Fetal malformation anencephaly with placenta accreta spectrum (increta): Two different tales in a pregnancy – A rare case report

Indu Lata; Ruchi Gupta; Amrit Gupta; Pallavi Prasad

doi:10.4103/jssrp.jssrp_13_22

CASE REPORT

Year : 2022 | Volume : 3 | Issue : 3 | Page : 74-76

Fetal malformation anencephaly with placenta accreta spectrum (increta): Two different tales in a pregnancy – A rare case report

Indu Lata¹, Ruchi Gupta¹, Amrit Gupta¹, Pallavi Prasad²
¹ Department of Maternal and Reproductive Health, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
² Department of Pathology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Date of Submission	29-Aug-2022
Date of Decision	17-Sep-2022
Date of Acceptance	08-Oct-2022
Date of Web Publication	19-Dec-2022

Correspondence Address:
Indu Lata
Department of Maternal and Reproductive Health, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jssrp.jssrp_13_22

Abstract

Placenta accreta spectrum (PAS) disorders (accreta, increta, and percreta) are rare but are potentially life-threatening obstetric conditions of high maternal and fetal or neonatal morbidity and mortality. In the patient at risk for accreta, obstetrical ultrasonography should be performed and it can be complemented with magnetic resonance imaging. In suspected cases, a multidisciplinary team with clinical expertise should be involved. Anencephaly is a major lethal fetal anomaly of severe central nervous system malformation and the most common type of neural tube defect (NTD). Although the etiology of anencephaly remains unclear, various maternal-related environmental and genetic risk factors have been reported. Termination of pregnancy is offered for all prenatally diagnosed cases. We are presenting management of the rare case of a pregnant woman having placenta increta with fetal NTD malformation in the form of anencephaly.

Keywords: Anencephaly, cesarean, multidisciplinary approach, placenta accreta spectrum, placenta increta

How to cite this article:
Lata I, Gupta R, Gupta A, Prasad P. Fetal malformation anencephaly with placenta accreta spectrum (increta): Two different tales in a pregnancy – A rare case report. J Surg Spec Rural Pract 2022;3:74-6

How to cite this URL:
Lata I, Gupta R, Gupta A, Prasad P. Fetal malformation anencephaly with placenta accreta spectrum (increta): Two different tales in a pregnancy – A rare case report. J Surg Spec Rural Pract [serial online] 2022 [cited 2023 Feb 2];3:74-6. Available from: http://www.jssrp.org/text.asp?2022/3/3/74/364429

Introduction

Placenta accreta spectrum (PAS) is the main cause of obstetric hemorrhage, perinatal hysterectomy, and related high obstetric mortality and morbidity. In this condition, the placenta abnormally attaches directly onto or into the myometrium. It can invade adjacent pelvic organs such as the bladder, rectum, and rarely broad ligament and leads to the highest maternal and perinatal morbidity and mortality.^[1] The incidence of PAS is estimated in 1 in 2510 deliveries (0.04%).^[2] The presence of placenta previa or a morbidly adherent placenta with or without coexisting previous cesarean sections can complicate 2.3% of second-trimester terminations of pregnancy and massive hemorrhage, spontaneous uterine rupture, or subsequent hysterectomy may occur, similar to term delivery.^[3]

Anencephaly may occur in about 0.5%–2% of every 1000 births.^[4] The mortality rate of acephaly fetus is 100% during intrauterine life or it may die within hours or days immediate after birth. The termination of pregnancy is done more than 83%. Anencephaly is associated with other structural anomalies in 12–25% patients and other genetic abnormalities in 1%-5%.^[5] Pregnancy termination in the second trimester with concurrent PAS disorders and lethal congenital malformation may place obstetricians in a nightmare situation due to rarity and limited data on its management. We are reporting successful management not a usual presentation of two separate entities, placenta increta, and anencephaly, of two different etiologies, in one pregnancy.

Case Report

32 years old, third gravida pregnant woman with a past obstetric history of a previous cesarean section three years back and one dilatation and curettage for missed abortion one year back was booked at antenatal care clinic. She was diagnosed with anencephaly in the fetus at 13 weeks at the time of the nuchal translucency scan at our center, was counseled, and advised for termination of pregnancy, which should be chosen in any patient with such malformation. Due to some personal reasons, she failed to get admitted for termination of pregnancy immediately at 13 weeks. At 16-week 3 days gestation, she again presented to the outpatient department visit, on repeat ultrasonography (USG) scan diagnosed with placenta covering internal os with myometrial thinning, lacunar spaces, loss of the retroplacental clear space, and increased vascularity [Figure 1]a. Findings were confirmed with magnetic resonance imaging (MRI) scan shown placenta previa with interruption of the myometrium and bladder wall irregularity along with anencephalic fetus [Figure 1]b.

Figure 1: (a) USG showing placenta previa with the presence of lacunae and increased vascularity. (b) MRI: Black arrow showing invasion of the placenta in myometrium and a white arrow showing anencephalic baby. (c) Specimen of the anencephalic fetus. (d) Uterus specimen showing placental invasion in the myometrium. (e) The section shows chorionic villi infiltrating the myometrial muscle fibers, without an intervening layer of the decidua. Associated fibrin deposition is also seen (H and E, ×20). MRI: Magnetic resonance imaging

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The patient was counseled and planned for hysterotomy with the consent of hysterectomy. On investigation, her hemoglobin (Hb) was 11.3 g%, and liver and kidney function tests and coagulation were within normal limits. We made a multidisciplinary team and case discussed with an interventional radiologist, anesthetist, urologist, transfusion medicine specialist, and intensivist. We arranged packed red cell units and blood products as per the massive transfusion protocol of the institute. In the preoperative period, bilateral internal iliac artery balloon occlusion was done by interventional radiologists. After giving balanced general anesthesia (due to the possibility of blood loss), the fetus was removed from a vertical incision in the upper uterine segment [Figure 1]c. The patient started bleeding profusely due to incomplete separation of the placenta and uterine atonicity.

Bilateral internal iliac artery balloon which was placed electively to secure the blood loss failed to reduce blood loss intraoperatively. The patient was given all recommended uterotonic, bilateral uterine, ovarian artery ligation, and aortic compression to control severe uterine bleeding. However, the patient became hemodynamically unstable and went into hemorrhagic shock (In arterial blood gas analysis [ABG], Hb – 6.0 g%). The patient had a massive hemorrhage (~2.0 Lt) intraoperatively. To save the life of the patient from life-threatening severe hemorrhage, balanced hemostatic resuscitation with blood products transfusion, use of loading and maintenance dose of tranexamic acid, with surgical hemostasis with a decision of hysterectomy was taken [Figure 1]d. During hysterectomy, the bladder was opened up accidently at the time of bladder separation due to the severe focal adherence of the bladder wall. The bladder was repaired in layers with the advice of a urethral catheter to be kept for 14 days postoperatively by the urologist. The patient was shifted to the postoperative intensive care unit for monitoring and stabilization. Repeat complete blood counts and coagulation parameters were checked and Hb – 7.0 g% was optimized with transfusion of two units of PRBC. The patient recovered uneventfully. The fetus and uterus specimens were sent for histopathology [Figure 1]d and [Figure 1]e which confirmed the diagnosis of placenta increta. The patient was discharged to home in stable condition on day 19 of the operation. On subsequent follow-up for the next 2 years, no events or complaints were reported.

Discussion

The frequency of PAS in first, second, third, fourth, and fifth cesarean sections is 3%, 11%, 40%, 61%, and 67%, respectively. The risk of placenta accreta exists upto 2%–5% with any case of placenta previa.^[6] This case has a history of previous cesarean and uterine curettage with partial placenta previa as risk factors. In this case, PAS was diagnosed on USG (with standard classical findings, described in the case report) but due to less gestation, there was difficulty to see bladder invasion, so MRI was done. For placental invasion, the sensitivity and specificity of US are 73% and 89%, respectively, and the sensitivity and specificity of MRI are 89% and 97%, respectively.^[7]

The best surgical management option for PAS is usually a hysterectomy. Conservative treatment in patients without massive hemorrhage and wishes to preserve her fertility option are leaving the placenta in situ is combined with intervention such as a uterine artery or internal iliac artery ligation, angiographic embolization, hysteroscopy resection, or medical treatment with mifepristone or methotrexate. As our patient had only one live child, she wished to preserve her future fertility. Hence, preoperatively, we planned to leave the placenta in situ with medical treatment with methotrexate and angiographic embolization if needed.

Preoperatively, bilateral internal iliac balloon occlusion was done but it failed. Balloon occlusion of the internal iliac artery can temporarily block the internal iliac artery, effectively reduce the blood supply of the uterine artery, and save the time available for the actual surgery. The failure of internal iliac artery balloon occlusion to reduce blood loss may be due to excessive uterine blood flow during pregnancy and extensive intrapelvic vascular anastomosis, the additional blood supply of the uterus by obturator artery, ovarian artery, and femoral artery.

In PAS with placenta previa, many abnormal vascular anastomotic branches exist in the lower part of the uterus, cervix, and upper part of the vagina.^[8] In the setting of uncontrolled uterine bleeding following delivery with a bladder wall invasion reconstructive surgery can be done when the patient is hemodynamically stable. Hence, in the presence of severe hemorrhage shock and for lifesaving, we had to proceed with a hysterectomy, which is standard surgical management in this situation.

The second abnormal condition, in this case, was the presence of anencephaly in the fetus which makes this situation worse for any obstetrician. Pregnancy with PAS but with a normal fetus can be continued until fetal lung maturity occurs. In this condition, a cesarean followed by a hysterectomy can be planned electively which may have less intraoperative or postoperative complications with adequate fetal outcome.

Anencephaly may be due to various maternal-related environmental and genetic risk factors. USG is described as a "Frog eye" sign in the coronal view of the head as no recognizable brain tissue, detected as early as in the first trimester. Definitive diagnosis during the first trimester will allow counseling of parents and termination of pregnancy.^[9] The complications associated with ongoing pregnancies are stillbirth, polyhydramnios, redundant cesarean deliveries, labor induction, shoulder dystocia, and antepartum or postpartum hemorrhage.^[10]

Conclusion

Placenta increta together with anencephaly in a fetus is a very rare entity, and its management is a real challenge in the prevention of any life-threatening hemorrhage. In our case, the aim and plan were to preserve the uterus for future fertility and successful termination of pregnancy. Unfortunately, due to the life-threatening severe hemorrhage shock, despite all measures and balanced hemostatic resuscitation, a hysterectomy had to be done as a lifesaving measure. This case highlights the importance and need of perioperative multidisciplinary team for planning and successful management of such difficult obstetric entities.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest. Consent taken as per institutional policy.

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