Palliative care protocol in fetal medicine for care of the pregnant women and their fetuses

Figueredo, Daniela Valle Almeida; Faquini, Silvia Lourdes Loreto; Guerra, Gláucia Virgínia de Queiroz Lins; Souza, Alex Sandro Rolland

doi:10.1590/1806-9304202520250325-en

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SPECIAL ARTICLE • Rev. Bras. Saude Mater. Infant. 25 • 2025 • https://doi.org/10.1590/1806-9304202520250325-en linkcopy

Palliative care protocol in fetal medicine for care of the pregnant women and their fetuses

Authorship SCIMAGO INSTITUTIONS RANKINGS

Abstract

Perinatal palliative care is an emerging field in fetal medicine, which encompasses fetal abnormalities with conditions that limit fetal or neonatal life in a humanized manner. This protocol was developed based on a literature review and proposes a care model for pregnant women with a prenatal diagnosis of such conditions at the Instituto de Medicina Integral Prof. Fernando Figueira (IMIP). The objective is to standardize care and ensure clarity in communication with parents and family members. The topics covered include: perinatal palliative care, fetal diagnosis of life-limiting conditions, communication, eligibility criteria, conduct, prenatal care, birth, perinatal bereavement, and the puerperal period. It should be noted that this protocol was developed considering regions and/or health institutions with limited technological and/or financial conditions and can be adapted to different contexts.

key words
Palliative care; Perinatology; Prenatal care; Congenital abnormalities

Fetal Condition	Diagnostic Investigation*	Preferred Type of Delivery
	Genetic Conditions
Trisomy 13 or 18	Ultrasound Fetal karyotype (if not performed, palliative care may be indicated depending on strong clinical suspicion and severity of associated fetal malformations).	Vaginal delivery
Triploidy	Ultrasound Quantitative β-hCG	Vaginal delivery
	Musculoskeletal System Abnormalities
“Lethal” skeletal dysplasias	Ultrasound showing severe micromelia, narrow thorax, CT/CA ratio < 0.6, and polyhydramnios Fetal karyotype	Vaginal delivery
	Central Nervous System Abnormalities
Anencephaly	Ultrasound report (signed by two physicians), including two images-one sagittal and one transverse-of the fetal head showing absence of cranial vault and cerebral parenchyma. If legal termination of pregnancy: may be performed in any maternity unit upon signing of the informed consent form, which must be attached to the medical record. If pregnancy is continued: maintain prenatal follow-up and palliative care.	Vaginal delivery
Alobar holoprosencephaly	Ultrasound showing fusion of cerebral hemispheres into a single ventricle. Fetal karyotype (may be associated with trisomy 13).	Vaginal delivery
Extensive encephalocele	Ultrasound showing skull defect with severe externalization of brain tissue (comparable to anencephaly).	Consider vaginal delivery. Cesarean section if exclusive palliative care not defined or in cases of macrocrania (HC >400 mm).
Hydranencephaly	Ultrasound with findings of severe ventriculomegaly occupying almost the entire skull, with no remaining cortex.	Vaginal delivery If BPD >10.5 cm or macrocrania, cesarean delivery at term (consider <37 weeks). If HC <400 mm at term, discuss elective induction to avoid cesarean.
Iniencephaly	Ultrasound showing occipital cranial defect, fixed extension of the fetal head, shortened spine, absence of some vertebrae, and exposure of the spinal canal.	Vaginal delivery
	Cardiac Abnormalities
Hypoplastic left heart syndrome	Ultrasound or fetal echocardiography showing small left ventricle, hypoplastic aorta, and mitral hypoplasia.	Vaginal delivery
Cantrell’s pentalogy	Ultrasound showing complete pentalogy (omphalocele with ectopia cordis, heart defect, diaphragmatic hernia, and pulmonary hypoplasia).	Vaginal delivery
	Urinary System Abnormalities
Bilateral renal agenesis	Ultrasound showing absence of kidneys or renal arteries.	Vaginal delivery
Bilateral polycystic kidney disease	Ultrasound showing enlarged hyperechogenic kidneys and oligo/anhydramnios after the 17^th week.	Vaginal delivery Consider cesarean section if AC increased due to renal volume.
Bilateral multicystic dysplastic kidney disease.	Ultrasound showing cysts replacing renal parenchyma, absence of renal pelvis, nonvisualized bladder, and anhydramnios in the second trimester.	Vaginal delivery
Urethral obstruction	Ultrasound showing megacystis, sometimes extending into the urethra. In complete obstruction, anhydramnios from the second trimester is observed.	Vaginal delivery If severe megacystis, consider aspiration to enable vaginal delivery, or cesarean if necessary.
	Other Anomalies
Cystic hygroma	Ultrasound showing a large septated nuchal edema. Fetal karyotype.	Vaginal delivery Cesarean if the hygroma is large enough to cause dystocia.
Conjoined twins (inoperable)	Ultrasound showing fused twins sharing vital structures. Severe prognosis, but each case must be evaluated individually by a multidisciplinary team.	Vaginal delivery Cesarean if advanced gestational age (prevents passage through the birth canal).
Body stalk syndrome	Ultrasound showing extensive thoracoabdominal wall defect with externalized organs covered by amnion and placenta, severe kyphoscoliosis, and short umbilical cord.	Vaginal delivery
Severe nonimmune hydrops fetalis	Ultrasound showing subcutaneous tissue edema and fluid accumulation (effusion) in at least one compartment, possibly with hydropic placenta and polyhydramnios. Fetal karyotype. Risk definition: degree of effusion, affected sites, gestational age, and associated conditions (genetic diseases).	Vaginal delivery Cesarean if severe edema prevents passage through the birth canal.
Acardia	Ultrasound in monochorionic twin pregnancy showing “pump” fetus and acardiac fetus. Explain to the family that the acardiac fetus can not be preserved.	Delivery route depends on the condition of the “pump” fetus (alive).
Sirenomelia	Ultrasound showing fusion of lower limbs with associated genitourinary and gastrointestinal anomalies.	Vaginal delivery
Congenital high airway obstruction syndrome (CHAOS)	Ultrasound showing enlarged, hyperechogenic lungs with cardiac compression, possibly ascites or hydrops. MRI if necessary to assess obstruction level.	Assess delivery route depending on CA due to risk of dystocia.
	Malformations with combined intensive and palliative care approaches
Congenital diaphragmatic hernia with severe pulmonary hypoplasia.	Ultrasound showing abdominal contents in the thoracic cavity, cardiac compression with axis deviation, and reduced lung size (O/E LHR <25%). Fetal karyotype	Vaginal delivery. Await spontaneous labor until 39 weeks (if no maternal comorbidities). At birth, plan early neonatal intubation

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Instituto de Medicina Integral Prof. Fernando Figueira Rua dos Coelhos, 300. Boa Vista, 50070-550 Recife PE Brasil, Tel./Fax: +55 81 2122-4141 - Recife - PR - Brazil
E-mail: revista@imip.org.br

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[TFN1] ^*
Obstetric or morphological ultrasound preferably performed by a specialist in fetal medicine. Fetal karyotype performed, if available.

[2] CT/CA = chest circumference/abdominal circumference ratio; HC = head circumference; O/E LHR = observed-to-expected lung-to-head ratio; EXIT = Ex Utero Intrapartum Treatment.