Table 4 Overview of advice mentioned in the included articles from the literature search AMC and pregnancy
Period | Counselling aspect | Explanation |
|---|---|---|
Prepregnancy | Genetic counseling | Geneticists informs about the types of AMC and update on possible genetic tests [1] |
Prepregnancy counselling | Multidisciplinary approach [8, 35] Contraceptive advice [35] Discussing facts and challenges of pregnancy and AMC [37] Respiratory function test if applicable [37] | |
Pregnancy | Medical history | Obstetric history (prior pregnancies, mode and time of delivery, birth weight) [24, 25] Prior operations, including type of anesthesia and possible advices [24, 27] Family history [30] Use of medication [30] Mobility: independent walking, walking with aids, wheelchair bound, immobility [27] |
Physical examination | Physical examination with extra focus of members of multidisciplinary team (e.g. gynaecologist, internist, pulmonologist, anesthesiologist) [25, 27] Weight, height, BMI [25] Extremities including mobility (including range of movements of the joints) [8] Cardiovascular and respiratory system [40] Head and neck area (e.g. micrognathia or high arched palate), including Mallampati score and neck mobility [24, 25, 39] Shoulders (e.g. deformity of the scapula) [25] Spine (e.g. scoliosis, spina bifida, sacral agenesia or vertebral anomalies) [25] Cardiovascular system (e.g. heart diseases) [25] Respiratory system (e.g. tracheoesophageal fistula or hypoplastic lungs) [25] Genitourinary system (e.g. rectal or labial defects) [25] Abdomen (e.g. inguinal hernia) [25] Venous access evaluation [30] | |
Home management | Needs for home management dependent of mobility (aids) [8] Local occupational therapist (for home modifications) [8] Social worker [8] | |
Tromboprophylaxis | Tailored counselling about using thromboprophylaxis during and after the pregnancy [28, 31] | |
Cervix length measurements | Suggested in relation to increased risk of preterm labour [34] | |
In case of breathlessness (AMC related) | Monitoring cardio-respiratory condition [31] Peak expiratory flow rates measurements [31, 37] Chest radiography (signs of infection?) [34] Evaluation of the uterine fundus in relation to diaphragm [34] Steroids for maternal lungs [34] Chest physiotherapy. [34] Advice upright sleeping position to reduce elevation of the diaphragm [34] In case of worsening: counselling about continuation or terminating the pregnancy [34] | |
Serial ultrasound investigations | Healthcare providers should be aware of recurrent AMC in the fetus [8, 37]: -Â Features due to limited motility: joint contractures (e.g. clubfoot), micrognathia, decreased fetal movements, altered amniotic fluid -Â Associated anomalies: brain and hearth anomalies, heart, joint webbing -Â Fetal growth restriction | |
Prenatal testing | First trimester test for aneuploidies [37] Genetic counselling about prenatal invasive testing: risk calculation for fetal AMC [24, 37] Genetic testing update possibilities(chromosomal or monogenic) [31] | |
Anesthetic | ||
Anesthetic assessment | Early in pregnancy anesthetic assessment [30, 39] Expected difficulties during administration of analgesia [30, 39] Craniofacial evaluation: cleft palate, laryngeal stenosis, craniosynosthosis, micrognathia Spinal abnormalities: scoliosis, spina bifida, or sacral agenesis could have abnormal cerebrospinal fluid dynamics [25, 39] Expected anesthetic problems during infusion placement (e.g. due to joint contractures or scarring), or insertion the catheter of the regional analgesia (e.g. due to spinal anomalies) [25] Choice of anesthesia technique should be tailored to the individual patient's anatomy, overall health, and the specific surgical procedure to optimize safety and efficacy [25] | |
General versus regional analgesia | Weigh the potential difficulties and risks [27, 31, 34]: - Additional risks during general analgesia compared to regional analgesia are: difficulties during intubation due to a limited neck mobility and problems related to the decreased cardiopulmonary function - Patients with AMC could react unpredictable on medications (e.g. muscle relaxants and inhalation anesthetics). Therefore, proper dosing and careful monitoring are crucial [25, 37] - Spinal analgesia could be challenging in patients with AMC who have spine deformities (e.g. scoliosis) Therefore, identifying and targeting nerves for blocks may be more difficult due to the altered anatomy | |
Delivery | Mode of delivery | Counselling about the mode of delivery, individualized and dependent of maternal and fetal investigations [8] |
Timing of delivery | In general term age. Challenge in case of for example maternal pulmonary discomfort (e.g. breathlessness) before term age, while the fetus is a good condition [31] A pulmonary function test is suggested after 28 weeks gestational age in symptomatic patients and an electrocardiogram in asymptomatic patients and adjustments in more upright sleeping position [34] | |
Postpartum | Maternal | |
Home management | Modifications to a bassinet to enable self-sufficient care of the newborn [8, 34] Social service provision: need for carers and housing [34] | |
Thromboprophylaxis | Continuation of 6 weeks, in line with recommendation of the Royal College of Obstetricians and Gynaecologists [31] | |
Physical examination (neonatal) | Joint stature including range of motion, features of AMC, general physical evaluation, and birth weight [24] | |