Treatment is conservative in the absence of neurologic compromise. The many studies now published on various treatment modalities for lumbopelvic pain have discordant results. Moderate quality evidence from single studies supports the use of structured exercise programs, acetaminophen (paracetamol) administration, osteomanipulative therapy, pelvic belts, and acupuncture in the treatment of lumbopelvic pain. A 2019 systematic review on prenatal exercise that included 32 studies concluded that exercise does not prevent the occurrence of lumbopelvic pain, but does reduce the severity of the pain. A 2021 systematic review and metaanalysis on conservative care strategies supports muscle relaxation therapy and taping for pain, and transcutaneous electrical nerve stimulation to improve physical motion. Patients with severe neurologic signs or symptoms of disc herniation should be assessed by a consultant neurosurgeon or neurologist who can provide recommendations for intrapartum and postpartum care. Because the disability associated with lumbopelvic pain, especially pelvic girdle pain, can impair the woman’s ability to function postpartum, it is important to diagnose pregnancy-related lumbopelvic pain and treat it appropriately, including into the postpartum period. A 2023 study looking at self-management techniques using an established self-efficacy scale for chronic medical conditions in a group of postpartum women showed that those with low self-efficacy scores were twice as likely to have ongoing pregnancy-related back pain.

No evidence suggests that epidural or spinal anesthesia is contraindicated in patients with pregnancy-related lumbopelvic pain. The anesthesia provider may administer neuraxial anesthesia, even to patients with sciatica. However, neurologic signs and symptoms should be first identified, delineated, and recorded. During epidural labor analgesia it is prudent to administer local anesthetics in dilute solutions, with or without an opioid, to minimize motor block to reduce any further stress on relaxed sacroiliac joints. Women with lumbopelvic pain may be reluctant to have neuraxial anesthesia because of concern that it may aggravate symptoms. Published evidence does not support this fear and reassurance may be required.

Careful attention to the positioning of the patient with back complaints is critical. The patient must not be placed in a position that they could not tolerate before the administration of neuraxial anesthesia. The lithotomy position puts significant stress on the lower back and should be avoided whenever possible; if used, care must be taken to raise and lower both legs simultaneously to prevent injury to the lumbar spine, and extreme positions must be avoided. Finally, caregivers should avoid rotational movements of the spine during transfer of the patient between the bed and the operating table.

It is not uncommon for obstetric providers to offer pregnant women who have had persistent chronic low back pain the option of cesarean delivery to decrease the potential for further back injury during labor. There are no data to either encourage or discourage this option.

The anesthesia provider may offer epidural or spinal anesthesia to patients with previous lumbar spine pathology or surgery after an appropriate history and screening examination to identify any neurologic deficits. A decreased success rate for epidural anesthesia is possible, especially in patients who have had extensive surgery. Nonetheless, the experienced anesthesia provider will likely administer epidural anesthesia successfully in most patients. Spinal anesthesia may be more reliable than epidural anesthesia in these patients.

When scoliosis develops secondary to a neurologic or myopathic disorder, abnormal respiratory function results not only from the skeletal deformity but also from abnormalities in the central control of respiration and the supraspinal innervation of the respiratory muscles, as well as from the loss of muscle function caused by the underlying disorder. Respiratory function may be further compromised by: (1) impairment of the defense mechanisms of the airways caused by loss of control of the pharynx and the larynx, (2) an ineffective cough mechanism, and (3) infrequent or reduced large breaths. Recurrent aspiration pneumonitis may result from compromised protective airway reflexes. In general, the prognosis of scoliosis caused by neuromuscular disease is poorer than that of idiopathic scoliosis and is determined predominantly by progression of the primary disorder. Affected patients typically develop irreversible respiratory failure at a younger age, and pulmonary hypertension is common.

In a systematic review of 22 studies that described pregnancy outcomes in women with adolescent idiopathic scoliosis (AIS) , Dewan et al. found that more women with AIS are likely to remain nulliparous; however, those who do have children have an equal number of children as the general population and at the same mean maternal age at time of first delivery, independent of surgery or bracing. Pregnancy may exacerbate the severity of the spinal curvature, with changes of 5 to 10 degrees in 25% of patients and more than 10 degrees in 10% of patients; however, the clinical significance of these changes is not known, and some studies have not shown a curve change due to pregnancy. Curve progression is more marked in those with prepregnancy curves greater than 50 degrees. The number of pregnancies is variably associated with curve progression. Development of nondisabling back pain during pregnancy is common in all patients with AIS, regardless of prior treatment and degree of curvature.

The physiologic changes of pregnancy include decreases in both functional residual and closing capacities and increases in minute ventilation and oxygen demand (see Chapter 2 ). Thoracic cage circumference normally increases during pregnancy because of increases in both anteroposterior and transverse diameters. If the chest cage is relatively fixed by scoliosis, the diaphragm is responsible for all increments in minute ventilation. As the enlarging uterus causes elevation of the diaphragm, diaphragmatic activity is restricted and further decreases in residual and closing capacities may occur, which may result in greater ventilation-perfusion mismatch and decreased arterial oxygen content. The antepartum onset of new symptoms of respiratory compromise or the exacerbation of preexisting symptomatology is associated with higher maternal morbidity and a greater likelihood that assisted ventilation will be required after cesarean delivery. Patients with a severe curve (i.e., Cobb angle > 60 degrees) but good cardiopulmonary function tolerate pregnancy well, but those with significant cardiopulmonary compromise, especially with pulmonary hypertension, have high mortality.

Exertional dyspnea is uncommon in patients with scoliosis who have curves greater than 70 degrees, but it becomes more common as the deformity exceeds 100 degrees. In younger patients with a curve < 70 degrees, exercise capacity is more likely to be impaired because of the lack of regular aerobic exercise and subsequent deconditioning rather than intrinsic ventilatory impairment. Although dyspnea is common in pregnancy, two features help distinguish physiologic from pathologic dyspnea. First, physiologic dyspnea tends to occur earlier in pregnancy and often plateaus or even improves as term approaches, whereas the pathologic dyspnea of cardiopulmonary decompensation more often begins in the second half of pregnancy and is progressive, often becoming most severe as gestation advances and the physiologic loading is maximal. Second, physiologic dyspnea is rarely extreme, and patients can maintain most daily activities. Dyspnea that is extreme or has a limiting effect on normal activity may signal maternal cardiorespiratory decompensation. Dyspnea at rest is also rare in the absence of cardiopulmonary dysfunction, as is dyspnea that is acute in onset or progressive and intractable.

Minute ventilation typically increases by 45% during pregnancy. Minute ventilation of the unmedicated parturient increases by a further 70% to 140% in the first stage of labor and by 120% to 200% in the second stage. Oxygen consumption increases above prelabor values by 40% in the first stage and 75% in the second stage (see Chapter 2 ). These levels may be unattainable by the scoliotic parturient with restrictive lung disease, and respiratory failure and hypoxemia may result during labor.

Pregnant women with pulmonary hypertension have limited ability to increase cardiac output. During normal pregnancy, cardiac output increases 40% to 50% above nonpregnant measurements; during labor and delivery, even greater increases are observed (see Chapter 2 ). These increases are achieved by way of both larger stroke volume and higher heart rate. These demands may put an excessive burden on the cardiovascular system in parturients who had marginal cardiac reserve before pregnancy. If the right ventricle fails in the presence of pulmonary hypertension, left ventricular filling will decrease and low-output failure and sudden death may occur.

Pregnant women with corrected scoliosis tolerate pregnancy, labor, and delivery well. In the absence of major lumbosacral deformity, there is little alteration of the pelvic cavity, and the incidence of malpresentation is not increased. Uterine function is normal and labor is not prolonged. Spontaneous vaginal delivery is anticipated and cesarean delivery should be reserved for obstetric indications.

The literature is conflicting as to whether the cesarean delivery rate is greater in women with corrected or uncorrected scoliosis. In a retrospective study from Israel, 98 women with idiopathic scoliosis from a cohort of more than 220,000 were reviewed for birth outcomes ; women with scoliosis (corrected or uncorrected) were not found to have an increased risk for cesarean delivery. Conversely, Swany et al. reviewed 60 female patients who underwent treatment for AIS with bracing, surgery, or observation between 1975 and 1992 and had obstetric history available. They found that the overall cesarean delivery rate of 31% for all AIS patients (operative and nonoperative) was greater than both state and national rates. Although the cesarean delivery rate was not significantly different between the operative group (35.5%) and the nonoperative (observed or braced) group (25.9%), the cesarean delivery rate was significantly higher than both state and national rates in the operative group but not in the nonoperative group. There are undoubtedly multiple confounding factors that influence the decision around mode of delivery, including patient preference, consideration of potentially difficult neuraxial anesthesia, and general obstetric preference to be “on the safe side.”

Pelvic abnormalities are more common when scoliosis is associated with neuromuscular disorders and in patients with a severe, uncorrected curve. In addition, abdominal and pelvic muscle weakness predisposes parturients to problems with expulsion of the infant during the second stage of labor and may necessitate instrumental vaginal delivery. The need for instrumental or cesarean delivery is likely related to the severity of skeletal deformity, the resulting maternal compromise, and cephalopelvic disproportion.

In the second stage of labor, the diaphragm has a nonrespiratory function. With expulsive efforts, maximal isometric contractions may be sustained for 20 seconds or more; diaphragmatic fatigue has been demonstrated even in normal laboring women. In parturients whose diaphragmatic function is compromised by neuromuscular disease or severe scoliosis, the potential for fatigue and failure is greater; expulsive forces are decreased, the second stage may be prolonged, and a trial of labor may fail, necessitating instrumental or cesarean delivery. In addition, women with severe cardiopulmonary disease (especially those with gestational decompensation) may require urgent or emergency cesarean delivery because of maternal compromise or nonreassuring fetal status.

Pregnant women who have thoracolumbar scoliosis with a Cobb angle > 30 degrees or who have undergone spinal instrumentation and fusion for scoliosis should be referred for antepartum anesthetic consultation. The anesthesia provider should (1) determine the etiology of the scoliosis as well as the severity and stability of the curve, (2) obtain a history of maternal musculoskeletal and cardiopulmonary symptoms, and (3) review prior obstetric and anesthetic experiences. For patients with scoliosis secondary to a neuromuscular disorder, the anesthesia provider should also become familiar with anesthetic considerations specific to the underlying disorder.

Women with suspected or evident pulmonary compromise should undergo evaluation by a pulmonologist, and pulmonary function studies and arterial blood gas measurements should be obtained. These patients must be reevaluated periodically to ensure that they are tolerating the increasing physiologic demands of pregnancy. Echocardiography is useful to assess right-sided heart function in patients with one or more of the following: (1) a curve ≥ 60 degrees, (2) hypoxemia on arterial blood gas measurement, (3) moderate or greater reductions in predicted lung volumes or flows, (4) pulmonary hypertension, and (5) evidence of decompensating cardiopulmonary status. Radiographic studies performed before pregnancy and operative notes describing spinal surgical procedures should be reviewed before neuraxial anesthesia is initiated in any patient with significant scoliosis or previous spinal surgery. The anesthesia provider should also examine the spine and note the surface landmarks and interspaces that are least affected by the deformity. Modes of analgesia and anesthesia for labor and delivery can be discussed during antepartum consultation.

Invasive hemodynamic monitoring is rarely indicated during labor and delivery. Pulmonary function studies that suggest significant respiratory compromise or clinical evidence of impending respiratory failure may warrant placement of an arterial catheter and serial assessment of blood gas measurements. Echocardiographic demonstration of significant right-sided heart dysfunction may warrant invasive or noninvasive cardiac function monitoring.

The anesthesia provider may offer neuraxial analgesia for labor and delivery to patients with severe thoracolumbar scoliosis. Identification of the epidural space is more difficult in these patients and the anesthesia provider should anticipate a greater incidence of complications. It is useful to remember the presence of the vertebral rotation during the performance of neuraxial anesthesia in a patient with a significant lumbar curve, which results in the spinous processes (which often may be structurally deformed) rotating into the concavity of the curve. Therefore, the midline of the epidural space is deviated toward the convexity of the curve relative to the spinous process palpable at the skin level (see Figs. 46.2 and 46.3 ). The extent of lateral deviation is determined largely by the severity of the deformity. One method of placing an epidural or spinal needle is to direct the needle from a palpated spinous process toward the convexity of the curve, often at a substantial angle. Alternatively, one can use a modified paramedian approach starting at the estimated true midline of the epidural space but directing the needle perpendicular to the skin into the epidural space (see Fig. 46.3 ). The experienced anesthesia provider can track the resistance of both the interspinous ligament and the ligamentum flavum to maintain the correct course into the epidural space. The extent of the local anatomic distortion is the limiting factor, and the selection of spaces that are least involved with the curve is advised.

Image of the Lumbosacral Vertebrae Affected by a Scoliotic Curve to the Left.

Note the axial and lateral rotation of the vertebral bodies, and the rotation of the affected spinous processes (blue dots) into the concavity of the curve; these spinous processes are frequently anatomically abnormal. The apparent midline (trajectory A) suggested by palpated surface landmarks is to the right of the true axial midline. The figure demonstrates two approaches to enter the epidural/subarachnoid space: one where the needle is directed along the line of a spinous process toward the convexity of the curve (trajectory B) , and the other a modified paramedian approach where needle entry starts on the convex side of the curve and is directed perpendicular to the skin (trajectory C) .

Illustration by Naveen Nathan, MD, NorthShore University HealthSystem, University of Chicago Pritzker School of Medicine, Evanston, IL.

Ultrasonography is useful to facilitate administration of neuraxial anesthesia in parturients with challenging spinal anatomy, including those with scoliosis. Asymmetry of the spine is not difficult to appreciate; learning how to use the information and determine the optimal space and needle direction requires more experience. The longitudinal paramedian view may provide the best information with respect to the widest interlaminar space, and the transverse view shows presence of rotation and asymmetry. There are a few studies on the use of preprocedural ultrasonography to identify the best space and angle of approach in parturients with difficult surface anatomy. Ekinci et al. and Creaney et al. randomized patients with poor palpable surface landmarks into ultrasonography-facilitated and landmark palpation-only groups. Both studies found fewer needle passes in the ultrasonography group with no increase in procedural time. Chin et al. described a simplified technique for facilitating neuraxial anesthesia in patients with difficult anatomy, including scoliosis. Using a transverse view, the anesthesia provider can visualize the rotation of the spinous process and use this information to perform essentially a paramedian approach ( Fig. 46.4 ). Park et al. randomized 44 patients with abnormal spinal anatomy to preprocedural ultrasound-assisted spinal placement or to a surface landmark–guided technique. In the ultrasound group, fewer needle passes were required (1.5 versus 6) and there was higher first-pass success (50% versus 9%) and less procedural pain.

Ultrasonography-Assisted Paramedian Approach to Neuraxial Blockade in Patients With Difficult Anatomy.

An illustration of needle trajectory in the paraspinous approach in (A) the axial view, (B) the posteroanterior view, and (C) the lateral view of the spine. The white dotted lines in (B) create a rectangular shape that represents the ultrasound probe held in the transverse position as one scans for the neuraxial midline and spinous processes. Ultrasonography (D) and computed tomography (E) axial images of spine rotation in a patient with lumbar scoliosis. When the ultrasound probe is placed perpendicular to the patient’s back (F), the rotation is evident from the angulation of the acoustic shadow of the spinous process. Paraspinous needle insertion should be performed 1 cm lateral to the midline (defined by the tip of the spinous process), but without medial angulation.

Reprinted with permission from Chin KJ, Perlas A, Chan V. The ultrasound-assisted paraspinous approach to lumbar neuraxial blockade: a simplified technique in patients with difficult anatomy. Acta Anaesthesiol Scand . 2015;59:668–673.

Local anesthetic dose requirements for epidural and spinal anesthesia in the patient with scoliosis are variable. Moreover, during administration of spinal anesthesia in a patient with a severe scoliotic curve, a hyperbaric local anesthetic solution may pool in dependent portions of the spine, resulting in an inadequate block, leading some anesthesia providers to choose an isobaric local anesthetic solution. It may be preferable to use a continuous technique in women with severe scoliosis so that the dose of local anesthetic agent can be titrated to the desired segmental level of anesthesia.

When offering neuraxial anesthesia to patients with a history of corrective spine surgery, the anesthesia provider must consider the following potential problems:

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  Persistent back pain occurs in many patients with corrected scoliosis and correlates with both the extent of fusion and the time since surgery.

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  Degenerative changes occur in the spine below the area of fusion, and there is a higher incidence of both retrolisthesis and spondylolisthesis.

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  MRI studies showed that 8% of patients with AIS had other neuraxial lesions, including syringomyelia (35%), Chiari I malformation with syrinx (28%), and isolated Chiari I malformation (25%). This finding supports consideration of MRI prior to administration of neuraxial anesthesia.

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  Twenty percent of patients undergo fusion to the lowest lumbar levels, limiting the potential for neuraxial anesthesia.

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  Insertion of an epidural needle in the fused area may not be possible because of the presence of instrumentation, scar tissue, and bone graft material.

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  Intraoperative trauma to the ligamentum flavum may result in adhesions in or obliteration of the epidural space; these changes may interfere with the spread of injected local anesthetic.

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  Obliteration of the epidural space may increase the incidence of unintentional dural puncture.

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  These patients often manifest a high level of anxiety about their backs and may be reluctant to have neuraxial anesthesia.

In 2009, Ko and Leffert published a qualitative literature review on neuraxial anesthesia in the parturient with scoliosis. The review described 117 attempted neuraxial procedures, the majority (93) in surgically corrected patients. Overall, 71% had a successful neuraxial block; however, the corrected patients proved to be more challenging, with a 69% success rate versus 79% for the uncorrected group. The challenges in corrected patients included inability to place the needle (22%), multiple attempts (13%), patchy analgesia (10%), excessive local anesthetic dose requirement (9%), unintentional dural puncture (4%), and inadequate analgesia (4%). In the uncorrected group, the issues included patchy, asymmetric, or unilateral blocks (each 8%) and multiple attempts or failed placement (each 4%). There were also two cases of persistent low back pain of unknown etiology. Complications occurred more frequently in patients with fusion that extended to the lower lumbar and lumbosacral interspaces than in those with fusion that ended in the upper lumbar spine. These findings were corroborated by Bauchat et al. in a prospective case-matched study in which 41 women with previous corrective surgery were compared with 41 healthy matched controls. The rate of neuraxial analgesia failure was higher (12% versus 0%) and the mean time to placement longer (by 41%) in the surgical correction group; however, there was no difference in local anesthetic requirement to maintain analgesia.

Thus, in patients with severe or corrected scoliosis, both the anesthesia provider and the patient should anticipate the possibility that neuraxial analgesia may fail with or without ultrasonography. Alternative modes of intrapartum analgesia include administration of single-shot spinal analgesia (which may be repeated), caudal analgesia, patient-controlled intravenous opioid analgesia, and inhalation analgesia especially with nitrous oxide. A continuous spinal technique is a reasonable alternative to an epidural technique for labor or cesarean delivery in parturients who have undergone major spinal surgery with instrumentation. The dural-puncture epidural technique may also be considered as the presence of cerebrospinal fluid (CSF) confirms the epidural needle tip location and may enhance spread of epidural local anesthetic (see Chapter 24 ).

Tethered cord syndrome is characterized by neurologic deterioration secondary to traction on the conus medullaris, which typically, but not invariably, is low lying (L2–L4). Congenital abnormalities of the spinal cord, such as lipoma, tight filum terminale, dermal sinus, and split cord malformations, are found in more than 50% of patients with adult-onset tethered cord syndrome.

Adult tethered cord syndrome associated with CSD is different from tethered cord syndrome occurring secondary to OSD. MRI studies suggest that tethering is present in virtually all patients with OSD. Although many patients with CSD do not have obvious neurologic impairment secondary to the tethering, at least 25% of adults with tethered cord syndrome have a long history of minor orthopedic, neurologic, and/or urologic issues. Others present with acute symptoms after a precipitating event that stretched the spinal cord, such as heavy lifting, multiple pregnancies, repeated bending, or placement in the lithotomy position. Of note, more than 50% of adults with tethered cord syndrome present with only a history of low back and leg pain before a precipitating event that leads to the diagnosis. Menezes et al. followed 24 adult patients recently diagnosed with tethered cord syndrome and found that 80% presented with nondermatomal low back or perineal pain and 87% with bladder dysfunction. The underlying dysraphic lesions were taut filum in eight patients, conus lipoma in seven, and diastematomyelia in seven. The majority had cutaneous manifestations, including dermal sinus in six patients, hypertrichosis in five, and skin tag/cleft/dimple/subcutaneous mass in five. The common isolated finding of a defective laminar arch (spina bifida) is not associated with a low-lying or tethered cord.

Low-lying spinal cord and the possibility of undiagnosed tethered cord syndrome have come to the attention of obstetric anesthesia providers with the publication of several case reports of neurologic injury after spinal or epidural anesthesia in women subsequently diagnosed with CSD, in whom typically there was no obvious history or cutaneous stigmata. An important feature of adult tethered cord syndrome is that the low-lying cord is located more posteriorly than a normal cord, increasing the likelihood of direct needle trauma during administration of spinal or epidural anesthesia ( Fig. 46.5 ).

Magnetic resonance image of the spine in a 27-y-old woman with history of a lumbar myelomeningocele excised as a neonate, with residual bladder dysfunction. A tethered spinal cord is present with a typical posterior low-lying position. The white arrow indicates the termination of the conus medullaris at L4 to L5, and the black arrow indicates the filum terminale located at L5 to S1. (This patient had a vaginal delivery at term, requiring only nitrous oxide for analgesia.)

OSD is usually associated with Chiari II malformation , which is characterized by cerebellar herniation through the foramen magnum and descent of the pons and medulla. Tethered cord from a tight filum terminale has also been deemed causal in the development of syringomyelia and subsequent Chiari II malformation. Symptoms are more common if the cerebellar herniation exceeds 12 mm or if syringomyelia is present.

As the number of patients with spinal dysraphism increases, more data are available on pregnancy-associated issues and pregnancy outcomes. Information from large US databases with more than 11,000 women with spinal dysraphism has revealed a set of complications that differs from the urinary tract infections and pressure sores that were commonly reported in the past. Complications now include increased risk for pulmonary embolism/deep venous thrombosis, seizures, and hemorrhage; patients with hydrocephalus or Chiari II malformation are more likely to suffer from the first two of these complications and are more likely to have cesarean delivery. Other publications have shown that women with OSD commonly suffer from urologic and mobility issues during pregnancy and are more likely to be obese and develop diabetes. Those with kyphoscoliosis may have rapidly compromised respiratory function as the uterus enlarges. Women with known tethered cord syndrome should avoid both the squatting position and a prolonged lithotomy position for delivery.

Cesarean delivery is reserved for obstetric indications, and its incidence is increased and proportional to the severity of the underlying defect and its consequences. Vaginal delivery is more common in women who are independently mobile, is less common in wheelchair-dependent patients, and is not associated with increased complications. Sterling et al. reviewed the records of 32 women with spinal cord injury, 69% of whom had spinal dysraphism, and found a 60% cesarean delivery rate. Shepard et al. examined 9 years of data from the National Inpatient Sample, identifying 9516 deliveries by women with spinal dysraphism. The rate of cesarean delivery was significantly higher in women with spinal dysraphism compared with those without the diagnosis (53% versus 32%), and was associated with increased maternal morbidity including preterm delivery, blood transfusion, and urinary tract infection. Those with more severe lesions and/or hydrocephalus were more likely to have cesarean delivery. Sciscent et al. published similar results from another large US database in 2023 that included 11,405 women with spinal dysraphism. Similar data have recently been published from Quebec, Canada, showing higher rates of cesarean delivery, significantly increased respiratory morbidity including the need for tracheal intubation, and significantly longer hospital length of stay.

Administration of epidural or spinal anesthesia may be considered in women with various forms of spinal dysraphism and stable neurologic function. However, Sciscent et al. found that patients with spinal dysraphism were only 40% as likely to receive neuraxial anesthesia compared with patients without spinal dysraphism. Imaging studies provide valuable information on neural anatomy and facilitate anesthetic management, allowing for complex collaborative planning that includes the patient and family. A neurologic examination, as well as a full discussion with the patient of the risks and benefits of neuraxial anesthesia, including risk for inadequate block, should be performed and documented before the administration of neuraxial anesthesia.

An isolated laminar arch defect rarely causes issues for administration of neuraxial anesthesia. First, the lesion typically occurs at the L5 to S1 segment, below the level at which most epidural and spinal anesthetics are administered. Second, the most common anomaly is a simple midline split in the lamina, and this defect rarely seems to interfere with either the performance or the establishment of spinal or epidural anesthesia. The epidural space may be incomplete or discontinuous across the level of an isolated laminar arch defect because of the variable formation of the ligamentum flavum at this site. An attempt to identify the epidural space at the site of this lesion may result in unintentional dural puncture.

In patients with surgically corrected OSD, the anesthesia provider should be aware that the terminal portion of the spinal cord typically lies at a vertebral level lower than normal; detethering surgery in the patient with OSD does not usually result in the conus ascending to a normal position. CSD without a subcutaneous mass is of concern because of the possibility of a low-lying, posteriorly located, and tethered spinal cord, potentially with no cutaneous stigmata. A neurologic history should be taken and a screening neurologic and lower limb examination should be performed in all women known to have a defective laminar arch, preferably antenatally, to determine whether MRI for spinal dysraphism is necessary. The presence of skin dimpling (>2.5 cm from the anal margin), hair tufts, pseudotails, and midline lumbosacral hemangiomas should raise suspicion of an underlying cord abnormality. In our judgment, in women with a known low-lying cord, epidural anesthesia performed by an experienced anesthesia provider is safer than spinal anesthesia, but should be performed below the level of the cord. If spinal dysraphism is suspected but no imaging studies are available (MRI being the preferred modality), it may be prudent to avoid neuraxial anesthesia.

In the patient with an OSD lesion, the anesthesia provider should determine the level of the lesion and whether the patient has residual spinal cord function below it. Patients with a complete lesion at or above T11 are likely to experience painless labor. However, the risk for autonomic hyperreflexia should be evaluated in patients with thoracic lesions, and neuraxial anesthesia should be provided if the patient is deemed to be at risk; this issue is especially important if the lesion is between T5 and T8 (see Chapter 47 ). If the patient has undergone ventricular shunt placement, the current status of the shunt should be determined. Neurosurgical consultation should be obtained if questions remain about the requirement for, or function of, the shunt. Baseline renal function should be assessed as well as pulmonary function, especially in patients with scoliosis.

There are published reports of the use of epidural and spinal anesthesia in patients with OSD. However, experience is limited, and most published series of pregnant women with OSD report neither the type of anesthesia nor complications. Tidmarsh and May reported the management of intrapartum analgesia in 16 patients with spinal dysraphism, eight of whom had myelomeningocele. Five of the eight patients with OSD received epidural analgesia for labor and delivery. Three patients had a “normal” block, one patient had a sensory level that was higher than expected (T3 after the administration of 10 mL of 0.25% bupivacaine), and one patient had poor sacral analgesia. In the UK Registry of High-Risk Obstetric Anesthesia, the period from 1997 to 2002 included 23 cases of parturients with spinal dysraphisms among 102 cases of neurologic conditions. Of those, the extent of the lesion was defined further in only 10 cases, and eight had only a bony arch defect. Neuraxial anesthesia was provided in eight cases. Only epidural anesthesia was used owing to concern about a low-lying or tethered cord; only five patients had MRI performed before delivery. Murphy et al. published a comprehensive review of spinal dysraphism in the parturient, including a summary of reported obstetric anesthesia care. Of the 139 published cases (some only abstracts), epidural anesthesia was used in 52 cases and spinal or CSE anesthesia was used in 15 cases. There were numerous complications ranging from difficult block placement; rapid onset of anesthesia; and asymmetric, high, and failed blocks, at rates far higher than in usual obstetric patients. However, no patient had neurologic sequelae.

Asakura et al. demonstrated the use of ultrasonography to detect a bony arch defect. Ultrasonographic imaging of the terminal portion of the cord is difficult, however, and although it has been shown to be feasible in children, experience in neuraxial ultrasonographic imaging is insufficient for most anesthesia providers to determine the safety of neuraxial anesthesia in adults. A recent case series of four pregnant patients did show the ability to confirm other radiologic imaging results using ultrasonography, but the only patient who received neuraxial anesthesia had an isolated bony defect. Spinal anesthesia should be performed below the known level of the conus medullaris or avoided in favor of epidural anesthesia. If possible, epidural analgesia should also be initiated below the level of cord termination in case of unintentional dural puncture. It should be noted that the epidural space is often abnormal, which increases the likelihood of inadequate epidural anesthesia. In women with minimal function of the lower extremities and sphincters, a detailed discussion about risks of neuraxial anesthesia in the presence of a low-lying spinal cord must take place.

Two retrospective case series described the obstetric and anesthetic management in parturients with Chiari I malformation; one reported on 95 deliveries in 63 patients and one assessed 185 deliveries in 148 patients. In one series, vaginal delivery was accomplished in 43% of cases and 78% had neuraxial anesthesia. In the other series, 54% had a vaginal delivery and 66% had neuraxial anesthesia. No patient had neurologic deterioration. The decision regarding the safety of neuraxial anesthesia in any individual patient will depend on whether the patient has signs of increased intracranial pressure and the degree of cerebellar herniation. We recommend neurologic consultation prior to delivery to aid in decision-making.

A final issue for consideration is the ongoing management of latex exposure and development of latex allergy in patients with spinal dysraphism, particularly OSD. Current guidance recommends that patients with OSD should avoid all skin contact with latex protein from birth. In addition, they should be protected from inhalation of powders that contain latex particles.

RA is a systemic autoimmune inflammatory disorder primarily affecting the joints. Articular features are characterized by symmetrical painful swelling of multiple joints (classically worse in the morning), ultimately progressing to joint destruction and deformity. Disease onset is typically insidious, with disease markers often evident before the onset of symptoms. Treatment should be aggressive because erosive effects are cumulative and irreversible. Extraarticular manifestations may affect multiple organ systems and are associated with high morbidity and mortality. Demonstrating substantial geographical variation, the worldwide prevalence of RA has increased by 7.4% between 1990 and 2017, with higher rates in North America, where prevalence is 0.54% to 0.63% in the United States and 0.65% to 0.78% in Canada. RA occurs at least twice as frequently in women as in men, and while classically considered to be a disease of older adults, it is increasingly diagnosed in women of childbearing age. Numerous risk factors for RA have been identified, implicating a combination of genetic, environmental, and infectious triggers in its pathophysiology. The diagnosis is made according to internationally agreed classification criteria.

While almost any joint can be affected by RA, changes affecting the vertebral column and airway are likely to be of most interest to the obstetric anesthesia provider. While the lumbosacral spine is affected in only 5% of patients with RA, the cervical spine is commonly involved, with radiographic evidence of cervical spine deformity becoming apparent in 40% of RA patients despite optimal medical management and decreasing disease severity scores. Atlantoaxial subluxation is of particular concern; it occurs secondary to disruption of the transverse ligament and erosion of the odontoid peg, allowing for dislocation of the C1 vertebra (the “atlas”) on the C2 vertebra (the “axis”) during neck movement. There are four subtypes of atlantoaxial subluxation: (1) anterior, occurring in 80% of patients and worsened during neck flexion; (2) posterior, occurring in 5% and worsened during neck extension; (3) vertical, occurring in 10% to 20%, causing subluxation of the odontoid peg through the foramen magnum with attendant compression of the cervico-medullary junction (cranial settling); and (4) lateral, which may lead to spinal nerve or vertebral artery compression. Subaxial subluxation (occurring at or below C3) may also occur and manifests as persistent headache or Lhermitte sign, an electric shocklike sensation that occurs with neck flexion. Atlantoaxial subluxation may be identified on lateral plain film radiographs of the cervical spine, performed in the neutral, flexed, and extended positions, on which the anterior and posterior atlantodental intervals can be measured ( Fig. 46.6 ). Open-mouth radiographs can assess for odontoid erosion. Radiographs should be obtained in patients with clinical suspicion of cervical involvement ; the onset of symptoms compatible with cervical myelopathy or radiculopathy should prompt MRI and neurosurgical evaluation.

Lateral radiographic study of the cervical spine (in flexion) in a 32-y-old woman with rheumatoid arthritis (RA). There is isolated atlantoaxial subluxation (6 mm) in the absence of other radiologic changes of RA. She presented with neck pain, and a wire was placed between the occiput and the spinous process of C2 to limit the subluxation.

Airway management in RA may be challenging. Vertical subluxation of the odontoid process is associated with a complex scoliotic deformity of the trachea and larynx, which can make direct laryngoscopy and tracheal intubation difficult. Fortunately, vertical subluxations occur primarily in older patients with severe, long-standing disease and are unlikely to be seen in women of childbearing age. Nonetheless, the cricoarytenoid and temporomandibular joints (TMJ) may be involved earlier in the disease and can complicate airway management of the parturient.

Extraarticular manifestations are common in patients with RA, with severe manifestations occurring in around 40% of affected patients. Anesthesia providers have a special interest in abnormalities that affect the airway and the cardiovascular and respiratory systems ( Box 46.3 ). Although these complications occur more typically in patients with long-standing disease, it is prudent to inquire about those associated with significant anesthetic implications. Cardiovascular mortality is especially high in patients with RA, and cardiovascular disease is often present in the absence of symptoms.

Medical Management

The 2021 American College of Rheumatology guideline for the treatment of RA divides drug therapy into conventional synthetic DMARDs (csDMARDs), biologic DMARDs (bDMARDs), targeted synthetic DMARDs (tsDMARDs), and glucocorticoids. Symptom-modifying therapies include nonselective cyclooxygenase inhibitors (nonsteroidal antiinflammatory drugs [NSAIDs]). While for many years NSAIDs and glucocorticoids were the mainstay of therapy in RA during pregnancy, the proliferation of novel therapies has prompted European and North American societies to publish recommendations on the management of DMARDs from preconception through pregnancy to lactation ( Table 46.1 ). There are limited data on the use of tsDMARDs prior to and during pregnancy and lactation; these agents are likely best avoided until their safety profile is established. While the multitude and complexity of these newer agents mandate multispecialty planning and medication management, the overarching consensus favors avoiding relapses or flares in RA disease activity by continuing therapies considered safe for use in pregnancy.

Table 46.1

Safety of Disease-Modifying Antirheumatic Drugs (DMARDs) in Pregnancy

Modified from the American College of Rheumatology and European League Against Rheumatism guidelines (Götestam Skorpen C, Hoeltzenbein M, Tincani A, et al. The EULAR points to consider for use of antirheumatic drugs before pregnancy, and during pregnancy and lactation. Ann Rheum Dis . 2016;75:795–810; and Sammaritano LR, Bermas BL, Chakravarty EE, et al. 2020 American College of Rheumatology Guideline for the management of reproductive health in rheumatic and musculoskeletal diseases. Arthritis Rheumatol . 2020;72:529–556.)

Class	Preconception	Pregnancy	Lactation
csDMARDs Hydroxychloroquine, sulfasalazine, colchicine, azathioprine, 6-mercaptopurine, cyclosporine, tacrolimus	Continue	Continue	Continue
csDMARDs Methotrexate, mycophenolate mofetil, leflunomide, cyclophosphamide	Discontinue (teratogenic)	Discontinue (May consider cyclophosphamide for life-threatening disease in first/second trimesters)	Avoid
Nonselective NSAIDs (COX-2 inhibitors not preferred)	Safe	May be considered in first and second trimesters. Avoid in third trimester .	Ibuprofen preferred
Glucocorticoids Prednisone	Taper to less than 20 mg/d.	Taper to less than 20 mg/d.	Delay breastfeeding for 40 h after dose > 20 mg
bDMARDs : TNFi Certolizumab	Continue	Continue	Continue
bDMARDs : TNFi Infliximab, etanercept, adalimumab, golimumab	Continue	Continue in first and second trimesters. Discontinue prior to third trimester.	Continue
bDMARDs : TNFi Rituximab	May continue while trying to conceive. Discontinue at conception.	Consider only in severe/life-threatening disease.	Continue
bDMARDs Anakinra, belimumab, abatacept, tocilizumab, secukinumab, ustekinumab	Limited data. Discontinue and replace with known safe medication.	Limited data. Discontinue and replace with known safe medication.	No data

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