What are the other Names for this Condition? (Also known as/Synonyms)

• Fetus Papyraceous
• Foetus Papyraceus
• Paper Doll Fetus

What is Fetus Papyraceus? (Definition/Background Information)

• Fetus Papyraceus is an uncommon condition that is associated with multiple gestational pregnancy, mostly twin pregnancy. In this condition, there is an intrauterine demise of one of the fetuses, which usually occurs in late first or early second trimester
• The fetal demise tissue is mechanically compressed to resemble a parchment paper, hence the name Fetus Papyraceus. In many cases, the amniotic fluid and placental tissue associated with the dead fetus usually gets absorbed during pregnancy, often leaving behind no trace of the incident (a condition called fetus vanescens or vanishing twin syndrome)
• In many cases, Fetus Papyraceus typically remains undiagnosed, until the fetal remnants are examined by a healthcare provider after childbirth. A placental exam may reveal the mummified fetus between the membranes of its well-developed twin and/or the uterine walls, if it is not absorbed completely as the pregnancy progresses
• It is reported that a fetal demise in (early) twin/multiple pregnancy may take place due to conditions such as membranous cord insertion, insufficiency of placenta, or twin-twin transfusion syndrome
• The signs and symptoms of Fetus Papyraceus may rarely include abnormal bleeding from the vagina; it may also compromise the growth and development of the surviving fetus. Nevertheless, in a majority of cases, no significant symptoms are noted
• The complications due to Fetus Papyraceus are usually observed in monochorionic pregnancies, where fetuses share the same placenta. This can result in intrauterine infection, sepsis, and consumptive coagulopathy. It can also lead to a medical emergency, necessitating a C-section surgery to deliver the surviving child
• Usually, the prognosis of Fetus Papyraceus is good for both the mother and child. A close monitoring and regular examination throughout pregnancy of both the mother and surviving fetus, until childbirth is extremely important, following discovery of Fetus Papyraceus. But, it is important to note that a recognition of Fetus Papyraceus during pregnancy is highly unusual

Who gets Fetus Papyraceus? (Age and Sex Distribution)

• Fetus Papyraceus may occur in pregnant women of all ages. This rare condition is only associated with twin or multiple pregnancies
• The incidence is estimated at 1 in 184-200 twin pregnancies, and overall 1 in 12,000 live births. However, the actual figures of intrauterine fetal demise (of one or more of the fetuses) may be much higher, since it remains an unrecognized condition in many
• Women who are pregnant with both male and female fetuses can be affected
• There is no racial, ethnic, or geographical predilection observed

What are the Risk Factors for Fetus Papyraceus? (Predisposing Factors)

The risk factors for Fetus Papyraceus may include:

• Multiple pregnancy (including twin, triplet, quadruplet pregnancy) is the primary risk factor
• Use of assisted reproductive techniques is known to elevate the risk
• Certain intrauterine conditions that affect the developing baby in the womb

Note: Age of the mother during pregnancy, the number of times one has been pregnant, and the number of pregnancies that resulted in viable gestations, are not believed to be risk factors for Fetus Papyraceus.

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Fetus Papyraceus? (Etiology)

• Fetus Papyraceus is a term used to describe the parchment-like remnants of one of the fetuses that died within the womb, in a pregnant woman with multiple/twin gestations. This may happen during gestational weeks 8 to 14
• The dead fetal remnants are either completely absorbed in the uterus (when no remnants are observed), or partially absorbed, to leave behind a much compressed and desiccated residual tissue, called Fetus Papyraceus
• Since, in many cases, such an event (fetal death and its disappearance) may remain unidentified, for both the expectant mother and the healthcare provider, it is also called the Vanishing Twin Syndrome. It is reported that one or more fetuses may “disappear”

The fetal demise during early pregnancy may take place from a variety of conditions that include:

• Stricture of umbilical cord
• True knot of umbilical cord
• Twin-twin transfusion syndrome
• Uteroplacental vascular insufficiency
• Velamentous insertion of umbilical cord
• Other genetic or chromosomal conditions causing congenital anomalies

It is important to note that Fetus Papyraceus is not caused by what the expectant mother did or did not do, either prior to or during pregnancy.

Fetus compressus: It is a condition that is very similar to Fetus Papyraceus. Reportedly, fetus compressus is fetal death with maceration (breaking down on softening in a fluid medium), whereas Fetus Papyraceus is fetal death with mummification without maceration. Fetus compresses eventually leads to Fetus Papyraceus.

What are the Signs and Symptoms of Fetus Papyraceus?

There are no specific signs and symptoms associated with Fetus Papyraceus. In many cases, it is an incidental finding following childbirth. However, occasionally, the condition may affect the surviving (twin) fetus and cause complications in the mother, during pregnancy. Besides, there may be significant symptoms from an underlying condition, if it was causative of fetal death.

The signs and symptoms may include:

• Abdominal pain, rarely
• Sometimes, abnormal vaginal bleeding may be observed in the first trimester for a few weeks, following fetal death (of one of the fetuses)
• Emotional distress during pregnancy

How is Fetus Papyraceus Diagnosed?

In a majority of cases, Fetus Papyraceus is diagnosed after birth during placental examination. In rare cases, early ultrasonographic exams or intra-vaginal ultrasound scans may reveal multiple gestations that subsequently seem to vanish. Often the 2nd and 3rd trimester ultrasound exams do not reveal abnormalities such as Fetus Papyraceus.

In general, the diagnosis of Fetus Papyraceus may involve:

• Physical examination along with an evaluation of medical history (of the mother)
• Regular examination of the growing fetus and placenta, during pregnancy
• The healthcare provider may check for any signs of fetal distress (non-reassuring fetal heart rate)
• Routine ultrasound examination of the abdomen of the pregnant woman; serial ultrasound exams are recommended, especially for “disappeared” gestations and suspected Fetus Papyraceus
• Color Doppler ultrasonography
• A careful examination of the placenta by a pathologist after birth of the child, to macroscopically (gross visual exam) and microscopically analyze the placental tissue may help establish a diagnosis
• Additional tests to diagnose the underlying condition, if any

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Fetus Papyraceus?

The development of complications is dependent on a set of factors that include:

• Underlying causative condition that resulted in fetal demise (of one of the fetuses)
• Whether it is a monochorionic twin pregnancy or dichorionic twin pregnancy
  • Monochorionic twin pregnancy - when twins share the same placenta (identical twins); this form of twin pregnancy can result in severe and several complications
  • Dichorionic twin pregnancy - when each twin has its own chorion and amniotic sac (fraternal twins); this form of twin pregnancy results in reduced complications

Complications due to Fetus Papyraceus, in the surviving fetus, may include:

• Monochorionic twin pregnancy may lead to an increased risk for:
  • Aplasia cutis congenita (skin disorder)
  • Cerebral palsy and other neurological impairments
  • Gastroschisis
  • Malformations such as small head (microcephaly) and fluid accumulation within the brain (hydrocephalus)
  • Perinatal mortality
• Twin-to-twin transfusion syndrome (TTTS)
• Fetus in fetu
• Twin reversed arterial perfusion (TRAP) sequence
• Intrauterine growth restriction (IUGR); stunted fetal growth
• Low birth weight of the baby
• Fetal distress
• Stillbirths and fetal mortality

In a vast majority of cases, Fetus Papyraceus does not cause any major complications in the mother. In some cases, the following maternal complications have been reported:

• Disseminated intravascular coagulation (DIC), which can affect the surviving fetus also
• Intra-uterine infection that arises from the fetal remnants including sepsis
• Pre-term labor
• Severe postpartum bleeding/hemorrhage
• Difficult birth resulting in caesarean delivery, due to obstruction by Fetus Papyraceus

How is Fetus Papyraceus Treated?

Fetus Papyraceus is found after childbirth and is seldom diagnosed during pregnancy, unless it is a twin pregnancy and one of the twins vanish. However, even in a twin pregnancy, it can only be speculated that there might have been a Fetus Papyraceus.

In rare cases of an early diagnosis, close monitoring of the mother and surviving fetus, until completion of pregnancy and childbirth is crucial. This is usually achieved through serial ultrasound scans and regular placental examinations.

• If needed, treatment is directed towards managing the maternal fetal complications associated with this condition
• Undertaking treatment of any underlying condition, if present, is important
• A careful and periodic monitoring of the pregnancy is necessary, since Fetus Papyraceus may be a high-risk pregnancy, in some cases
• Psychological support and counseling for the mother

How can Fetus Papyraceus be Prevented?

• Currently, there are no definitive methods available to prevent Fetus Papyraceus
• A careful and periodic monitoring of the pregnancy is advised and recommended

What is the Prognosis of Fetus Papyraceus? (Outcomes/Resolutions)

In a vast majority of cases, Fetus Papyraceus does not significantly affect the growth and development of the surviving fetus. In many cases, there are no associated adverse effects on the mother too. However, some of the following factors may influence the prognostic values:

• Whether it is a monochorionic twin pregnancy or dichorionic twin pregnancy; monochorionic pregnancies are associated with many complications and much poorer outcomes
• The underlying cause of fetal demise and its severity
• Total number of fetuses present; the higher the number, the greater is the risk
• Gestational age at death of fetus
• Time (number of weeks) from fetal death to delivery of surviving child; the larger the period, the lower is the risk

Additional and Relevant Useful Information for Fetus Papyraceus:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/diseases-conditions/pregnancy-related-disorders/