Journal of Clinical Gynecology and Obstetrics, ISSN 1927-1271 print, 1927-128X online, Open Access |
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Volume 9, Number 4, December 2020, pages 129-133
Rare Presentation of Fetus in Fetu - Laparoscopic Approach: A Case Report
Vandana Menon a, c , Bedaya Amro a , Tasnim E.V. Keloth b , Arnaud Wattiez a , Maan Hachmi b
a Latifa Women and Children Hospital, Dubai, UAE b Dubai Hospital, Dubai, UAE c Corresponding Author: Vandana Menon, Latifa Women and Children Hospital, Dubai, UAE
Manuscript submitted July 30, 2020, accepted October 12, 2020, published online December 15, 2020 Short title: Rare Presentation of FIF doi: https://doi.org/10.14740/jcgo681
Fetus in fetu (FIF) is an extremely rare condition in which a malformed fetus is found most commonly in the abdomen of a living twin. We report a case of FIF in a young adult woman, which presented as a twisted ovarian cyst and was successfully managed by laparoscopy. This is the first reported case of FIF with an acute presentation, the first case in an adult ovary and the first to be managed successfully by laparoscopy. The excised ovarian mass was diagnosed as FIF with benign teratoma based on histopathological examination and radiography.
Keywords: Case report; Fetus in fetu; Dermoid; Laparoscopy; Abdominal mass
Fetus in fetu (FIF) is a rare condition which usually presents as a vertebrate fetiform mass in a newborn or a child and occasionally in an adult man. It occurs in about 1 in 500,000 live births [ 1 ] and less than 200 cases have been reported in the medical literature. This is the third case in an adult female [ 2 ] and the first case managed by laparoscopy.
We present the case of a 26-year-old nulliparous woman who attended our emergency department with acute, severe lower abdominal pain associated with vomiting. She did not have any relevant medical, gynecological or surgical history. She had an ultrasound in another facility which revealed a twisted multiloculated cyst of 11 × 7 cm size, most probably dermoid cyst.
Clinical examination revealed a tender abdomen with guarding. A firm mass of 16 cm size was palpable arising from the pelvis. A bedside ultrasound revealed a cyst of 16 cm with solid and cystic areas with significantly compromised vascularity. The tumour markers (beta-human chorionic gonadotropin (hCG), alpha-fetoprotein (AFP) and carcinoembryonic antigen (CEA)) were found to be normal. CA125 was slightly elevated at 78 U/mL (reference range: 0 - 35). Imaging modalities like magnetic resonance imaging (MRI) and computed tomography (CT) could not be performed due to the severe pain at presentation and non-availability of MRI and CT in our facility. With a provisional diagnosis of twisted ovarian cyst, probably dermoid, we decided to perform emergency diagnostic laparoscopy and to proceed accordingly. Consent was taken for ovarian cystectomy and/or adnexectomy. Intraoperatively, the left ovary was found to be twisted six times around its pedicle with an ovarian cyst of 18 × 16 cm ( Fig. 1 ) containing both solid and cystic areas. The whole ovary was grayish with a hemorrhagic surface, indicating ischemic changes. However, it had a smooth surface and had no suspicious features. Peritoneal fluid was aspirated for cytology. A systematic examination of the rest of the pelvis and upper abdomen was implemented and was found to be normal. The ovary was gently untwisted, after which it gradually started regaining its color. Taking into consideration the young age and nulliparity of the patient, ultrasound features of dermoid and no suspicious features of malignancy on inspection, we decided to go for laparoscopic cystectomy. After cyst aspiration, cystectomy was carried out in a large endo bag and the procedure was completed with minimal spillage of cheesy material. At extraction, a large bony part was felt significantly with sharp edges. So one lateral port was extended to 4 - 5 cm ( Fig. 2 ) and cyst was completely extracted, which revealed a partly formed fetus. Reconstruction of the ovary was done with trimming of ovarian tissue and suturing using 2-0 poliglecaprone 25 (Ethicon Monocryl). Intraoperative blood loss was minimal (around 50 mL). The patient was discharged the next day with uneventful and fast recovery as expected from laparoscopic surgeries.
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Gross specimen analysis, radiography and microscopic analysis in histopathology department were undertaken and a final diagnosis of FIF with teratoma was rendered. The presence of fetal skull, rudimentary limbs with digits, malformed trunk with vertebral column, gluteal region with central cleft and a polypoid structure resembling external genitalia support the FIF entity ( Figs. 3 - 7 ).
The present case is the ninth reported case of adult FIF (89% occurring before 18 months) and the third case in an adult female ( Table 1 ) [ 2 - 9 ]. To our knowledge, this is the only known case of FIF reported in an adult ovary. Furthermore, this is the only reported case which has been managed laparoscopically and an acute presentation has so far not been described. Although benign, malignant recurrence has been recorded with FIF [ 10 ].
| Reported Cases of Adult FIF and Their Characteristics Between 1992 and 2019 [ - ] |
The term FIF was first described by Friedrich Meckel. There is controversy as to whether this is a distinct entity or a highly organised teratoma. The most accepted theory is the twin theory which states that FIF is a monochorionic, diamniotic twin which becomes incorporated in the body of host twin after anastomosis of vitelline circulation [ 11 ]. According to Willis in 1953, identification of vertebral column ensures the diagnosis of FIF and differentiates this from teratoma. Identification of vertebral column indicates that fetal evolution must have advanced at least to a primitive streak stage to develop notochord [ 12 ]. Some investigators like Prescher et al hypothesized that FIF represents a well-differentiated and highly organized teratoma [ 13 ]. Our case supports the twin theory of origin of FIF due to the high level of organization of tissues and not a cluster of primitive tissues.
The common presentation of FIF is a mass in the abdomen, almost 80% in the retroperitoneum [ 2 ], although it has been reported at various sites right from the cranial cavity to the scrotum. Different organs can be seen in FIF, including vertebral column (91%), limbs (82.5%), central nervous system (55.8%), gastrointestinal tract (45%), vessels (40%) and genitourinary tract (26.5%) [ 14 ]. The mass may compress the surrounding organs leading to symptoms such as abdominal distension, feeding difficulty, vomiting, jaundice or pressure effects on the renal or respiratory system. A presumptive diagnosis can be made by ultrasound, plain radiography, CT scan or MRI which will reveal the presence of axial skeleton and vertebral column. Nevertheless, this should not lead to diagnostic exclusion as an underdeveloped spinal column may not be visualized by radiography [ 15 ]. Molecular analysis using a genetic marker for uniparental isodisomy of chromosomes 14 and 15 has been described [ 16 ]. Treatment of FIF is surgical. Although majority of the cases are benign, complete excision of FIF together with the capsule is crucial as malignant recurrence has been reported rarely, especially if part of the tissue is not completely excised [ 10 ]. A few authors have advocated follow-up for tumor recurrence using tumor markers like AFP, with some of them suggesting 2 years as the ideal time frame, especially when FIF and teratoma are considered as part of the same spectrum [ 13 ].
Conclusions
Although FIF is a rare entity with a chronic course, the possibility should be kept in mind while dealing with an emergency like a twisted ovarian cyst, in spite of the unusual location and sex predilection. The operation to remove FIF can be challenging if the mass is highly vascular with multiple feeding vessels and has been traditionally performed by laparotomy. The aforementioned case proves that laparoscopy is a feasible option in the management of FIF and other partly solid adnexal masses in expert hands. Postoperative follow-up with screening using tumor markers, especially AFP is mandatory if initial levels are raised to rule out malignant recurrence.
Acknowledgments
None to declare.
Financial Disclosure
Conflict of Interest
Informed Consent
Written informed consent was obtained from the patient for publication of this case report.
Author Contributions
Vandana Menon performed surgery and postoperative follow-up, and manuscript preparation. Bedaya Amro performed surgery and manuscript preparation. Tasnim E. V. Keloth contributed to histopathology examination and manuscript preparation. Arnaud Wattiez contributed to manuscript supervision. Maan Haachmi contributed to radiology examination and diagnosis.
Data Availability
The authors declare that data supporting the findings of this study are available within the article.
References |
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Frank breech. When a baby's feet or buttocks are in place to come out first during birth, it's called a breech presentation. This happens in about 3% to 4% of babies close to the time of birth. The baby shown below is in a frank breech presentation. That's when the knees aren't bent, and the feet are close to the baby's head.
If the fetus is in a different position, lie, or presentation, labor may be more difficult, and a normal vaginal delivery may not be possible. Variations in fetal presentation, position, or lie may occur when. The fetus is too large for the mother's pelvis (fetopelvic disproportion). The uterus is abnormally shaped or contains growths such as ...
Cephalic occiput anterior. Your baby is head down and facing your back. Almost 95 percent of babies in the head-first position face this way. This position is considered to be the best for ...
Occiput or cephalic anterior: This is the best fetal position for childbirth. It means the fetus is head down, facing the birth parent's spine (facing backward). Its chin is tucked towards its chest. The fetus will also be slightly off-center, with the back of its head facing the right or left. This is called left occiput anterior or right ...
Fetal lie: Relation of the fetus to the long axis of the uterus; longitudinal, oblique, or transverse. Normal fetal lie is longitudinal, normal presentation is vertex, and occiput anterior is the most common position. Abnormal fetal lie, presentation, or position may occur with. Fetopelvic disproportion (fetus too large for the pelvic inlet)
Fetal presentation, or how your baby is situated in your womb at birth, is determined by the body part that's positioned to come out first, and it can affect the way you deliver. At the time of delivery, 97 percent of babies are head-down (cephalic presentation). But there are several other possibilities, including feet or bottom first (breech ...
Toward the end of pregnancy, the fetus moves into position for delivery. Normally, the presentation is vertex (head first), and the position is occiput anterior (facing toward the pregnant patient's spine) with the face and body angled to one side and the neck flexed. Abnormal presentations include face, brow, breech, and shoulder. Occiput ...
Breech Presentation. When the fetus presents as a breech, the three general configurations are frank, complete, and footling presentations and are described in Chapter 28 ... As a rule, the fetus forms an ovoid mass that corresponds roughly to the shape of the uterine cavity. The fetus becomes folded or bent upon itself in such a manner that ...
This presentation can lead to more back pain (sometimes referred to as "back labor") and slow progression of labor. In the right occiput posterior position (ROP), the baby is facing forward and slightly to the right (looking toward the mother's left thigh). This presentation may slow labor and cause more pain. Tips to Reduce Discomfort
Position and Presentation of the Fetus. Toward the end of pregnancy, the fetus moves into position for delivery. Normally, the position of a fetus is facing rearward (toward the woman's back) with the face and body angled to one side and the neck flexed, and presentation is head first.
The fetus undergoes a series of changes in position, attitude, and presentation during labor. This process is essential for the accomplishment of a vaginal delivery. The presence of a fetal malpresentation or an abnormality of the maternal pelvis can significantly impede the likelihood of a vaginal delivery. The contractile aspect of the uterus ...
Lie. Facing the patient's head, place hands on either side of the top of the uterus and gently apply pressure. Move the hands and palpate down the abdomen. One side will feel fuller and firmer - this is the back. Fetal limbs may be palpable on the opposing side. Fig 2 - Assessing fetal lie and presentation.
If your baby is headfirst, the 3 main types of presentation are: anterior - when the back of your baby's head is at the front of your belly. lateral - when the back of your baby's head is facing your side. posterior - when the back of your baby's head is towards your back. Top row: 'right anterior — left anterior'.
Fetal lie refers to the relationship between the long axis of the fetus with respect to the long axis of the mother. The possibilities include a longitudinal lie, a transverse lie, and, on occasion, an oblique lie. Fetal presentation is a reference to the part of the fetus that is overlying the maternal pelvic inlet.
The fetus faces the maternal spinal cord and the smallest part of the head (the posterior aspect called the occiput) exits the birth canal first. In fewer than 5 percent of births, the infant is oriented in the breech presentation, or buttocks down.
presentation. Double-head fetoscope and traditional Pinard Horn Follow Step 5 - a complementary diagnostic procedure after performing the Leopold Maneuver Fetus in breech presentation In breech presentation, the fetal heart rate will be found around or above the mothers' navel. A fetal heart rate found at this spot can confirm the suspicion of
Breech presentation refers to when the fetus is in a longitudinal lie with its buttocks as the lowest part. The document discusses the different types of breech presentations as well as their incidence, classifications, positions, etiology, diagnosis, and management both during pregnancy and delivery. ... the fetus forms an ovoid mass that ...
Leopold maneuvers are a systematic four-step physical examination performed to evaluate the fetal lie, presentation, and position of the fetus in the uterus. ... If you feel broad, firm, irregular soft mass indicates fetal buttocks is in the fundus. It means presentation is cephalic and the lie is longitudinal. This is the normal findings which ...
There can be many variations in the fetal presentation which is determined by which part of the fetus is projecting towards the internal cervical os. This includes: cephalic presentation: fetal head presenting towards the internal cervical os, considered normal and occurs in the vast majority of births (~97%); this can have many variations ...
Management is conservative and depends on mass size, symptoms, and presence of complications. Masses greater than 4 cm to 5 cm are associated with a risk of torsion approaching 50% to 78% and typically warrant postnatal surgical resection. 3-5 Neonatal cyst aspiration is generally not recommended because of recurrence risk and the need to ...
By: Amos Grünebaum. Updated on March 25, 2019. A malpresentation or malposition of the fetus is when the fetus is in any abnormal position, other than vertex (head down) with the occiput anterior or posterior. The following are considered malpresentations or malpositions: Unstable lie. Breech.
The common presentation of FIF is a mass in the abdomen, almost 80% in the retroperitoneum [2], ... Kanhaiya C, Sadhna M, Sabiha M. Fetus in fetu: a rare presentation in an adult female. Oman Med J. 2010;25. doi; Sharma N, Verma P, Pathak P. 27 cm fetus in fetu presenting in a 36-year-old man: report of a rare case and brief review of ...