimen showed vertebral bodies. Another section, through the lower extrem- ity showed long bones.

Results Postoperative recovery of the patient was uneventful. At

follow-up after 3 months, he was perfectly well. Postoperative intravenous pyelography showed normal function of both kidneys.

DISCUSSION

I n 1961 in h i s c o m p a r a t i v e s t u d y o f t h e f e t u s in

f e t u a n d r e t r o p e r i t o n e a l t e r a t o m a , L e w i s

From the Pediatric Surgical Department, Safdarjang Hospital, New Delhi.

Address reprint requests to Dr. T. Dutta, D-II/30, E A S T KIDWAI NAGAR, New Delhi, 110023, India.

�9 1983 by Grune & Stratton, Inc. 0022-3468/83/1803~)016501.00/0 Fig. 1. Specimen covered with fine-hair-bearing skin.

288 Journal of Pediatric Surgery, Vol. 18, No. 3 (June), 1983

Page 2: Fetus in fetu

FETUS IN FETU 289

Fig. 2. Two hands and one foot with fingers and toes.

repor ted tha t the fetus in fetu is usual ly sus- pended by a pedicle within a capsule conta ining a l i t t le f lu id) This pedicle is una t t ached to the host and, usually, ends in a th ickened vascular a rea of the sac wall. H e fur ther s ta ted tha t more than one par t of a fetus should be recognizable and the suspending pedicle ident i f iable in order to d iag- nose a fetus in fetu. Also, a ver tebra l column at some s tage of development should be found, together with other bones. Proof of the genuine fetal na ture of a p resumed in t r a -abdomina l fetus in fetu requires the unequivocal x - ray or dissec- t ional demons t ra t ion of par t or the whole of a ver tebra l axial skeleton, 4 a demons t ra t ion tha t will be re inforced if o ther appropr i a t e ly s i tuated bones or organs are shown to be present. ~

The findings in our case, as descr ibed in the case repor t above, suppor t diagnosis of a fetus in fetu as defined by this cr i ter ia .

REFERENCES

1. Lord JM: Foetus in fetu. J Pathol Bacteriol 72:627, 4. Broghammer B J: Foetus in fetu. Radiology 80:844, 1956 1963

2. Willis RA: The structure of a teratoma. J Pathol Bacteriol 40:1, 1935

3. Lewis RH: Foetus in fetu and retroperitoneal teratoma. 5. Numanogolu: Foetus in fetu. J Pediatr Surg 5:472, Arch Dis Child 36:220, 1961 1970