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Cesarean Scar Pregnancy with Iniencephaly and Progression to Placenta Accreta Due to Early Management Rejection

Nieto-Calvache, Albaro José1,2,∗; López-Girón, María Camila3; López-Tenorio, Jaime1; Quintero-Mejía, Juan Carlos4; Zambrano-Regalado, María Andrea1; Pachajoa-Londoño, Harry3,5; Escobar-Vidarte, María Fernanda1,2

Section Editor(s): Pan, Yang; Shi, Dan-Dan

Author Information
doi: 10.1097/FM9.0000000000000025
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To editor:

Cesarean scar pregnancy (CSP) is a rare pathology, with an increasingly clear association with morbidly adherent placenta (MAP).1 Although the most recommended treatment is pregnancy termination by cesarean section and scar resection shortly after diagnosis.2 The final decision regarding management depends on the patient's choice, medical advice, the fertility desire, and the social, religious and emotional background that should also be taken into account.

This is a case report of a CSP diagnosed within the first trimester, coexisting with a fetal malformation incompatible with life (iniencephaly) in a patient who chose to continue her pregnancy and subsequently developed MAP. The complexity and the technical demands required to manage this pathology will be reviewed as well as the considerations of the patient's will.

Case presentation

A 37-year-old catholic patient, gravid 4, cesarean 2, abortion 1, previously asymptomatic with an ultrasound at gestational week 7 that showed CSP (Fig. 1A), for which immediate pregnancy termination was proposed. She received extensive information about the risks of continuing her pregnancy; however, she refused to terminate it. At gestational week 9, an abnormality of the embryonic cephalic pole was detected, suggesting anencephaly (Fig. 1B) and greater distortion of the placental–myometrial interface was seen. Pregnancy termination was recommended again after a multidisciplinary medical board meeting. However, the patient decided to continue with her pregnancy based on the possibility of fetal healing associated with her religious beliefs. An ultrasound follow-up showed the increasingly extensive placental invasion of the myometrium, thus confirming the diagnosis of MAP (Fig. 1C).

Figure 1
Figure 1:
A Ultrasound obtained at gestational week 7. Gestational sac located on the cesarean scar. Thinning of the myometrium between the gestational sac and bladder (arrow) and hypervascularization of the surrounding myometrium toward the cervix (asterisk). B Ultrasound obtained at gestational week 9. Shows abnormality of the embryonic cephalic pole, suggesting morphological alteration that requires verification. Rhombencephalon and prosencephalon are not clearly seen. Ultrasound follow-up suggests anencephaly. C Ultrasound obtained at gestational week 21. Shows placental lacunae (asterisk), the loss of placental–myometrium interface, and the hypervascularization of the vesicouterine space (arrow). D Dissection of vesicouterine space with tweezers and scissors. Obvious neovascularization of the uterine segment and vesicouterine space. Anterocaudal bladder traction with clamps. Right femoral introducer with resuscitative endovascular balloon occlusion of the aorta (REBOA) inside (arrow). Pubis (asterisk).

A specific plan was designed during prenatal follow-up for immediate consultation in case of alarming symptoms. This plan included contacting the MAP clinic coordinator's personal telephone who would alert the entire interdisciplinary team, as well as provide clear written instructions to the paramedical personnel who would provide care during an emergency.

A placental magnetic resonance image was performed at gestational week 32, which revealed a MAP with left parametrial invasion. At this point, a new interdisciplinary meeting was held. After providing extensive counseling, the patient agreed to terminate the pregnancy only after receiving antenatal steroids for inducing fetal lung maturation (even after informing them about the futility of that intervention). A diagnosis of placenta percreta with severe bladder and left parametrial involvement was established during surgery (Fig. 1D). Fetal extraction was performed through fundic hysterotomy and a subsequent total hysterectomy. Intraoperative blood loss was 3 000 cc, which required a transfusion of three units of red blood cells, 30 minutes of aortic occlusion, and postoperative hospitalization for 3 days, of which two of them were at the intensive care unit. Neonate was stillborn, weighing 850 gr with neural tube defect sequence-type myelomeningocele-anencephaly-iniencephaly with retroflection of the upper part of the spine with open flap, and spine defect (myelomeningocele and anencephaly). The neonate also presented mandibular hypoplasia, inadequate neck visibility, posteriorly rotated ears, elbow joint rigidity, and bilateral talipes equinovarus or clubfoot.

Discussion

CSP is a rare type of ectopic pregnancy, with an incidence of 1 in 1 800–2 500 gestations.3 Although its pathophysiology is still unclear, an important factor is a trauma to the uterine wall, with a defect in the basal decidua, which facilitates implantation of the fertilized ovum in the myometrial tissue. Although an early diagnosis is important for adequate management, initial diagnosis using ultrasonography is inaccurate in approximately 14.2% of cases.4 In the present case, the diagnosis was established in an asymptomatic woman at week 7 of pregnancy during the first obstetric ultrasound; therefore, pregnancy termination was recommended. As the patient requested to continue with her pregnancy, the growth of the gestational sac toward the endometrial cavity, compatible with Type-1 cesarean scar ectopic pregnancy, was documented.2

A fetal abnormality incompatible with life, called iniencephaly, is recognized as one of the defects of the neural tube. Its incidence, as described in the literature, does not exceed 10 in 10 000 gestations in the general population. These defects occur due to inadequate development of ventral and dorsal mesodermic mass that leads to anatomical deformities at the base of the skull and in the dorsal column during the first trimester of a pregnancy before the closure of the cephalic pole at 24 days of gestation. When these cases occur an interruption of pregnancy is carried out since it is considered unfeasible. In week 9, when this pathology was documented it was recommended the termination of the pregnancy. However, the patient, strongly influenced by her religious beliefs, decided to continue with her pregnancy waiting for her fetus to heal.

Treatment options included expectant management, medical treatment with methotrexate, surgical management (hysterectomy, dilation, and curettage; vaginal, laparoscopic or laparotomy local resection), uterine artery embolization, or a combination of these strategies. As there is no consensus on a standard management technique, the recommended treatment should consider the patient's hemodynamic status, pregnancy location based on ultrasound (Type 1 or 2 CSP), gestational age, desire for future fertility, patient's and family's level of commitment to follow instructions, and the interdisciplinary team's expertise. In general, pregnancy termination shortly after the diagnosis is advised because continuing the pregnancy is associated with a high rate of morbidity, uterine rupture, placentation abnormalities, the involvement of surrounding organs, hemorrhage, and the requirement of cesarean section and hysterectomy in almost all cases.5 In the present case, surgical resection using laparotomy was proposed at week 7; however, expectant management was established due to the patient's choice. Despite electively performing a cesarean section with prolonged aortic occlusion and ensuring a strict follow-up, massive bleeding was observed. Only carefully selected patients who oppose pregnancy termination, mainly with Type-1 cesarean scar ectopic pregnancy (where the growth of the gestational sac toward the endometrial cavity seems to lead to a low incidence of uterine rupture or bladder involvement), are candidates for expectant management; in such patients, close monitoring is required and there exists significant risks.2

Few cases of cesarean scar ectopic pregnancy, that have resulted in viable neonates, have been reported. A review of the literature conducted in 2014 reported 11 cases of cesarean scar ectopic pregnancy with this type of result; all of these cases required a hysterectomy and were histologically diagnosed with placenta accreta.1

Specifically for this case, neither bleeding nor symptoms associated with cesarean scar ectopic pregnancy were noted. The average gestational age reached (32 weeks) was similar to that observed in the study by Timor-Tritsch.6 The average blood loss in patients with cesarean scar ectopic pregnancy who allow pregnancy progression has been reported as 1 650 mL.1 However, in the present case, a greater volume of bleeding (3 000 mL) was recorded, which required three units of red blood cells, despite aortic endovascular occlusion, making the complexity of this pathology evident.

This is the first study describing the desire to continue with a pregnancy without a prognosis of fetal survival. Reportedly, some patients are willing to “sacrifice their uterus” to obtain a neonate alive.1 However, the situation can be even more critical in medium- or low-income countries where remission is delayed and reference centers for the management of placenta accreta are scarce. From this point of view, patients who choose expectant management endanger their lives.

The decision to terminate a pregnancy owing to fetal abnormality includes complex personal and family processes, which involve grief, anxiety, and the evaluation of moral and religious principles.7 Although a majority of the Latin America and Caribbean populations support voluntary pregnancy termination in cases of risk to the mother's health, most patients with particular religious beliefs have a critical attitude toward this issue.8 However, 27% of women who oppose pregnancy termination change their point of view when they are personally involved in such scenarios.9 In the present case, the patient preferred to put her life at risk instead of performing an act condemned by her religion (ending her pregnancy).

The religious beliefs of patients, their families, and the health care team10 comprise a factor that should be considered by interdisciplinary teams in emergencies in obstetrics. This factor imparts additional complexity to technical and operative demands, which are already considerably high in cases of CSP and MAP.

Conclusions

CSP may progress to morbidity adherent placenta. Both pathologies should be managed by experienced interdisciplinary groups by considering the specific psychological needs of the patient and her family.

Funding

None.

Conflicts of Interest

None.

References

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[8]. Yam EA, Dries-Daffner I, García SG. Abortion opinion research in Latin America and the Caribbean: a review of the literature. Stud Fam Plann 2006;37(4):225–240. doi:10.1111/j.1728-4465.2006.00102.x.
[9]. Zaręba K, Ciebiera M, Bińkowska M, et al. Moral dilemmas of women undergoing pregnancy termination for medical reasons in Poland. Eur J Contracept Reprod Health Care 2017;22(4):305–309. doi:10.1080/13625187.2017.1326095.
[10]. Berer M. Termination of pregnancy as emergency obstetric care: the interpretation of Catholic health policy and the consequences for pregnant women. An analysis of the death of SavitaHalappanavar in Ireland and similar cases. Reprod Health Matters 2013;21(41):9–17. doi:10.1016/S0968-8080(13)41711-1.
Keywords:

Placenta accreta; Cesarean scar pregnancy; Morbidity adherent placenta; Pregnancy termination; Ultrasonography; Prenatal

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