E CLINICAL CARE
Anesthetic Implications of an Obstetric Patient with Blue Rubber Bleb Nevus Syndrome Jessica Galey, MD,* Shobana Bharadwaj, MBBS,* Sarah Crimmins, DO,† Caron M. Hong, MD, MSc,* and Andrew M. Malinow, MD*† Blue rubber bleb nevus syndrome, a syndrome of multifocal venous malformations, has been reported rarely during pregnancy. This syndrome has been associated with airway lesions in some patients and neuraxial abnormalities in other patients. We report the anesthetic and obstetric management of a patient with an extensive distribution of both airway and neuraxial lesions. (A&A Case Reports. 2016;6:146–9.)
B
lue rubber bleb nevus syndrome (BRBNS) is a rare syndrome of multifocal venous malformations usually found in cutaneous, subcutaneous, and/or different visceral tissues. We believe this to be the first report of the existence of multiple neuraxial (including lumbosacral) and airway lesions together in an otherwise-asymptomatic parturient. Recommendations from multiple medical specialists modified the patient’s delivery plan. As we outline the many management decisions in medical management of this patient with a rare syndrome, we discuss the risk/benefit analysis that led ultimately to a scheduled cesarean delivery. The patient provided written consent for permission to use information and photographs relating to her care.
CASE DESCRIPTION
A 21-year-old woman, after 4 pregnancies, with 3 terminated early, presents pregnant at 30 weeks gestation for initial consultation with our obstetric anesthesiology service. Diagnosed with BRBNS as a neonate, she demonstrated only cutaneous lesions. She reported a history of episodic gastrointestinal bleeding with associated iron-deficiency anemia. In the past, she required iron supplementation and occasional transfusions; her current hemoglobin was 8.4 g/dL. Findings from recent magnetic resonance imaging (MRI) did not reveal lesions in the abdominal wall/viscera or cervix. On our examination, a previously unreported lesion was found on the right extreme posterior aspect of the tongue (Fig. 1). We requested another MRI, which revealed multiple, large vascular lesions in the epidural space (at levels L3–L4, T7–T9, and C2–C4; Fig. 2) and multiple paraspinal lesions (at levels C1–C2, T2, T6, and T12), some of which extended to the neural foramina. We requested a consultation with an otorhinolaryngologist. Subsequent fiberoptic examination of the airway revealed that the tongue lesion did not extend into the posterior oropharynx, From the Departments of *Anesthesiology and †Obstetrics/Gynecology and Reproductive Sciences, University of Maryland School of Medicine, Baltimore, Maryland. Accepted for publication September 3, 2015. Funding: Departmental. The authors declare no conflicts of interest. This report was previously presented, in part, at the Society of Obstetric Anesthesia and Perinatology, Toronot, Canada. Address correspondence to Jessica Galey, MD, University of Maryland School of Medicine, 22 S. Greene St., S11C, Baltimore, MD 21201. Address e-mail to [email protected]. Copyright © 2015 International Anesthesia Research Society DOI: 10.1213/XAA.0000000000000265
146 cases-anesthesia-analgesia.org
hypopharynx, or larynx. Another previously unreported lesion was identified in the vestibule of the left nares. The otorhinolaryngologist was concerned about iatrogenic hemorrhage with instrumentation of the airway that could occur at any time during the conduct of general anesthesia. The obstetricians requested a consult with a neurosurgeon, who concurred with the plan not to site a neuraxial catheter. A subsequently requested MRI of the brain did not reveal any intracranial lesions. As her obstetric anesthesiologists, we proffered that the low thoracic and mid-lumbar epidural and paraspinal lesions were relative contraindications to lumbar neuraxial anesthesia. It was decided that neuraxial anesthesia would not be offered to the patient in labor or for cesarean delivery. Because she did not have perineal lesions precluding vaginal delivery, there were no contraindications to labor without neuraxial analgesia (e.g., with IV analgesia or paracervical and/or pudendal block). We could not guarantee that airway management (i.e., tracheal intubation/extubation or even potential emergency placement of a supraglottic airway device) would not lead to hemorrhage. After consulting with the patient, the obstetric team chose to schedule an elective primary abdominal delivery at 39 weeks’ gestation. During the interim, the patient was diagnosed with gestational hypertension; her arterial blood pressures measured as high as 149/87 mm Hg but without proteinuria. The patient was rescheduled for abdominal delivery at 37 weeks gestational age. On the day of delivery, maternal blood pressure was measured at 141/87 mm Hg but now demonstrated a urine protein:creatinine ratio compatible with preeclampsia. The obstetricians elected to initiate IV magnesium sulfate after delivery. After routine gastric chemoprophylaxis, the patient was positioned on the operating room table with left uterine displacement. The abdomen was cleaned, prepared, and draped. General anesthesia was induced in rapid sequence with the injection of IV alfentanil (10 μg/kg) to supplement IV injection of propofol (2.5 mg/kg) and succinylcholine (1.5 mg/kg). A video laryngoscope blade (GlideScope, Verathon Inc., Bothell, WA) was placed on the midline of the tongue. The lingual lesion was noted intact before, during, and after tracheal intubation. The tracheal tube was taped to the left. General anesthesia was maintained with inhaled isoflurane, nitrous oxide, and oxygen. Paralysis was maintained using a variable-rate IV succinylcholine infusion. No lesions were encountered on the lower uterine segment. Five minutes after skin incision, the neonate was delivered with Apgar scores of 6 and 8 at 1 and 5 minutes, respectively. March 15, 2016 • Volume 6 • Number 6
Copyright © 2015 International Anesthesia Research Society. Unauthorized reproduction of this article is prohibited.
Figure 1. Right top posterior tongue lesion (arrow).
Figure 2. Magnetic resonance image of epidural/paravertebral lesion at the L3 to L4 spinal level (arrow).
Cord blood gas analysis revealed an umbilical artery pH = 7.31, Pco2 = 55, Po2 = 25; umbilical vein pH = 7.39, Pco2 = 42, Po2 = 44. General anesthesia was deepened, increasing the concentration of nitrous oxide and injecting IV doses of morphine and diazepam. Skin incision was closed 28 minutes after delivery. With the patient inhaling 100% oxygen, awake, and demonstrating recovery from paralysis, her trachea was extubated without disruption of the tongue lesion. Immediately, the obstetricians initiated and maintained routine IV magnesium sulfate therapy for the next 24 hours. The patient demonstrated no further stigmata of preeclampsia. The neonate immediately required “blow-by” oxygen but was transitioned that day to the fullterm nursery. No lesions were visible on the neonate. After routine recovery from surgery, both mother and neonate were discharged on the third postoperative day.
DISCUSSION
Although first described in 1860 by Gascoyen,1 Bean2 fully characterized BRBNS in 1958. Reported in 1:14,000 births, this syndrome is described typically as a sporadic genetic
March 15, 2016 • Volume 6 • Number 6
mutation, although there are case reports of autosomaldominant transmission.3–6 Associated cutaneous lesions vary in size, shape, and color. Lesions are thin walled, and after compression, an empty sac slowly refills. Occasionally, cutaneous lesions cause pain. Lesions also occur in the gastrointestinal tract and other abdominal/pelvic viscera. Irondeficiency anemia may occur.7 In the natural evolution of BRBNS, lesions can increase in size, whereas new lesions develop over time. There are several cases of BRBNS that complicated the patient’s pregnancy; lesions on the uterus, cervix, and vagina that impacted mode of delivery, intracranial lesions, and other lesions along the neuraxis or in the oropharynx that impacted the provision of analgesia/anesthesia during labor and delivery have been reported.3,8–13 Because pregnancy can increase the size and number of venous malformations seen with other syndromes,3,12 it could be postulated that pregnancy similarly affects the venous malformations seen in BRBNS. Given the high risk of bleeding, lesions at or near the birth canal are considered a contraindication to vaginal delivery. Lesions on the uterus require antepartum identification, given the potential for bleeding during cesarean delivery. Our patient had no visual or MRI evidence of lesions on abdominal/pelvic organs or the perineum allowing for labor and vaginal or abdominal delivery per obstetric indications. Lesions also have been reported to occur in the neuraxis, specifically in the epidural space.3 We informed the obstetricians that the possibility of neuraxial labor analgesia would be dependent on the findings on MRI. Nirmal et al.12 did report the induction of spinal anesthesia for scheduled primary abdominal delivery in a patient whose screening MRI, done “as near to the to the operation date as possible,” did not reveal any neuraxial lesions. When weighing the risks of inducing regional anesthesia, these authors cautioned for the presence of “any lesion in the central nervous system” and mentioned possible growth of the lesions during pregnancy between MRI and the date of delivery.12 Indeed, an MRI at approximately 34 weeks’ gestation revealed that our asymptomatic patient had multiple lesions in the cervical, thoracic, and lumbar epidural and paravertebral spaces. It could be argued that an epidural catheter or spinal needle for single injection could be safely sited in the neuraxis rostral to the L3 to L4 epidural lesion but caudal to the T12 paraspinal lesion thought contiguous to the neural foramen. Indeed, techniques to reduce the incidence of epidural catheter vein insertion have been proffered in pregnant patients not thought to have epidural venous malformations.14 The possibility of epidural hematoma after a rupture of a lesion in BRBNS in a pregnant woman with an epidural catheter has never been reported. Yet, there is still the possibility of laceration leading to hematoma requiring emergent spinal decompression. In addition, the lumbar epidural lesion already compressed the thecal sac (Fig. 2). We were uncertain of any further compression effect of an epidural or a spinal injection of local anesthetic in the volume needed to provide anesthesia for operative vaginal or abdominal delivery.15 Admittedly conservative in our decision, we eschewed the use of neuraxial anesthesia. We counseled the patient and informed the obstetricians that we would not perform neuraxial
cases-anesthesia-analgesia.org
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Copyright © 2015 International Anesthesia Research Society. Unauthorized reproduction of this article is prohibited.
analgesia/anesthesia. The neurosurgeon endorsed the plan to avoid neuraxial anesthesia. If a trial of labor was an option for delivery, then IV sedation with paracervical and/ or pudendal nerve block could have been offered. Our institution does not offer nitrous oxide–inhaled analgesia. Although the patient did not have an obstetric contraindication to labor and vaginal delivery, it is commonly suggested that many physiologic changes during the hyperdynamic cardiovascular state seen in late pregnancy, labor, and delivery (e.g., repetitive uterine contractions, complete or partial caval compression, Valsalva) could increase the volume of blood in the veins of Batson.15–17 The neurosurgeon could not predict what the effects of (even pain free) labor would be on the incidence of rupture of an epidural venous malformation seen in BRBNS. The obstetricians elected a primary abdominal delivery for maternal indications. In our planning of airway management, we were concerned with avoiding a laceration of the large right-sided tongue lesion. The need for emergency induction of general anesthesia accompanying any immediate cesarean delivery might increase the chance that we lacerate the tongue lesion, perhaps leading to catastrophic bleeding in the airway. Given this warning, the obstetricians then scheduled the elective primary abdominal delivery. A laryngoscope blade routinely is placed in the right side of the mouth, sweeping the tongue to the left. Insertion and removal of the tracheal tube, or movement during the anesthetic even while taped at the lips, could also potentially cause a bleed in the airway. In addition, there has been a report of an afflicted parturient whose laryngoscopy was made difficult because of the restricted mouth opening thought secondary to an oropharyngeal nevus.9 Instead of a typical metal laryngoscopy blade (e.g., MAC3 or Miller 2), we chose a GlideScope, routinely inserted along the midline of the tongue without need for suspension to visualize the glottis. In an attempt to keep the tracheal tube from irritating the right-sided tongue lesion during the remainder of general anesthesia, we secured the tracheal tube away from the lingual lesion. We also attempted to avoid the right side of the tongue when we later removed the tracheal tube. Although her body mass index was 35 kg/m2, preanesthetic examination of airway anatomy was reassuring that we would be able to visualize her glottic aperture with a GlideScope. However, if needed in a “can’t intubate–can’t ventilate” scenario, we were concerned that insertion of a supraglottic airway might also cause bleeding. We did request that the otorhinolaryngologists be present at the time of induction of general anesthesia to emergently assist either with a surgical airway or if insertion of a needed laryngeal mask caused bleeding in the airway. Although their equipment is mobile, the otorhinolaryngologists asked that any general anesthetic be done near their preferred operating rooms where cauterization could be better accomplished. It could be argued that delivery outside the delivery suite is less than optimal for the physicians, nurses, and the patient’s family. Because of the everincreasing need for complex obstetric surgery (e.g., placenta percreta, uterine vascular abnormalities) in gravidae
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transferred to our institution, the entire perinatal team has established an often-used protocol for moving designated scheduled cases from our smaller (approximately 180 sq ft) operating rooms in the labor and delivery suite to the more standard-sized (approximately 500 sq ft) general operating rooms large enough to house supplemental personnel and equipment. The procedure was scheduled into a general surgical operating room that was both convenient for the otorhinolaryngologists but familiar to the anesthesiologists, obstetricians, neonatologists, and nurses. Even with a recent diagnosis of gestational hypertension, the patient had not exhibited other stigmata of preeclampsia. In the morning of her delivery, the obstetricians elected to send blood and urine for laboratory analysis. A diagnosis of preeclampsia was made immediately before scheduled surgery. The obstetricians elected not to begin magnesium sulfate for seizure prophylaxis until after surgery. To blunt the hemodynamic response to laryngoscopy, alfentanil was injected IV as a part of the rapid sequence induction of general anesthesia. Alfentanil routinely is used by obstetric anesthesiologists in our institution to blunt the pressor response of laryngoscopy.18 IV injection of other opioids (e.g., remifentanil, fentanyl), antihypertensives (e.g., labetalol, hydralazine, esmolol), or even lidocaine also could have been used. Even with several previous case reports of BRBNS in pregnancy, we were surprised by the number and extent of newly discovered lesions, especially in the epidural space of this asymptomatic patient. Eventually the patient underwent MRI on 3 occasions; a sequence not well planned. Although the insurance company readily consented to reimburse an abdomen/pelvis study, given the patient’s history of gastrointestinal bleeding, they delayed approval for the neuraxial MRI for almost 2 weeks. The insurance company readily agreed to a subsequent MRI of the brain. We strongly advise that any pregnant patient with BRBNS undergo a well-coordinated antepartum consultation, including MRI of the neuraxis, brain, and abdominal/pelvic organs and examination of the entire airway. Recently, the patient returned pregnant to our institution, again late to first antenatal care. Diagnosed with gestational hypertension but now with a previous uterine scar, she was offered and received an elective repeat cesarean delivery and bilateral partial salpingectomy. The insurance company approved a request for an abdominal/pelvic MRI (still negative for lesions) but refused requests for follow-up neuraxial and brain MRI. General anesthesia was induced and maintained for delivery, in a manner consistent with the reported case. Although there is as high as a 1:2 chance of genetic transmission, neither the neonate nor his recently born sibling demonstrated any visible lesions. The neonatologists elected not to perform an MRI of either neonate. The patient was counseled about each child’s risk for affliction. A coordinated multidisciplinary approach involving obstetric anesthesiologists, maternal–fetal medicine specialists, otorhinolaryngologists, neurosurgeons, and neonatologists is needed to efficiently provide antepartum and intrapartum evaluation and care. Possible difficulty or challenges in the management of analgesia/anesthesia
A & A case reports
Copyright © 2015 International Anesthesia Research Society. Unauthorized reproduction of this article is prohibited.
indicates early anesthetic consultation for any obstetric patient with BRBNS. E REFERENCES 1 Gascoyen M. Case of naevus involving the parotid gland and causing death from suffocation: naevi of the viscera. Trans Pathol Soc Lond 1860;11:267 2 Bean W. Blue rubber-bleb nevi of the skin and gastrointestinal tract. In: Thomas CC, ed. Vascular Spiders and Related Lesions of the Skin. Springfield, IL: Charles C. Thomas, 1958:178–85 3. Ochiai D, Miyakoshi K, Yakubo K, Fukuiya T, Yoshimura Y. Familial blue rubber bleb nevus syndrome in pregnancy with spinal epidural involvement. Case Rep Obstet Gynecol 2013;2013:141506 4. Kisu T, Yamaoka K, Uchida Y, Mori H, Nakama T, Hisatsugu T, Miyaji H, Motooka M. A case of blue rubber bleb nevus syndrome with familial onset. Gastroenterol Jpn 1986;21:262–6 5. Munkvad M. Blue rubber bleb nevus syndrome. Dermatologica 1983;167:307–9 6 Martinez CA, Rodrigues MR, Sato DT, Silveira Junior PP, Gama RF, Mattavelli CB, Pereira JA. Blue rubber bleb nevus syndrome as a cause of lower digestive bleeding. Case Rep Surg 2014;2014:683684 7 Maisnam I, Das T, Kundu AK, Ghosh A. Blue rubber bleb nevus syndrome causing refractory anemia. J Assoc Physicians India 2020;58:246–9 8. Terata M, Kikuchi A, Kanasugi T, Fukushima A, Sugiyama T. Association of blue rubber bleb nevus syndrome and placenta previa: report of a case. J Clin Ultrasound 2013;41:517–20 9. Adeniji AA, Fairlie FM, Jones TH, Wilkinson P. Pregnancy and blue rubber bleb naevus syndrome. Br J Obstet Gynaecol 1999;106:1316–8
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10. Wada O, Unno N, Matsuoka R, Taketani Y. A case report: pregnancy complicated by blue rubber-bleb nevus syndrome. J Obstet Gynaecol Res 1999;25:261–3 11. Tanaka N, Tsuda M, Samura O, Miyoshi H, Hara T, Kudo Y. Blue rubber bleb nevus syndrome: report of a patient with hemangiomas of the vaginal portion of the cervix appearing during pregnancy. J Obstet Gynaecol Res 2007;33:546–8 12. Nirmal DM, Rhodes S, Hassanaien M. Pregnancy in blue rubber bleb syndrome: a case report. Am J Obstet Gynecol 2008;199:e14–5 13. Bouchghoul H, Nizard J. Pregnancy and blue rubber bleb nevus syndrome. Eur J Obstet Gynecol Reprod Biol 2013;169:415–6 14. Mhyre JM, Greenfield ML, Tsen LC, Polley LS. A systematic review of randomized controlled trials that evaluate strategies to avoid epidural vein cannulation during obstetric epidural catheter placement. Anesth Analg 2009;108:1232–42 15 Reynolds F. Neurologic complications of pregnancy and neuraxial anesthesia. In: Chestnut DH, Wong CA, Tsen LC, Kee WDN, Beilin Y, Mhyre J, eds. Chestnut’s Obstetric Anesthesia: Principles and Practice. 5th ed. Philadelphia, PA: Saunders, 2014:749 16 Jea A, Moza K, Levi AD, Vanni S. Spontaneous spinal epidural hematoma during pregnancy; case report and literature review. Neurosurgery 2005;56:1156–60 17 Luyendijk W. Anatomy of the lumbar and sacral spinal canal. In: van Zundert A, Ostheimer GW, eds. Pain Relief and Anesthesia in Obstetrics. New York: Churchill Livingston, 1996:172–3 18. Gin T, Ngan-Kee WD, Siu YK, Stuart JC, Tan PE, Lam KK. Alfentanil given immediately before the induction of anesthesia for elective cesarean delivery. Anesth Analg 2000;90:1167–72
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Anesthetic Implications of an Obstetric Patient with Blue Rubber Bleb Nevus Syndrome Jessica Galey, MD,* Shobana Bharadwaj, MBBS,* Sarah Crimmins, DO,† Caron M. Hong, MD, MSc,* and Andrew M. Malinow, MD*† Blue rubber bleb nevus syndrome, a syndrome of multifocal venous malformations, has been reported rarely during pregnancy. This syndrome has been associated with airway lesions in some patients and neuraxial abnormalities in other patients. We report the anesthetic and obstetric management of a patient with an extensive distribution of both airway and neuraxial lesions. (A&A Case Reports. 2016;6:146–9.)
B
lue rubber bleb nevus syndrome (BRBNS) is a rare syndrome of multifocal venous malformations usually found in cutaneous, subcutaneous, and/or different visceral tissues. We believe this to be the first report of the existence of multiple neuraxial (including lumbosacral) and airway lesions together in an otherwise-asymptomatic parturient. Recommendations from multiple medical specialists modified the patient’s delivery plan. As we outline the many management decisions in medical management of this patient with a rare syndrome, we discuss the risk/benefit analysis that led ultimately to a scheduled cesarean delivery. The patient provided written consent for permission to use information and photographs relating to her care.
CASE DESCRIPTION
A 21-year-old woman, after 4 pregnancies, with 3 terminated early, presents pregnant at 30 weeks gestation for initial consultation with our obstetric anesthesiology service. Diagnosed with BRBNS as a neonate, she demonstrated only cutaneous lesions. She reported a history of episodic gastrointestinal bleeding with associated iron-deficiency anemia. In the past, she required iron supplementation and occasional transfusions; her current hemoglobin was 8.4 g/dL. Findings from recent magnetic resonance imaging (MRI) did not reveal lesions in the abdominal wall/viscera or cervix. On our examination, a previously unreported lesion was found on the right extreme posterior aspect of the tongue (Fig. 1). We requested another MRI, which revealed multiple, large vascular lesions in the epidural space (at levels L3–L4, T7–T9, and C2–C4; Fig. 2) and multiple paraspinal lesions (at levels C1–C2, T2, T6, and T12), some of which extended to the neural foramina. We requested a consultation with an otorhinolaryngologist. Subsequent fiberoptic examination of the airway revealed that the tongue lesion did not extend into the posterior oropharynx, From the Departments of *Anesthesiology and †Obstetrics/Gynecology and Reproductive Sciences, University of Maryland School of Medicine, Baltimore, Maryland. Accepted for publication September 3, 2015. Funding: Departmental. The authors declare no conflicts of interest. This report was previously presented, in part, at the Society of Obstetric Anesthesia and Perinatology, Toronot, Canada. Address correspondence to Jessica Galey, MD, University of Maryland School of Medicine, 22 S. Greene St., S11C, Baltimore, MD 21201. Address e-mail to [email protected]. Copyright © 2015 International Anesthesia Research Society DOI: 10.1213/XAA.0000000000000265
146 cases-anesthesia-analgesia.org
hypopharynx, or larynx. Another previously unreported lesion was identified in the vestibule of the left nares. The otorhinolaryngologist was concerned about iatrogenic hemorrhage with instrumentation of the airway that could occur at any time during the conduct of general anesthesia. The obstetricians requested a consult with a neurosurgeon, who concurred with the plan not to site a neuraxial catheter. A subsequently requested MRI of the brain did not reveal any intracranial lesions. As her obstetric anesthesiologists, we proffered that the low thoracic and mid-lumbar epidural and paraspinal lesions were relative contraindications to lumbar neuraxial anesthesia. It was decided that neuraxial anesthesia would not be offered to the patient in labor or for cesarean delivery. Because she did not have perineal lesions precluding vaginal delivery, there were no contraindications to labor without neuraxial analgesia (e.g., with IV analgesia or paracervical and/or pudendal block). We could not guarantee that airway management (i.e., tracheal intubation/extubation or even potential emergency placement of a supraglottic airway device) would not lead to hemorrhage. After consulting with the patient, the obstetric team chose to schedule an elective primary abdominal delivery at 39 weeks’ gestation. During the interim, the patient was diagnosed with gestational hypertension; her arterial blood pressures measured as high as 149/87 mm Hg but without proteinuria. The patient was rescheduled for abdominal delivery at 37 weeks gestational age. On the day of delivery, maternal blood pressure was measured at 141/87 mm Hg but now demonstrated a urine protein:creatinine ratio compatible with preeclampsia. The obstetricians elected to initiate IV magnesium sulfate after delivery. After routine gastric chemoprophylaxis, the patient was positioned on the operating room table with left uterine displacement. The abdomen was cleaned, prepared, and draped. General anesthesia was induced in rapid sequence with the injection of IV alfentanil (10 μg/kg) to supplement IV injection of propofol (2.5 mg/kg) and succinylcholine (1.5 mg/kg). A video laryngoscope blade (GlideScope, Verathon Inc., Bothell, WA) was placed on the midline of the tongue. The lingual lesion was noted intact before, during, and after tracheal intubation. The tracheal tube was taped to the left. General anesthesia was maintained with inhaled isoflurane, nitrous oxide, and oxygen. Paralysis was maintained using a variable-rate IV succinylcholine infusion. No lesions were encountered on the lower uterine segment. Five minutes after skin incision, the neonate was delivered with Apgar scores of 6 and 8 at 1 and 5 minutes, respectively. March 15, 2016 • Volume 6 • Number 6
Copyright © 2015 International Anesthesia Research Society. Unauthorized reproduction of this article is prohibited.
Figure 1. Right top posterior tongue lesion (arrow).
Figure 2. Magnetic resonance image of epidural/paravertebral lesion at the L3 to L4 spinal level (arrow).
Cord blood gas analysis revealed an umbilical artery pH = 7.31, Pco2 = 55, Po2 = 25; umbilical vein pH = 7.39, Pco2 = 42, Po2 = 44. General anesthesia was deepened, increasing the concentration of nitrous oxide and injecting IV doses of morphine and diazepam. Skin incision was closed 28 minutes after delivery. With the patient inhaling 100% oxygen, awake, and demonstrating recovery from paralysis, her trachea was extubated without disruption of the tongue lesion. Immediately, the obstetricians initiated and maintained routine IV magnesium sulfate therapy for the next 24 hours. The patient demonstrated no further stigmata of preeclampsia. The neonate immediately required “blow-by” oxygen but was transitioned that day to the fullterm nursery. No lesions were visible on the neonate. After routine recovery from surgery, both mother and neonate were discharged on the third postoperative day.
DISCUSSION
Although first described in 1860 by Gascoyen,1 Bean2 fully characterized BRBNS in 1958. Reported in 1:14,000 births, this syndrome is described typically as a sporadic genetic
March 15, 2016 • Volume 6 • Number 6
mutation, although there are case reports of autosomaldominant transmission.3–6 Associated cutaneous lesions vary in size, shape, and color. Lesions are thin walled, and after compression, an empty sac slowly refills. Occasionally, cutaneous lesions cause pain. Lesions also occur in the gastrointestinal tract and other abdominal/pelvic viscera. Irondeficiency anemia may occur.7 In the natural evolution of BRBNS, lesions can increase in size, whereas new lesions develop over time. There are several cases of BRBNS that complicated the patient’s pregnancy; lesions on the uterus, cervix, and vagina that impacted mode of delivery, intracranial lesions, and other lesions along the neuraxis or in the oropharynx that impacted the provision of analgesia/anesthesia during labor and delivery have been reported.3,8–13 Because pregnancy can increase the size and number of venous malformations seen with other syndromes,3,12 it could be postulated that pregnancy similarly affects the venous malformations seen in BRBNS. Given the high risk of bleeding, lesions at or near the birth canal are considered a contraindication to vaginal delivery. Lesions on the uterus require antepartum identification, given the potential for bleeding during cesarean delivery. Our patient had no visual or MRI evidence of lesions on abdominal/pelvic organs or the perineum allowing for labor and vaginal or abdominal delivery per obstetric indications. Lesions also have been reported to occur in the neuraxis, specifically in the epidural space.3 We informed the obstetricians that the possibility of neuraxial labor analgesia would be dependent on the findings on MRI. Nirmal et al.12 did report the induction of spinal anesthesia for scheduled primary abdominal delivery in a patient whose screening MRI, done “as near to the to the operation date as possible,” did not reveal any neuraxial lesions. When weighing the risks of inducing regional anesthesia, these authors cautioned for the presence of “any lesion in the central nervous system” and mentioned possible growth of the lesions during pregnancy between MRI and the date of delivery.12 Indeed, an MRI at approximately 34 weeks’ gestation revealed that our asymptomatic patient had multiple lesions in the cervical, thoracic, and lumbar epidural and paravertebral spaces. It could be argued that an epidural catheter or spinal needle for single injection could be safely sited in the neuraxis rostral to the L3 to L4 epidural lesion but caudal to the T12 paraspinal lesion thought contiguous to the neural foramen. Indeed, techniques to reduce the incidence of epidural catheter vein insertion have been proffered in pregnant patients not thought to have epidural venous malformations.14 The possibility of epidural hematoma after a rupture of a lesion in BRBNS in a pregnant woman with an epidural catheter has never been reported. Yet, there is still the possibility of laceration leading to hematoma requiring emergent spinal decompression. In addition, the lumbar epidural lesion already compressed the thecal sac (Fig. 2). We were uncertain of any further compression effect of an epidural or a spinal injection of local anesthetic in the volume needed to provide anesthesia for operative vaginal or abdominal delivery.15 Admittedly conservative in our decision, we eschewed the use of neuraxial anesthesia. We counseled the patient and informed the obstetricians that we would not perform neuraxial
cases-anesthesia-analgesia.org
147
Copyright © 2015 International Anesthesia Research Society. Unauthorized reproduction of this article is prohibited.
analgesia/anesthesia. The neurosurgeon endorsed the plan to avoid neuraxial anesthesia. If a trial of labor was an option for delivery, then IV sedation with paracervical and/ or pudendal nerve block could have been offered. Our institution does not offer nitrous oxide–inhaled analgesia. Although the patient did not have an obstetric contraindication to labor and vaginal delivery, it is commonly suggested that many physiologic changes during the hyperdynamic cardiovascular state seen in late pregnancy, labor, and delivery (e.g., repetitive uterine contractions, complete or partial caval compression, Valsalva) could increase the volume of blood in the veins of Batson.15–17 The neurosurgeon could not predict what the effects of (even pain free) labor would be on the incidence of rupture of an epidural venous malformation seen in BRBNS. The obstetricians elected a primary abdominal delivery for maternal indications. In our planning of airway management, we were concerned with avoiding a laceration of the large right-sided tongue lesion. The need for emergency induction of general anesthesia accompanying any immediate cesarean delivery might increase the chance that we lacerate the tongue lesion, perhaps leading to catastrophic bleeding in the airway. Given this warning, the obstetricians then scheduled the elective primary abdominal delivery. A laryngoscope blade routinely is placed in the right side of the mouth, sweeping the tongue to the left. Insertion and removal of the tracheal tube, or movement during the anesthetic even while taped at the lips, could also potentially cause a bleed in the airway. In addition, there has been a report of an afflicted parturient whose laryngoscopy was made difficult because of the restricted mouth opening thought secondary to an oropharyngeal nevus.9 Instead of a typical metal laryngoscopy blade (e.g., MAC3 or Miller 2), we chose a GlideScope, routinely inserted along the midline of the tongue without need for suspension to visualize the glottis. In an attempt to keep the tracheal tube from irritating the right-sided tongue lesion during the remainder of general anesthesia, we secured the tracheal tube away from the lingual lesion. We also attempted to avoid the right side of the tongue when we later removed the tracheal tube. Although her body mass index was 35 kg/m2, preanesthetic examination of airway anatomy was reassuring that we would be able to visualize her glottic aperture with a GlideScope. However, if needed in a “can’t intubate–can’t ventilate” scenario, we were concerned that insertion of a supraglottic airway might also cause bleeding. We did request that the otorhinolaryngologists be present at the time of induction of general anesthesia to emergently assist either with a surgical airway or if insertion of a needed laryngeal mask caused bleeding in the airway. Although their equipment is mobile, the otorhinolaryngologists asked that any general anesthetic be done near their preferred operating rooms where cauterization could be better accomplished. It could be argued that delivery outside the delivery suite is less than optimal for the physicians, nurses, and the patient’s family. Because of the everincreasing need for complex obstetric surgery (e.g., placenta percreta, uterine vascular abnormalities) in gravidae
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transferred to our institution, the entire perinatal team has established an often-used protocol for moving designated scheduled cases from our smaller (approximately 180 sq ft) operating rooms in the labor and delivery suite to the more standard-sized (approximately 500 sq ft) general operating rooms large enough to house supplemental personnel and equipment. The procedure was scheduled into a general surgical operating room that was both convenient for the otorhinolaryngologists but familiar to the anesthesiologists, obstetricians, neonatologists, and nurses. Even with a recent diagnosis of gestational hypertension, the patient had not exhibited other stigmata of preeclampsia. In the morning of her delivery, the obstetricians elected to send blood and urine for laboratory analysis. A diagnosis of preeclampsia was made immediately before scheduled surgery. The obstetricians elected not to begin magnesium sulfate for seizure prophylaxis until after surgery. To blunt the hemodynamic response to laryngoscopy, alfentanil was injected IV as a part of the rapid sequence induction of general anesthesia. Alfentanil routinely is used by obstetric anesthesiologists in our institution to blunt the pressor response of laryngoscopy.18 IV injection of other opioids (e.g., remifentanil, fentanyl), antihypertensives (e.g., labetalol, hydralazine, esmolol), or even lidocaine also could have been used. Even with several previous case reports of BRBNS in pregnancy, we were surprised by the number and extent of newly discovered lesions, especially in the epidural space of this asymptomatic patient. Eventually the patient underwent MRI on 3 occasions; a sequence not well planned. Although the insurance company readily consented to reimburse an abdomen/pelvis study, given the patient’s history of gastrointestinal bleeding, they delayed approval for the neuraxial MRI for almost 2 weeks. The insurance company readily agreed to a subsequent MRI of the brain. We strongly advise that any pregnant patient with BRBNS undergo a well-coordinated antepartum consultation, including MRI of the neuraxis, brain, and abdominal/pelvic organs and examination of the entire airway. Recently, the patient returned pregnant to our institution, again late to first antenatal care. Diagnosed with gestational hypertension but now with a previous uterine scar, she was offered and received an elective repeat cesarean delivery and bilateral partial salpingectomy. The insurance company approved a request for an abdominal/pelvic MRI (still negative for lesions) but refused requests for follow-up neuraxial and brain MRI. General anesthesia was induced and maintained for delivery, in a manner consistent with the reported case. Although there is as high as a 1:2 chance of genetic transmission, neither the neonate nor his recently born sibling demonstrated any visible lesions. The neonatologists elected not to perform an MRI of either neonate. The patient was counseled about each child’s risk for affliction. A coordinated multidisciplinary approach involving obstetric anesthesiologists, maternal–fetal medicine specialists, otorhinolaryngologists, neurosurgeons, and neonatologists is needed to efficiently provide antepartum and intrapartum evaluation and care. Possible difficulty or challenges in the management of analgesia/anesthesia
A & A case reports
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indicates early anesthetic consultation for any obstetric patient with BRBNS. E REFERENCES 1 Gascoyen M. Case of naevus involving the parotid gland and causing death from suffocation: naevi of the viscera. Trans Pathol Soc Lond 1860;11:267 2 Bean W. Blue rubber-bleb nevi of the skin and gastrointestinal tract. In: Thomas CC, ed. Vascular Spiders and Related Lesions of the Skin. Springfield, IL: Charles C. Thomas, 1958:178–85 3. Ochiai D, Miyakoshi K, Yakubo K, Fukuiya T, Yoshimura Y. Familial blue rubber bleb nevus syndrome in pregnancy with spinal epidural involvement. Case Rep Obstet Gynecol 2013;2013:141506 4. Kisu T, Yamaoka K, Uchida Y, Mori H, Nakama T, Hisatsugu T, Miyaji H, Motooka M. A case of blue rubber bleb nevus syndrome with familial onset. Gastroenterol Jpn 1986;21:262–6 5. Munkvad M. Blue rubber bleb nevus syndrome. Dermatologica 1983;167:307–9 6 Martinez CA, Rodrigues MR, Sato DT, Silveira Junior PP, Gama RF, Mattavelli CB, Pereira JA. Blue rubber bleb nevus syndrome as a cause of lower digestive bleeding. Case Rep Surg 2014;2014:683684 7 Maisnam I, Das T, Kundu AK, Ghosh A. Blue rubber bleb nevus syndrome causing refractory anemia. J Assoc Physicians India 2020;58:246–9 8. Terata M, Kikuchi A, Kanasugi T, Fukushima A, Sugiyama T. Association of blue rubber bleb nevus syndrome and placenta previa: report of a case. J Clin Ultrasound 2013;41:517–20 9. Adeniji AA, Fairlie FM, Jones TH, Wilkinson P. Pregnancy and blue rubber bleb naevus syndrome. Br J Obstet Gynaecol 1999;106:1316–8
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