Anesthesia & Analgesia:
May 2002 - Volume 94 - Issue 5 - pp 1315-1317
doi: 10.1097/00000539-200205000-00049
CASE REPORT: Case Report
Pregnancy Complicated By Severe Osteogenesis Imperfecta: A Report Of Two Cases
Vogel, Tracey M. MD*,; Ratner, Emily F. MD*,; Thomas, Robert C. Jr., MD†, and; Chitkara, Usha MD*
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Author Information
*Department of Anesthesia, Stanford University School of Medicine, Stanford, California; and †Department of Anesthesia, Covenant HealthCare, Saginaw, Michigan
December 28, 2001.
Address correspondence and reprint requests to Tracey M. Vogel, MD, Magee-Womens Hospital, Department of Anesthesia & CCM, 300 Halket Street, Pittsburgh, Pennsylvania 15213-3180. Address e-mail to
[email protected].
We report two parturients with severe osteogenesis imperfecta (OI) complicated by profound short stature, severe kyphoscoliosis and, in one patient, respiratory compromise, presenting for cesarean delivery. We discuss two different approaches to anesthetic management for such severe presentations of the disease.
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Case Reports
Case #1
A 30-yr-old gravida 1 para 0 female with severe, type IV OI presented at 24 wk gestation for pregnancy termination via hysterotomy secondary to fetal Trisomy 18. The patient had severe kyphoscoliosis and significant deformities of all extremities secondary to multiple long bone fractures. She denied any history of bleeding diathesis. On physical examination she measured 14 in. from her buttocks to her C7 prominence. Her pelvis was severely contracted, the lower extremities were fixed in semiflexion, and she was wheelchair bound. If her lower extremities could be extended, her estimated total height would be 36 in. Although she presented at 24 wk gestation, the fundus of the uterus was at her xiphoid. She was unable to flex or extend her neck, had a Mallampati Class I airway
(1), and had poor dentition. Pulmonary function tests revealed significant restrictive disease (forced vital capacity = 18%, forced expiratory volume1/forced vital capacity = 109% predicted), and a normal arterial blood gas, but the patient denied respiratory difficulties while supine. Hematologic and coagulation studies (platelet count, prothrombin time, and partial thromboplastin time) were normal. The patient denied any history of bleeding abnormalities.
Preoperatively, 500 mL of lactated Ringer’s solution, metoclopramide 10 mg IV and sodium bicitrate 30 mL
per os were administered. The patient was positioned in a modified supine position (with her head elevated approximately 30°) on the operating room (OR) table with foam pads, towels, and pillows to avoid additional bone fractures (
Fig. 1). All standard monitors, except a blood pressure cuff, were placed. An automatic blood pressure cuff or vigorous inflation of a manual cuff could increase the risk of fracturing the humerus
(2). A right radial arterial catheter was placed to allow blood pressure monitoring and assessment of arterial blood gases. The patient was given supplemental oxygen via a nasal cannula at a rate of 3 L/min. An epidural anesthetic was planned. Before regional anesthesia, direct laryngoscopy under topical anesthesia of the oral pharynx was performed to assess ease of intubation should a regional block fail or ascend too high as to cause respiratory insufficiency. Epiglottis and posterior arytenoids were seen, and it was determined that intubation via direct laryngoscopy could be accomplished with minimal difficulty. The patient was then positioned in a modified sitting position for epidural placement. Only one intervertebral space was identified on palpation of the back. After three attempts, an epidural catheter was inserted via a Tuohy needle to a depth of 3 cm. A negative test dose with 3 mL of 2% lidocaine and 1:200,000 epinephrine was administered. An additional 10 mL, in two 5-mL aliquots, of a 2% lidocaine solution with 50 μg fentanyl and 1:200,000 epinephrine was necessary to achieve a T4 sensory level. The cesarean delivery proceeded uneventfully, without uterine atony or other complications. One intraoperative evaluation of arterial blood gases revealed a mild respiratory acidosis (pH = 7.33, Pco2 = 46.5) but adequate oxygenation (Po2 = 188). No respiratory interventions were required. Postoperative recovery was uncomplicated and she was discharged home on postoperative day 4.
Figure 1
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Case #2
A 35-yr-old parturient with type III OI, presented at 32 wk with twin gestation for elective cesarean delivery, after having been hospitalized for preterm labor for approximately 1 mo. It was felt that further delay in delivering the twins could result in maternal respiratory embarrassment resulting from continuing fetal growth and decreases in functional residual capacity. She was at this point unable to tolerate the supine position because of severe respiratory distress. A cesarean delivery was chosen as the route of delivery because of the severity of the patient’s underlying disease. Physical examination revealed marked limb deformity, severe kyphoscoliosis, and an overall estimated height of 34 in. (a 7-in. distance between her L4-5 interspace to C7 prominence). Other skeletal abnormalities included a pectus carinatum and immobility of her cervical spine. She had a Mallampati Class II airway
(1), a 4-cm thyromental distance, and extremely poor dentition. In addition to OI, her medical history was significant for mitral valve prolapse with moderate mitral valve insufficiency. The platelet count was 127; however, the patient had no history of any bleeding diathesis. No pulmonary function tests were available.
In the OR, the patient was carefully placed in the sitting position and padded with towels and pillows. A right radial arterial catheter was placed, in addition to a 5-lead electrocardiogram, pulse oximeter, and temperature probe. A general anesthetic was necessary for supine positioning of the patient and optimal surgical exposure. Direct laryngoscopy under topical anesthesia of the posterior oral pharynx revealed no laryngeal structures, and an awake fiberoptic nasal tracheal intubation with a #5.5 cuffed endotracheal tube was performed. The nares were topicalized with cocaine (4%), and transtracheal lidocaine (4%) was injected. Given the patient’s unusual anatomy, a nasal versus an oral intubation was performed for presumed ease of placement of the fiberoptic scope. General anesthesia was induced immediately after successful intubation with sodium thiopental (150 mg) and maintained with O2/N2O (50%/50%), desflurane (3%), and mivacurium (8 mg). Ventilatory support was instituted using tidal volumes of 280–300 mL at rates sufficient to maintain normocarbia. No intraoperative arterial blood gases were obtained. After delivery of the second baby, a propofol infusion (40 μg · kg−1 · min−1) was substituted for desflurane, and 75 μg of fentanyl was given IV. In addition to oxytocin (20 U/L normal saline), methylergonovine maleate was administered for control of uterine atony and postpartum hemorrhage. She was brought to the recovery room with her trachea intubated, and, after 5 min and after meeting extubation criteria (ability to follow commands, sustained head lift for 5 s) her trachea was extubated. No maternal complications occurred and the patient was discharged home 4 days after the cesarean delivery.
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Discussion
OI is a rare inherited disease of connective tissue that primarily involves ossification of the endochondral bone, resulting in very fragile bones and multiple fractures. Depending on the inheritance pattern of OI, the disease can result in a spectrum of severity. Type I is the common, mild form with minimal deformity, Type II (perinatal lethal OI) is incompatible with life, and Types III and IV are severe, more debilitating forms of the disease resulting in short stature and kyphoscoliosis
(3).
Other abnormalities associated with this disease include hyperthermia, hyperhidrosis, blue sclerae, conductive hearing loss, poor dentition, platelet dysfunction, cor pulmonale, congenital heart disease, valvular heart disease, joint laxity, and thin skin
(3–6).
OI superimposed on the preexisting physiologic changes of pregnancy presents unique anesthetic challenges. In severe forms of the disease, the bone abnormalities, metabolic disorders, and cardiac and pulmonary insufficiency can significantly increase maternal morbidity
(3). Additionally, many of these patients require cesarean delivery because of a contracted maternal pelvis, cephalopelvic disproportion, or a fetus with OI
(3).
Although there have been several case reports describing the successful use of regional anesthesia
(7–9) and general anesthesia
(4,10) for cesarean delivery in patients with mild to moderate OI, the two cases presented here illustrate two anesthetic techniques that were successfully used in two patients with severe forms of the disease.
A regional technique can be safely and effectively used as illustrated by the first case. By using lumbar epidural anesthesia, we were able to avoid tracheal intubation and its inherent risk of aspiration and mandibular injury. The increased metabolic rate and hyperthermia associated with OI and general anesthesia were also avoided
(11,12). In the event that regional anesthesia failed, or that the block extended unexpectedly high, several precautionary measures were taken: 1) topical anesthesia of the oral pharynx and a brief direct laryngoscopy were performed before initiating the block to assess the presumed ease of intubation, 2) the fiberoptic bronchoscope was readily available, and 3) the surgeons were prepared to continue the cesarean delivery, if necessary, using a local anesthetic technique.
Technical difficulty with block placement, inability to tolerate the supine position awake, and a preexisting coagulopathy from platelet abnormalities may preclude regional anesthesia in patients with OI. Further, assessment of platelet dysfunction is difficult. A targeted history and physical examination remain the best determinants for the individual patients
(13). Kyphoscoliosis can predispose these patients to inadvertent dural puncture
(3) and, coupled with short stature, may make it difficult to predict the level of any block produced by a given dose of local anesthetic. We believe that any local anesthetic should be administered incrementally to reduce the likelihood of respiratory insufficiency developing from an unintended high block. Continuous spinal anesthesia performed either with a standard epidural needle intentionally placed in the subarachnoid space and then using a standard epidural catheter, or a spinal needle and a microcatheter may be other options for this patient cohort
(14). The risk of postdural puncture headache must be considered if this technique is used.
In the second case, the patient had significant pulmonary compromise. Awake fiberoptic intubation followed by a general anesthetic was chosen because her airway appeared difficult to intubate with direct laryngoscopy, and she could not tolerate lying supine secondary to respiratory insufficiency. Although there is a risk of epistaxis with nasal intubation, the nasal versus oral route was chosen based on presumed ease of placement. Small doses of opioids and short-acting anesthetics such as propofol and desflurane may be preferable to other longer-acting drugs for anesthetic maintenance, as they may decrease the incidence of postoperative respiratory depression resulting from prolonged drug effect. Volatile anesthetics, however, must be used cautiously as they can contribute to uterine atony. These patients may also have abnormal uterine connective tissue, which may already predispose them to excessive uterine bleeding
(15).
These two cases illustrate two very severe examples of pregnant women with OI and review the use of two different yet successful anesthetic techniques for cesarean delivery. It is important to understand and consider the advantages and complications associated with both continuous regional and general anesthesia in this patient population. The authors feel that it is also helpful to use a multidisciplinary approach in planning a technique not only appropriate for the level of expertise of the anesthesiologist and obstetrician but also suited to the severity of physiologic and anatomic abnormalities of the individual patient.
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