- Email: [email protected]
Anesthesia for the parturient with pseudoxanthoma elasticum
International Journal of Obstetric Anesthesia (2003) 12, 45–47 Ó 2002 Elsevier Science Ltd. All rights reserved. doi:10.1016/S0959-289X(02)00161-9
CASE REPORT
Anesthesia for the parturient with pseudoxanthoma elasticum M. J. Douglas, V. B. Gunka, P. von Dadelszen Departments of Anaesthesia and Obstetrics, University of British Columbia and British Columbia’s Women’s Hospital and Health Centre, Vancouver, BC, Canada SUMMARY. We present our experience in the anesthetic management of two parturients with pseudoxanthoma elasticum. The first had an epidural catheter inserted for labor analgesia and ultimately had a forceps delivery. The second had a cesarean section under epidural anesthesia and had a complicated postoperative course. There were no untoward effects of regional anesthesia in either of these two women. The anesthetic implications for parturients with pseudoxanthoma elasticum are discussed. Ó 2002 Elsevier Science Ltd. All rights reserved.
other medical complaints. She was allergic to penicillin and was on no medications. The pregnancy was uneventful other than for gestational diabetes, which was controlled by diet. At 39 weeks, labor was induced for oligohydramnios, using oxytocin. Nitrous oxide/oxygen (50:50) provided analgesia for early labor, followed by insertion of an epidural catheter when the cervix was approximately 3 cm dilated. The epidural catheter (Arrow Flextip PlusTM) was inserted easily at L3–4. After a 3-mL test dose of lidocaine 1.5% with epinephrine 1:200,000, analgesia was obtained using incremental bupivacaine 0.125% plain to a total of 8 mL. Epidural fentanyl 75 lg was also given. An epidural infusion of bupivacaine 0.125% with fentanyl 2 lg/mL was started at a rate of 10 mL/h. Approximately 2 h later the cervix was fully dilated and she started pushing 1 h after that. Because of failure of the presenting part to descend she had a forceps assisted delivery following an epidural top-up with carbonated lidocaine 1.73% with epinephrine 1:200,000 (total 12 mL). She had an uneventful post-partum course.
INTRODUCTION Pseudoxanthoma elasticum (PXE) is a rare inherited disorder of connective tissue that mainly involves the skin, eyes and cardiovascular system. There are several case reports of pregnancy in women with PXE1–7 but there are no reports about the use of analgesia and anesthesia for labor and delivery. Additionally, anesthesia for PXE in the non-pregnant population is rarely described.8;9 Recently we have been involved in the care of two women with this disorder and report our experience.
CASE REPORT 1 This nulliparous woman was diagnosed with PXE at age 11. She had typical skin changes and the diagnosis was confirmed with skin biopsy. Ophthalmological examination at age 21 revealed pigmentary changes and angioid streaks that are characteristic of PXE. During her pregnancy she was seen in consultation by a member of the anesthesia department and a plan for labor analgesia was developed. She was followed by the cardiology department throughout the pregnancy for clinical evidence of mitral valve prolapse (mid-systolic click, grade II/VI apical systolic crescendo murmur) although an echocardiogram revealed normal mitral valve structure. She had no history of gastrointestinal bleeding or intermittent claudication. She reported no
CASE REPORT 2 This nulliparous woman was diagnosed with PXE at age nine. She had typical skin changes; the skin of the neck was the major area involved but she also had changes to the skin of her axillae, antecubital fossae and groins. Ophthalmological examination revealed angioid streaks in her retina. She denied having any gastrointestinal bleeding, hypertension or cardiac symptoms before pregnancy. She was followed during her pregnancy by a cardiologist. At times she was aware of ‘‘pounding in
Accepted June 2002 Correspondence to: M. J. Douglas, Department of Anesthesia, BC WomenÕs Hospital, 4500 Oak Street, Vancouver, BC, V6H 3N1. Fax: +1-(604)-875-2733; E-mail: [email protected] 45
46 International Journal of Obstetric Anesthesia her chest’’ that was present during exertion and at rest and would stop spontaneously after 2–3 min. Twentyfour hour Holter monitoring showed occasional atrial premature beats; a 12-lead ECG was normal as was an echocardiogram. Following communication with other women with PXE who had undergone pregnancy, this parturient requested an elective cesarean section due to concerns that vaginal delivery could lead to poor vaginal wound healing with calcification. She also requested removal of excess abdominal skin at the time of surgery as she had information that it would remain stretched when she was no longer pregnant. She was seen in consultation in the anesthesia clinic at 36 weeksÕ gestation when anesthetic options, risks and benefits were discussed. One unit of autologous blood was collected before surgery. Three days before her scheduled cesarean section, her membranes ruptured and labor began spontaneously. On admission, she reported a recent upper respiratory tract infection with residual cough. As the operating room was busy an epidural catheter (Arrow Flextip PlusTM) was inserted at L3–4 for labor analgesia. Analgesia was induced with an 8-mL incremental injection of 0.25% bupivacaine and 3 mL of 0.5% bupivacaine. Later the block was extended to provide anesthesia for cesarean section with incremental injection of 15 mL of carbonated lidocaine 1.73% with epinephrine 1:200,000. Epidural morphine 3 mg was given for postoperative analgesia. Prophylactic cefazolin 2 g was administered i.v. during surgery. Shivering was troublesome and was treated with i.v. meperidine 25 mg twice. After delivery of the baby the parturient had a persistent tachycardia at 140 bpm. Estimated blood loss was 2 L. In the recovery room her oral temperature was 38.5 °C. Antibiotics were continued and it was decided to monitor her for at least 24 h in the high risk area. The following morning her oxygen saturation decreased to 90% on room air and she had bilateral basal crepitations. As fluid overload was considered a factor (fluid balance +4 L), furosemide 10 mg was administered i.v. The chest X-ray did not show any evidence of fluid overload but there was a left lower lobe infiltrate leading to a differential diagnosis of atelectasis or pneumonia. Her condition gradually improved over the next 24 h. Her preoperative hemoglobin was 9.9 g/dL and this decreased to 8.8 g/dL postoperatively. She was discharged on day 6. There were no anesthetic complications.
DISCUSSION Pseudoxanthoma elasticum is a systemic disorder that affects elastic tissue in the skin, eyes and arteries.10 The term pseudoxanthoma refers to the typical yellow papules that coalesce to form plaques in the skin at flexural areas, such as the neck, groins and antecubital fossae. Histo-
logically the elastic fibers are calcified and fragmented. These changes occur in the dermis as well as in large arteries, including the coronaries. Clinically significant complications of PXE include visual loss from subretinal hemorrhage, gastrointestinal hemorrhage, angina, hypertension and intermittent claudication.10 Both autosomal dominant and autosomal recessive patterns of inheritance are described. Research using linkage studies in familial PXE has identified a gene on chromosome 16 in those families with the autosomal recessive form.11 At present, prenatal diagnosis is not available for PXE. Both of the cases described above have no family history of PXE and genetic studies have not been done. Angioid streaks are red to brown bands that radiate from the peripapillary area or are circumferential with the optic disc. They result from breaks in BruchÕs membrane; a membrane containing elastin that is situated between the retina and choroid.12 Angioid streaks, per se, are asymptomatic but subretinal hemorrhage, choroidal neovascularization and macular atrophy may occur. Case reports of PXE in pregnancy suggest that intrauterine growth retardation may present more frequently, although the total number of reported cases is small.3–5 Increased fetal loss in the first trimester is also reported.6 Necrotic changes and mineralization are more common in the placentas of women with PXE.6;7;13 The reported effects of pregnancy on PXE mainly relate to an aggravation of the skin lesions, particularly on the abdomen.3;6;7 Gastrointestinal hemorrhage has been reported during pregnancy3 and aspirin and non-steroidal anti-inflammatory drugs should be avoided. There are few reports of anesthesia in any patients with PXE and none in parturients. As some of the parturients reported have had operative deliveries it is obvious that they have received anesthesia. However, the case reports do not state whether the parturients received regional or general anesthesia. Regional anesthesia theoretically may benefit the parturient with PXE. By stabilizing the hemodynamic changes that accompany labor there may be less risk of hemorrhage from affected arteries and less demand on the heart. As women with PXE may have accelerated atherosclerosis,14 it is important to maintain normotension and avoid tachycardia during labor. Patients with PXE may develop retinal bleeding secondary to heavy straining so consideration should be given to shortening the second stage of labor through the use of forceps or vacuum. There is a theoretical risk that insertion of an epidural catheter might lead to an epidural hematoma. However, the benefits would seem to outweigh the risks. Certainly, the use of a flexible epidural catheter should decrease the risk of trauma to epidural veins. Epidural rather than spinal anesthesia may be the best option for cesarean section, because of the more gradual onset of sympathetic block and lower risk of hypoten-
The parturient with pseudoxanthoma elasticum 47 sion. The hypertensive response to intubation must be avoided during induction of general anesthesia as it may cause hemorrhage from an affected vessel. There is one case report of a difficult endotracheal intubation in a 39year-old male with PXE.9 The authors postulated that rigidity and deformity of the larynx were secondary to calcification of the elastic fibers in the laryngeal ligaments and cartilage. There are no other reports of difficult intubation in the literature. These two cases illustrate the factors that have to be taken into consideration when administering anesthesia to parturients with PXE. Epidural anesthesia was used successfully and uneventfully in both. However, there is little experience to confirm that this is a safe technique for all parturients with PXE. These women should be seen in consultation early in pregnancy so that a thorough evaluation of the clinical condition can be made. Such a consultation also provides an opportunity for discussion of the risks and benefits of the various anesthetic options. REFERENCES 1. Berde C, Willis D C, Sandberg E C. Pregnancy in women with pseudoxanthoma elasticum. Obstet Gynecol Surv 1983; 38: 339–344. 2. Lao T T, Walters B N J, De Swiet M. Pseudoxanthoma elasticum and pregnancy. Two case reports. Br J Obstet Gynaecol 1984; 91: 1049–1050.
3. Viljoen D L, Beatty S, Beighton P. The obstetric and gynaecological implications of pseudoxanthoma elasticum. Br J Obstet Gynaecol 1987; 94: 884–888. 4. Elejalde B R, de Elejalde M M, Samter R, Burgess J, Lombardi J, Gilbert E F. Manifestations of pseudoxanthoma elasticum during pregnancy: a case report and review of the literature. Am J Med Genet 1984; 18: 755–762. 5. Broekhuizen F F, Hamilton P R. Pseudoxanthoma elasticum and intrauterine growth retardation. Am J Obstet Gynecol 1984; 148: 112–114. 6. Yoles A, Phelps R, Lebwohl M. Pseudoxanthoma elasticum and pregnancy. Cutis 1996; 58: 161–164. 7. Valenzano M, Corticelli A, Podesta M, Nicoletti L, Saffioti S, Derchi L. Pseudoxanthoma elasticum and pregnancy: a case report. Clin Exp Obstet Gynecol 2000; 27: 215–217. 8. Wilson Krechel S L, Ramirez-Inawat R C, Fabian L W. Anesthetic considerations in pseudoxanthoma elasticum. Anesth Analg 1981; 60: 344–347. 9. Levitt M W D, Collison J M. Difficult endotracheal intubation in a patient with pseudoxanthoma elasticum. Anaesth Intensive Care 1982; 10: 62–64. 10. Sherer D W, Sapadin A N, Lebwohl M G. Pseudoxanthoma elasticum: an update. Dermatology 1999; 199: 3–7. 11. Perdu J, Germain D P. Identification of novel polymorphisms in the pM5 and MRP1 (ABCC1) genes at locus 16p13.1 and exclusion of both genes as responsible for pseudoxanthoma elasticum. Hum Mutat 2001; 17: 74–75. 12. Vanslembrouck I, Schurgers M. Pseudoxanthoma elasticum. Acta Clin Belg 2000; 55: 170–173. 13. Gheduzzi D, Taparelli F, Quaglino Jr. D, et al. The placenta in pseudoxanthoma elasticum: clinical, structural and immunochemical study. Placenta 2001; 22: 580–590. 14. Nolte K B. Sudden cardiac death owing to pseudoxanthoma elasticum: a case report. Hum Pathol 2000; 31: 1002–1004.
CASE REPORT
Anesthesia for the parturient with pseudoxanthoma elasticum M. J. Douglas, V. B. Gunka, P. von Dadelszen Departments of Anaesthesia and Obstetrics, University of British Columbia and British Columbia’s Women’s Hospital and Health Centre, Vancouver, BC, Canada SUMMARY. We present our experience in the anesthetic management of two parturients with pseudoxanthoma elasticum. The first had an epidural catheter inserted for labor analgesia and ultimately had a forceps delivery. The second had a cesarean section under epidural anesthesia and had a complicated postoperative course. There were no untoward effects of regional anesthesia in either of these two women. The anesthetic implications for parturients with pseudoxanthoma elasticum are discussed. Ó 2002 Elsevier Science Ltd. All rights reserved.
other medical complaints. She was allergic to penicillin and was on no medications. The pregnancy was uneventful other than for gestational diabetes, which was controlled by diet. At 39 weeks, labor was induced for oligohydramnios, using oxytocin. Nitrous oxide/oxygen (50:50) provided analgesia for early labor, followed by insertion of an epidural catheter when the cervix was approximately 3 cm dilated. The epidural catheter (Arrow Flextip PlusTM) was inserted easily at L3–4. After a 3-mL test dose of lidocaine 1.5% with epinephrine 1:200,000, analgesia was obtained using incremental bupivacaine 0.125% plain to a total of 8 mL. Epidural fentanyl 75 lg was also given. An epidural infusion of bupivacaine 0.125% with fentanyl 2 lg/mL was started at a rate of 10 mL/h. Approximately 2 h later the cervix was fully dilated and she started pushing 1 h after that. Because of failure of the presenting part to descend she had a forceps assisted delivery following an epidural top-up with carbonated lidocaine 1.73% with epinephrine 1:200,000 (total 12 mL). She had an uneventful post-partum course.
INTRODUCTION Pseudoxanthoma elasticum (PXE) is a rare inherited disorder of connective tissue that mainly involves the skin, eyes and cardiovascular system. There are several case reports of pregnancy in women with PXE1–7 but there are no reports about the use of analgesia and anesthesia for labor and delivery. Additionally, anesthesia for PXE in the non-pregnant population is rarely described.8;9 Recently we have been involved in the care of two women with this disorder and report our experience.
CASE REPORT 1 This nulliparous woman was diagnosed with PXE at age 11. She had typical skin changes and the diagnosis was confirmed with skin biopsy. Ophthalmological examination at age 21 revealed pigmentary changes and angioid streaks that are characteristic of PXE. During her pregnancy she was seen in consultation by a member of the anesthesia department and a plan for labor analgesia was developed. She was followed by the cardiology department throughout the pregnancy for clinical evidence of mitral valve prolapse (mid-systolic click, grade II/VI apical systolic crescendo murmur) although an echocardiogram revealed normal mitral valve structure. She had no history of gastrointestinal bleeding or intermittent claudication. She reported no
CASE REPORT 2 This nulliparous woman was diagnosed with PXE at age nine. She had typical skin changes; the skin of the neck was the major area involved but she also had changes to the skin of her axillae, antecubital fossae and groins. Ophthalmological examination revealed angioid streaks in her retina. She denied having any gastrointestinal bleeding, hypertension or cardiac symptoms before pregnancy. She was followed during her pregnancy by a cardiologist. At times she was aware of ‘‘pounding in
Accepted June 2002 Correspondence to: M. J. Douglas, Department of Anesthesia, BC WomenÕs Hospital, 4500 Oak Street, Vancouver, BC, V6H 3N1. Fax: +1-(604)-875-2733; E-mail: [email protected] 45
46 International Journal of Obstetric Anesthesia her chest’’ that was present during exertion and at rest and would stop spontaneously after 2–3 min. Twentyfour hour Holter monitoring showed occasional atrial premature beats; a 12-lead ECG was normal as was an echocardiogram. Following communication with other women with PXE who had undergone pregnancy, this parturient requested an elective cesarean section due to concerns that vaginal delivery could lead to poor vaginal wound healing with calcification. She also requested removal of excess abdominal skin at the time of surgery as she had information that it would remain stretched when she was no longer pregnant. She was seen in consultation in the anesthesia clinic at 36 weeksÕ gestation when anesthetic options, risks and benefits were discussed. One unit of autologous blood was collected before surgery. Three days before her scheduled cesarean section, her membranes ruptured and labor began spontaneously. On admission, she reported a recent upper respiratory tract infection with residual cough. As the operating room was busy an epidural catheter (Arrow Flextip PlusTM) was inserted at L3–4 for labor analgesia. Analgesia was induced with an 8-mL incremental injection of 0.25% bupivacaine and 3 mL of 0.5% bupivacaine. Later the block was extended to provide anesthesia for cesarean section with incremental injection of 15 mL of carbonated lidocaine 1.73% with epinephrine 1:200,000. Epidural morphine 3 mg was given for postoperative analgesia. Prophylactic cefazolin 2 g was administered i.v. during surgery. Shivering was troublesome and was treated with i.v. meperidine 25 mg twice. After delivery of the baby the parturient had a persistent tachycardia at 140 bpm. Estimated blood loss was 2 L. In the recovery room her oral temperature was 38.5 °C. Antibiotics were continued and it was decided to monitor her for at least 24 h in the high risk area. The following morning her oxygen saturation decreased to 90% on room air and she had bilateral basal crepitations. As fluid overload was considered a factor (fluid balance +4 L), furosemide 10 mg was administered i.v. The chest X-ray did not show any evidence of fluid overload but there was a left lower lobe infiltrate leading to a differential diagnosis of atelectasis or pneumonia. Her condition gradually improved over the next 24 h. Her preoperative hemoglobin was 9.9 g/dL and this decreased to 8.8 g/dL postoperatively. She was discharged on day 6. There were no anesthetic complications.
DISCUSSION Pseudoxanthoma elasticum is a systemic disorder that affects elastic tissue in the skin, eyes and arteries.10 The term pseudoxanthoma refers to the typical yellow papules that coalesce to form plaques in the skin at flexural areas, such as the neck, groins and antecubital fossae. Histo-
logically the elastic fibers are calcified and fragmented. These changes occur in the dermis as well as in large arteries, including the coronaries. Clinically significant complications of PXE include visual loss from subretinal hemorrhage, gastrointestinal hemorrhage, angina, hypertension and intermittent claudication.10 Both autosomal dominant and autosomal recessive patterns of inheritance are described. Research using linkage studies in familial PXE has identified a gene on chromosome 16 in those families with the autosomal recessive form.11 At present, prenatal diagnosis is not available for PXE. Both of the cases described above have no family history of PXE and genetic studies have not been done. Angioid streaks are red to brown bands that radiate from the peripapillary area or are circumferential with the optic disc. They result from breaks in BruchÕs membrane; a membrane containing elastin that is situated between the retina and choroid.12 Angioid streaks, per se, are asymptomatic but subretinal hemorrhage, choroidal neovascularization and macular atrophy may occur. Case reports of PXE in pregnancy suggest that intrauterine growth retardation may present more frequently, although the total number of reported cases is small.3–5 Increased fetal loss in the first trimester is also reported.6 Necrotic changes and mineralization are more common in the placentas of women with PXE.6;7;13 The reported effects of pregnancy on PXE mainly relate to an aggravation of the skin lesions, particularly on the abdomen.3;6;7 Gastrointestinal hemorrhage has been reported during pregnancy3 and aspirin and non-steroidal anti-inflammatory drugs should be avoided. There are few reports of anesthesia in any patients with PXE and none in parturients. As some of the parturients reported have had operative deliveries it is obvious that they have received anesthesia. However, the case reports do not state whether the parturients received regional or general anesthesia. Regional anesthesia theoretically may benefit the parturient with PXE. By stabilizing the hemodynamic changes that accompany labor there may be less risk of hemorrhage from affected arteries and less demand on the heart. As women with PXE may have accelerated atherosclerosis,14 it is important to maintain normotension and avoid tachycardia during labor. Patients with PXE may develop retinal bleeding secondary to heavy straining so consideration should be given to shortening the second stage of labor through the use of forceps or vacuum. There is a theoretical risk that insertion of an epidural catheter might lead to an epidural hematoma. However, the benefits would seem to outweigh the risks. Certainly, the use of a flexible epidural catheter should decrease the risk of trauma to epidural veins. Epidural rather than spinal anesthesia may be the best option for cesarean section, because of the more gradual onset of sympathetic block and lower risk of hypoten-
The parturient with pseudoxanthoma elasticum 47 sion. The hypertensive response to intubation must be avoided during induction of general anesthesia as it may cause hemorrhage from an affected vessel. There is one case report of a difficult endotracheal intubation in a 39year-old male with PXE.9 The authors postulated that rigidity and deformity of the larynx were secondary to calcification of the elastic fibers in the laryngeal ligaments and cartilage. There are no other reports of difficult intubation in the literature. These two cases illustrate the factors that have to be taken into consideration when administering anesthesia to parturients with PXE. Epidural anesthesia was used successfully and uneventfully in both. However, there is little experience to confirm that this is a safe technique for all parturients with PXE. These women should be seen in consultation early in pregnancy so that a thorough evaluation of the clinical condition can be made. Such a consultation also provides an opportunity for discussion of the risks and benefits of the various anesthetic options. REFERENCES 1. Berde C, Willis D C, Sandberg E C. Pregnancy in women with pseudoxanthoma elasticum. Obstet Gynecol Surv 1983; 38: 339–344. 2. Lao T T, Walters B N J, De Swiet M. Pseudoxanthoma elasticum and pregnancy. Two case reports. Br J Obstet Gynaecol 1984; 91: 1049–1050.
3. Viljoen D L, Beatty S, Beighton P. The obstetric and gynaecological implications of pseudoxanthoma elasticum. Br J Obstet Gynaecol 1987; 94: 884–888. 4. Elejalde B R, de Elejalde M M, Samter R, Burgess J, Lombardi J, Gilbert E F. Manifestations of pseudoxanthoma elasticum during pregnancy: a case report and review of the literature. Am J Med Genet 1984; 18: 755–762. 5. Broekhuizen F F, Hamilton P R. Pseudoxanthoma elasticum and intrauterine growth retardation. Am J Obstet Gynecol 1984; 148: 112–114. 6. Yoles A, Phelps R, Lebwohl M. Pseudoxanthoma elasticum and pregnancy. Cutis 1996; 58: 161–164. 7. Valenzano M, Corticelli A, Podesta M, Nicoletti L, Saffioti S, Derchi L. Pseudoxanthoma elasticum and pregnancy: a case report. Clin Exp Obstet Gynecol 2000; 27: 215–217. 8. Wilson Krechel S L, Ramirez-Inawat R C, Fabian L W. Anesthetic considerations in pseudoxanthoma elasticum. Anesth Analg 1981; 60: 344–347. 9. Levitt M W D, Collison J M. Difficult endotracheal intubation in a patient with pseudoxanthoma elasticum. Anaesth Intensive Care 1982; 10: 62–64. 10. Sherer D W, Sapadin A N, Lebwohl M G. Pseudoxanthoma elasticum: an update. Dermatology 1999; 199: 3–7. 11. Perdu J, Germain D P. Identification of novel polymorphisms in the pM5 and MRP1 (ABCC1) genes at locus 16p13.1 and exclusion of both genes as responsible for pseudoxanthoma elasticum. Hum Mutat 2001; 17: 74–75. 12. Vanslembrouck I, Schurgers M. Pseudoxanthoma elasticum. Acta Clin Belg 2000; 55: 170–173. 13. Gheduzzi D, Taparelli F, Quaglino Jr. D, et al. The placenta in pseudoxanthoma elasticum: clinical, structural and immunochemical study. Placenta 2001; 22: 580–590. 14. Nolte K B. Sudden cardiac death owing to pseudoxanthoma elasticum: a case report. Hum Pathol 2000; 31: 1002–1004.