Skip to main content Accessibility help
  • Get access
    Check if you have access via personal or institutional login
  • Cited by 2
  • Print publication year: 2008
  • Online publication date: October 2009

8 - Miscellaneous skeletal and connective tissue disorders in pregnancy

from Section 2 - Musculoskeletal disorders



This chapter discusses a variety of miscellaneous conditions found during pregnancy, each with different degrees of rarity. It focuses on the pathophysiologic changes that occur with each disease in order to highlight the impact on both anesthetic and obstetric management. However, as some of the conditions described have a wide and varied organ involvement, firm management conclusions cannot be made. Each case should be assessed individually and the risk/benefit of any given anesthetic choice should be evaluated for each particular patient.

Gorlin syndrome (Gorlin-Golty syndrome or nevoid basal cell carcinoma syndrome)

Gorlin syndrome was first described in 1960. The characteristic features are multiple basal cell nevi, odontogenic keratocysts of the mandible, and other congenital, mostly skeletal, abnormalities. A variety of other neurological, genital, endocrine, and ophthalmic manifestations are also associated with this disease. A predisposition to tumor formation is well documented; those described include ovarian fibromas, central nervous system (CNS) tumors (mostly medulloblastomas), skin, and, occasionally, cardiac tumors. Although this disorder has an autosomal dominant mode of inheritance, 50% arise from spontaneous mutations. The causative gene mutation is located on chromosome 9q22.3, which is normally required for the function of a transmembrane receptor involved in patterning and growth. This Gorlin mutation reduces its ability to act as a tumor suppressor gene resulting in the increased incidence of cancers. Males and females are equally affected and the disease prevalence is about 1 per 60,000. The condition has a variable phenotypic expression with most cases reported in Caucasians.

Related content

Powered by UNSILO
Gorlin, R. J. & Goltz, R. W.Multiple nevoid basal cell epithelioma, jaw cysts and bifid ribs: a syndrome. N. Engl. J. Med. 1960; 262: 908–12.
Gorlin, R. J.Nevoid basal cell carcinoma syndrome. Medicine 1987; 66: 98–113.
Herman, T. E., Siegel, M. J. & McAlister, W. H.Cardiac tumor in Gorlin syndrome. Nevoid basal cell carcinoma syndrome. Pediatr. Radiol. 1991; 21: 234–5.
Gorlin, R. J.Nevoid basal cell carcinoma (Gorlin) syndrome: unanswered issues. J. Lab. Clin. Med. 1999; 134: 551–2.
Manfredi, M., Vescovi, P., Bonanini, M. & Porter, S.Nevoid basal cell carcinoma syndrome: a review of the literature. Int. J. Oral. Maxillofac. Surg. 2004; 33: 117–24
Southwick, G. J. & Schwartz, R. A.The basal cell nevus syndrome. Disasters occurring among a series of 36 patients. Cancer 1979; 44: 2294–305.
Yoshizumi, J., Vaughan, R. S. & Jasani, B.Pregnancy associated with Gorlin's syndrome. Anaesthesia 1990; 45: 1046–8.
Fox, R., Eckford, S., Hirschowitz, L., Browning, J. & Lindop, G.Refractory gestational hypertension due to a renin secreting ovarian fibrothecoma associated with Gorlin's syndrome. Br. J. Obstet. Gynaecol. 1994; 101: 1015–17.
Noonan, J. A. & Ehmke, D. A.Associated non cardiac malformations in children with congenital heart disease. J. Pediatr. 1963; 63: 468–70.
Mendez, H. M. M. & Opitz, J. M.Noonan syndrome: a review. Am. J. Med. Genet. 1985; 21: 493–506.
Tartaglia, M., Mehler, E. L., Goldberg, al. Mutations in PTPN11, encoding the protein tyrosine phosphatase SHP 2, cause Noonan syndrome. Nat. Genet. 2001; 29: 465–8.
Sugar, A. W., Ezsias, A., Bloom, A. L. & Morcos, W. E.Orthognathic surgery in a patient with Noonan's syndrome. J. Oral Maxillofac. Surg. 1994; 52: 421–5.
Hauwaert, L. G., Fryns, J. P., Dumoulin, M. & Logghe, N.Cardiovascular malformations in Turner's and Noonan's syndrome. Br. Heart J. 1978; 40: 500–9.
Sharland, M., Burch, M., McKenna, W. M. & Pattern, M. A.A clinical study of Noonan syndrome. Arch. Dis. Childhood. 1992; 67: 178–83.
Pearl, W.Cardiovascular anomalies in Noonan's syndrome. Chest 1977; 71: 677–9.
Witt, D. R., McGillivray, B. C., Allanson, J. al. Bleeding diathesis in Noonan syndrome – a common association. Am. J. Med. Genet. 1988; 31: 305–17.
Lee, C. K., Chang, B. S., Hong, Y. al. Spinal deformities in Noonan syndrome: a clinical review of sixty cases. J. Bone Joint Surg. 2001; 83: 10: 1495–502.
Berkowitz, I. D., Raja, S. N., Bender, K. S. & Kopits, S. E.Dwarfs: pathophysiology and anesthetic implications. Anesthesiology 1990; 73: 739–59.
Schwartz, N. & Eisenkraft, J.Anesthetic management of a child with Noonan's syndrome and idiopathic hypertrophic subaortic stenosis. Anesth. Analg. 1992; 74: 464–6.
Campbell, A. M. & Bousfield, J. D.Anaesthesia in a patient with Noonan's syndrome and cardiomyopathy. Anaesthesia 1992; 47: 131–3.
Dadabhoy, Z. P. & Winnie, A. P.Regional anesthesia for cesarean section in a parturient with Noonan's syndrome. Anesthesiology 1988; 68: 636–8.
McLure, H. A. & Yentis, S. M.General anaesthesia for Caesarean section in a parturient with Noonan's syndrome. Br. J. Anaesth. 1996; 77; 665–8.
Magboul, M. A.Anaesthetic management of emergency caesarean section in a patient with Noonan's syndrome. Middle East J. Anaesth. 2000; 15: 611–17.
Cullimore, A. J., Smedstad, K. G. & Brennan, B. G.Pregnancy in women with Noonan syndrome: report of two cases. Obstet. Gynecol. 1999; 5: 813–15.
Grange, C. S., Heid, R., Lucas, S. B., Ross, P. L. E. & Douglas, M. J.Anaesthesia in a parturient with Noonan's syndrome. Can. J. Anaesth. 1998; 45: 332–6.
Lammert, M., Friedman, J. M., Kluwe, L. & Mautner, V. F.Prevalence of neurofibromatosis 1 in German children at elementary school enrollment. Arch. Dermatol. 2005; 141: 71–4.
Ledbetter, D. H., Rich, D. C., O'Connell, P., Leppert, M. & Carey, J. C.Precise localization of NF1 to 17q11.2 by balanced translocation. Am. J. Hum. Genet. 1989; 44: 20– 4.
Huson, S. M.Recent developments in the diagnosis and management of neurofibromatosis. Arch. Dis. Child. 1989; 64: 745–9.
North, K. N.Neurofibromatosis type 1: review of the first 200 patients in an Australian clinic. J. Child. Neurol 1993; 8: 395–402.
Gutmann, D. H., Aylsworth, A., Carey, J. al. The diagnostic evaluation and multidisciplinary management of neurofibromatosis 1 and neurofibromatosis 2. J. Am. Med. Assoc. 1997; 278: 51–7.
Kloos, R. T., Rufini, V., Gross, M. D. & Shapiro, B.Bone scans in neurofibromatosis: neurofibroma, plexiform neurofibroma and neurofibrosarcoma. J. Nucl. Med. 1996; 37: 1778–83.
Gutmann, D. H.Recent insights into neurofibromatosis type 1: clear genetic progress. Arch. Neurology. 1998; 55: 778–80.
Korf, B. R.Malignancy in neurofibromatosis type 1. Oncologist 2000; 5: 477–85.
Barkovich, A. J. & Kuzniecky, R. I. Congenital, developmental, and neurocutaneous disorders. In Goldman, L. & Ausiello, D. (eds.), Cecil Textbook of Medicine, 22nd edn. Philadelphia, PA: Sanders, 2004.
Segal, D., Holcberg, G., Sapir, al. Neurofibromatosis in pregnancy: maternal and perinatal outcome. Eur. J. Obstet. Gynecol. Reprod. Biol. 1999; 84: 59–61.
Sharma, J. B., Gulati, N. & Malik, S.Maternal and perinatal complications in neurofibromatosis during pregnancy. Int. J. Gynecol. Obstet. 1991; 34: 221–7.
Hagymásy, L., Tóth, M., Szücs, N. & Rigó, J.Neurofibromatosis type 1 with pregnancy-associated renovascular hypertension, and the syndrome of hemolysis, elevated liver enzymes, and low platelets. Am. J. Obstet. Gynecol. 1998; 179: 272–4.
Posma, E., Aalbers, R., Kurniawan, Y. al. Neurofibromatosis type 1 and pregnancy: a fatal attraction? Development of malignant schwannoma during pregnancy in a patient with neurofibromatosis type 1. Br. J. Obstet. Gynaecol. 2003; 110: 530–2.
Hadi, H. A.Clinical significance of neurofibromatosis and pregnancy. Am. J. Perinatol. 1995; 12: 459–61.
Dounas, M., Mercier, F. J., Lhuissier, C. & Benhamou, D.Epidural analgesia for labor in a parturient with neurofibromatosis. Can. J. Anaesth. 1995; 42: 420–4.
Sakai, T., Vallejo, M. C. & Shannon, K. T.A parturient with neurofibromatosis type 2: anesthetic and obstetric considerations for delivery. Int. J. Obstet. Anesth. 2005; 14: 332–5.
Spiegel, J. E., Hapgood, A. & Hess, P. E.Epidural anesthesia in a parturient with neurofibromatosis type 2 undergoing cesarean section. Int. J. Obstet. Anesth. 2005; 14: 336–9.
Esler, M. D., Durbridge, J. & Kirby, S.Epidural haematoma after dural puncture in a parturient with neurofibromatosis. Br. J. Anaesth. 2001; 87: 932–4.
Walther, M. M., Herring, J., Enquist, E., Keiser, H. R. & Linehan, W. M.Von Recklinghausen's disease and phaeochromocytoma. J. Urol. 1999; 162: 1582–6.
Hirsch, N. P., Murphy, A. & Radcliffe, J. J.Neurofibromatosis: clinical presentations and anaesthetic implications. Br. J. Anaesth. 2001; 86: 555–64.
Richardson, M. G., Setty, G. K. & Rawoof, S. A.Responses to nondepolarizing neuromuscular blockers and succinylcholine in the von Recklinghausen neurofibromatosis. Anesth. Analg. 1996; 82: 382–5.
Roach, E. S., Gomez, M. R. & Northrup, H.Tuberous sclerosis complex consensus conference: revised clinical diagnostic criteria. J. Child. Neurol. 1998; 13: 624–8.
Northrup, H., Kwiatkowski, D. J., Roach, E. al. Evidence for genetic heterogeneity in tuberous sclerosis: one locus on chromosome 9 and at least one locus elsewhere. Am. J. Hum. Genet. 1992; 51: 709–20.
Cleary-Goldman, J., Sanghvi, A. V., Nakuda, G. S. & Robinson, J. N.Conservative management of pulmonary lymphangioleiomyomatosis and tuberous sclerosis complicated by renal angiomyolipomas in pregnancy. J. Matern. Fetal Neonatal Med. 2004; 15: 132–4.
Gounden, Y. P.Tuberous sclerosis in pregnancy: a case report and review of the literature. Aust. N. Z. Obstet. Gynaecol. 2002; 42: 551–2.
Petrikovsky, B. M., Vintzileos, A. M., Cassidy, S. B. & Egan, J. F.Tuberous sclerosis in pregnancy. Am. J. Perinatol. 1990; 7: 133–5.
Carter, S. M., Chazotte, C. & Caride, D.Pregnancy courses in a patient with tuberous sclerosis. Obstet. Gynecol. 1996; 88: 724.
King, J. A. & Stamilio, D. M.Maternal and fetal tuberous sclerosis complicating pregnancy: a case report and overview of the literature. Am. J. Perinatol. 2005; 22: 103–8.
Mitchell, A. L., Parisi, M. A. & Sybert, V. P.Effects of pregnancy on the renal and pulmonary manifestations in women with tuberous sclerosis complex. Gene Med. 2003; 5: 154–60.
Forsnes, E. V., Eggleston, M. K. & Burtman, M.Placental abruption and spontaneous rupture of renal angiomyolipoma in a pregnant woman with tuberous sclerosis. Obstet. Gynecol. 1996; 88: 725.
McLoughlin, L., Thomas, G. & Hasan, K.Pregnancy and lymphangioleiomyomatosis: anaesthetic management. Int. J. Obstet. Anesth. 2003; 12: 40–4.
Lee, J. J., Imrie, M. & Taylor, V.Anaesthesia and tuberous sclerosis. Br. J. Anaesth. 1994; 73: 421–5.
Ong, E. L. & Koay, C. K.Tuberous sclerosis presenting for laparotomy. Anaesth. Intensive Care. 2000; 28: 94–6.
Wanebo, J. E., Lonser, R. R., Glenn, G. M. & Oldfield, E. H.The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease. J. Neurosurg. 2003; 98: 82–94.
Eisenhofer, G., Walther, M. M., Huynh, T. al. Pheochromocytomas in von Hippel-Landau Syndrome and multiple endocrine neoplasia type 2 display distinct biochemical and clinical phenotypes. J. Clin. Endocrinol. Metab. 2001; 86: 1999–2008.
Grimbert, P., Chauveau, D., Remy, S. R. & Grunfeld, J. P.Pregnancy in von Hippel-Lindau disease. Am. J. Obstet. Gynecol. 1999; 180: 110–11.
Berl, M., Dubois, L., Belkacem, H., Dailland, P. & Carli, P.Von Hippel-Lindau disease and obstetric anaesthesia: 3 case reports. Ann. Fr. Anesth. Reanim. 2003; 22: 359–62.
Joffe, D., Robbins, R. & Benjamin, A.Caesarean section and phaeochromocytoma resection in a patient with von Hippel-Lindau disease. Can. J. Anaesth. 1993; 40: 870–4.
Demiraran, Y., Ozgon, M., Utku, T. & Bozkurt, P.Epidural anaesthesia for Caesarean section in a patient with von Hippel-Lindau disease. Eur. J. Anaesthesiol. 2001; 18: 330–2.
Wang, A. & Sinatra, R. S.Epidural anesthesia for cesarean section in a patient with von Hippel-Lindau disease and multiple sclerosis. Anesth. Analg. 1999; 88: 1083–4.
Delisle, M. F., Valimohamed, F., Money, D. & Douglas, M. J.Central nervous system complications of von Hippel-Lindau disease and pregnancy perinatal considerations: case report and literature review. J. Matern. Fetal Med. 2000; 9: 242–7.
Hughes, B. W., Casillas, M. L. M. & Kaminski, H. J.Pathophysiology of myasthenia gravis. Semin. Neurol. 2004; 24: 21–30.
Phillips, L. H.The epidemiology of myasthenia gravis. Semin. Neurol. 2004; 24: 17–20.
Tan, J. H. & Ho, K. H.Familial autoimmune myasthenia gravis. Singapore Med. J. 2001; 42: 178–9.
Keesey, J. C.Clinical management of myasthenia gravis. Muscle Nerve 2004; 29: 485–505.
Meriggioli, M. N. & Sanders, D. B.Myasthenia gravis: diagnosis. Semin. Neurol. 2004; 24: 31–9.
Pascuzzi, R. M.The edrophonium test. Semin. Neurol. 2003; 23: 83–8.
Saperstein, D. S. & Barohn, R. J.Management of myasthenia gravis. Semin. Neurol. 2004; 24: 41–8.
Jaretzki, A., Steinglass, K. M. & Sonett, J. R.Thymectomy in the management of myasthenia gravis. Semin. Neurol. 2004; 24: 49–62.
Meriggioli, M. N., Ciafaloni, E., Al-Hayk, K. al. Mycophenolate mofetil for myasthenia gravis: an analysis of efficacy, safety, and tolerability. Neurology 2003; 61:1438–40.
Skeie, G. O., Apostolski, S., Evoli, al. Guidelines for the treatment of autoimmune neuromuscular transmission disorders. Eur. J. Neurol. 2006; 13: 691–9.
Dalakas, M. C.Intravenous immunoglobulin in the treatment of autoimmune neuromuscular disease: present status and practical therapeutic guidelines. Muscle Nerve 1999; 22: 1479–97.
Ferrero, S., Pretta, S., Nicoletti, A., Petrera, P. & Ragni, N.Myasthenia gravis: management issues during pregnancy. Eur. J. Obstet. Gynecol. Reprod. Biol. 2005; 121: 129–38.
Djelmis, J., Sostarko, M., Mayer, D. & Ivanisevic, M.Myasthenia gravis in pregnancy: report on 69 cases. Eur. J. Obstet. Gynecol. Reprod. Biol. 2002; 104: 21–5.
Roth, T. C., Raths, J., Carboni, G., Rosler, K. & Schmid, R. A.Effect of pregnancy and birth on the course of myasthenia gravis before or after transsternal radical thymectomy. Eur. J. Cardiothorac. Surg. 2006; 29: 231–5.
Hoff, J. M., Daltveit, A. K. & Gilhus, N. E.Myasthenia gravis in pregnancy and birth: identifying risk factors, optimising care. Eur. J. Neurol. 2007; 14: 38–43.
Melber, D.Maternal–fetal transmission of myasthenia gravis with negative acetylcholine receptor antibody. N. Engl. J. Med. 1988; 318: 996.
Téllez-Zenteno, J. F., Hernández-Ronquillo, L., Salinas, V., Estanol, B. & da Silva, O.Myasthenia gravis and pregnancy: clinical implications and neonatal outcome. BMC Musculoskelet. Disord. 2004; 5: 42.
Hoff, J. M., Daltveit, A. K. & Gilhus, N. E.Myasthenia gravis: consequences for pregnancy, delivery and the newborn. Neurology 2003; 61: 1362–6.
Baraka, A.Anaesthesia and myasthenia gravis. Can. J. Anaesth 1992; 39: 476–86.
Rolbin, S. H., Levinson, G., Shnider, S. M. & Wright, R. G.Anesthetic considerations for myasthenia gravis and pregnancy. Anesth. Analg. 1978; 57: 441–7.
D'Angelo, R. & Gerancher, J. C.Combined spinal and epidural analgesia in a parturient with severe myasthenia gravis. Reg. Anesth. Pain Med. 1998; 23: 201–3.
Koh, L. K. L., Ip-Yam, P. C. & Tan, A. S. A.Perioperative management of a patient with congenital myasthenia gravis for elective caesarean section. Singapore Med. J. 2001; 42: 61–3.
Saito, Y., Sakura, S., Takatori, T. & Kosaka, Y.Epidural anaesthesia in a patient with myasthenia gravis. Acta Anaesthesiol. Scand. 1993; 37: 513–15.
O'Flaherty, D., Pennant, J. H., Rao, K. & Giesecke, A. H.Total intravenous anesthesia with propofol for transsternal thymectomy in myasthenia gravis. J. Clin. Anesth. 1992; 4: 241–4.
Eisenkraft, J. B., Book, W. J., Mann, S. M., Papatestas, A. E. & Hubbard, M.Resistance to succinylcholine in myasthenia gravis: a dose response study. Anesthesiology 1988; 69: 760–3.
Baraka, A. & Tabboush, Z.Neuromuscular response to succinylcholine-vecuronium sequence in three myasthenic patients undergoing thymectomy. Anesth. Analg. 1991; 72: 827–30.
Lumb, A. B. & Calder, I.‘Cured’ myasthenia gravis and neuromuscular blockage. Anaesthesia 1989; 44: 828–30.
Dillon, F. X.Anesthetic issues in the perioperative management of myasthenia gravis. Semin. Neurol. 2004; 24: 83–94.
Alper, G. & Narayanan, V.Friedreich's ataxia. Pediatr. Neurol. 2003; 28: 335–41.
Bencze, K. Z., Kondapalli, K. C., Cook, J. al. The structure and function of frataxin. Crit. Rev. Biochem. Mol. Biol. 2006; 41: 269–91.
Lodi, R., Tonon, C., Calabrese, V. & Schapira, A. H.Friedreich's ataxia: from disease mechanisms to therapeutic interventions. Antioxid. Redox. Signal 2006; 8: 438–43.
Durr, A., Cossee, M., Agid, al. Clinical and genetic abnormalities in patients with Friedreich's ataxia. N. Engl. J. Med. 1996; 335: 1169–75.
Alboliras, E. T., Shub, C., Gomez, M. al. Spectrum of cardiac involvement in Friedreich's ataxia: clinical electrocardiographic and echocardiographic observations. Am. J. Cardiol. 1986; 58: 518–24.
Hausse, A. O., Aggoun, Y., Bonnet, al. Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia. Heart 2002; 87: 346–9.
Mackenzie, W.Pregnancy in women with Friedriech's ataxia. Br. Med. J. (Clin. Res. Ed.) 1986; 293: 308.
Chuileannain, F. N., Macphail, S. & Misra, P.Pregnancy in a woman with Friedreich's ataxia. J. Obstet. Gynaecol. 1997; 17: 586–7.
Bruner, J. P. & Yeast, J. D.Pregnancy associated with Friedreich's ataxia. Obstet. Gynecol. 1990; 76: 976–7.
Schmitt, H. J., Wick, S. & Münster, T.Rocuronium for muscle relaxation in two children with Friedreich's ataxia. Br. J. Anaesth. 2004; 92: 592–6.
Pancaro, C. & Renz, D.Anesthetic management in Friedreich's ataxia. Paediatr. Anaesth. 2005; 15: 433–40.
Mouloudi, H., Katsanoulas, C. & Frantzeskos, G.Requirements for muscle relaxation in Friedreich's ataxia. Anaesthesia 1998: 53: 177–80.
Wyatt, S. & Brighouse, D.Anaesthetic management of vaginal delivery in a woman with Friedreich's ataxia complicated by cardiomyopathy and scoliosis. Int. J. Obstet. Anesth. 1998; 7: 185–8.
Alon, E. & Waespe, W.Epidural anaesthesia in a patient with Friedreich's Ataxia. Reg. Anaesth. 1988; 11: 58–60.
Kubal, K., Pasricha, S. K. & Bhargava, M.Spinal anesthesia in a patient with Friedreich's ataxia. Anesth. Analg. 1991; 72: 257–8.
Harmon, D.Anaesthesia for caesarean section in a parturient with Friedreich's ataxia. Int. J. Obstet. Anesth. 2001; 10: 147–8.
Kumar, R., Healy, K. & Young, S. J.Combined spinal-epidural anesthesia for caesarean section in a patient with Friedreich's ataxia. Int. J. Obstet. Anesth. 2002; 11: 73–4.
Oberoi, G. S., Kaul, H. L., Gill, I. S. & Batra, R. K.Anaesthesia in arthrogryposis multiplex congenita: case report. Can. J. Anaesth. 1987; 34: 288–90.
Hall, J. G.Arthrogryposis multiplex congenital: etiology, genetics, classification, diagnostic approach and general aspects. J. Pediatr. Orthoped. 1997; 6: 159–66.
Hoff, J. M., Daltveit, A. K. & Gilhus, N. E.Arthrogryposis multiplex congenita – a rare fetal condition caused by maternal myasthenia gravis. Acta Neurol. Scand. Suppl. 2006; 183: 26–7.
Hardwick, J. C. & Irvine, G. A.Obstetric care in arthrogryposis multiplex congenital. Br. J. Obstet. Gynaecol. 2002; 109: 1303–4.
Baines, D. B., Douglas, I. D. & Overton, J. H.Anaesthesia for patients with arthrogryposis multiplex congenita: what is the risk of malignant hyperthermia?Anaesth. Intensive Care. 1986; 14: 370–2.
Oda, Y., Yukioka, H. & Fujimori, M.Anesthesia for arthrogryposis multiplex congenital – report of 12 cases. J. Anesth. 1990; 4: 275–8.
Nguyen, N. H., Morvant, E. M. & Mayhew, J. F.Anesthetic management for patients with arthrogryposis multiplex congenital and severe micrognathia: case reports. J. Clin. Anesth. 2000; 12: 227–30.
Szmuk, P., Ezri, T., Warters, D. & Katz, J.Anesthetic management of a patient with arthrogryposis multiplex congenita and limited mouth opening. J. Clin. Anesth. 2001; 13: 59–60.
Mentzelopoulos, S. D., Armaganidis, A., Niokou, al. MRI of the upper airway and McCoy-balloon laryngoscopy with left molar approach in a patient with arthrogryposis multiplex congenital and previous unsuccessful endotracheal intubation. Anesth. Analg. 2004; 99: 1879–80.
Quance, D. R.Anaesthetic management of an obstetrical patient with arthrogryposis multiplex congenita. Can. J. Anaesth. 1988; 35: 612–14.
Rozkowski, A.Smyczek, D. & Birnbach, D. J.Continuous spinal anesthesia for cesarean delivery in a patient with arthrogryposis multiplex congenita. A clinical report. Reg. Anesth. 1996; 21: 477–9.
Spooner, L.Caesarean section using a combined spinal epidural technique in a patient with arthrogryposis multiplex congenital. Int. J. Obstet. Anesth. 2000; 9: 282–5.
Beighton, P., De Paepe, A., Steinmann, B., Tsipouras, P. & Wenstrup, R. J.Ehlers-Danlos syndromes: revised nosology, Villefranche, 1997. Ehlers-Danlos National Foundation (USA) and Ehlers-Danlos Support Group (UK). Am. J. Med. Genet. 1998; 77: 31–7.
Pyeritz, R. E.Ehlers-Danlos syndrome. N. Engl. J. Med. 2000; 342: 730–2.
Pepin, M., Schwarze, U., Superti-Furga, A. & Byers, P. H.Clinical and genetic features of Ehlers-Danlos syndrome type VI, the vascular type. N. Engl. J. Med. 2000; 342: 673–80.
Lind, J. & Wallenburg, H. C.Pregnancy and the Ehlers-Danlos syndrome: a retrospective study in a Dutch population. Acta Obstet. Gynecol. Scand. 2002; 81: 293–300.
Volkov, N., Nisenblat, V., Ohel, G. & Gonen, R.Ehlers-Danlos syndrome: insights on obstetric aspect. Obstet. Gynecol. Surv. 2007; 62: 51–7.
Lurie, S., Manor, M. & Hagay, Z. J.The threat of type IV Ehlers-Danlos syndrome on maternal well-being during pregnancy: early delivery may make the difference. J. Obstet. Gynecol. 1998; 18: 245–8.
Rudd, N. L., Nimrod, C., Holbrook, K. A. & Byers, P. H.Pregnancy complications in type IV Ehlers-Danlos syndrome. Lancet 1983; 1: 50–3.
Pope, F. M. & Nicholls, A. C.Pregnancy and Ehlers-Danlos syndrome type IV. Lancet 1983; 1: 249–50.
Sorokin, Y., Johnson, M. P., Rogowski, N., Richardson, D. A. & Evans, M. I.Obstetric and gynecologic dysfunction in the Ehlers-Danlos syndrome. J. Reprod. Med. 1994; 39: 281–4.
Snow, R. E. & Neubert, G.Peripartum pubis symphysis separation: a case series and review of literature. Obstet. Gynecol. Surv. 1997; 52: 438–43.
Peaceman, A. M. & Cruikshank, D. P.Ehlers-Danlos syndrome and pregnancy: association of type IV disease with maternal death. Obstet. Gynecol. 1987; 69: 428–31.
Roop, K. A. & Brost, B. C.Abnormal presentation in labor and fetal growth of affected infants with type III Ehlers-Danlos syndrome. Am. J. Obstet. Gynecol. 1999; 181: 752–3.
Athanassiou, A. M. & Turrentine, M. A.Myocardial infarction and coronary artery dissection during pregnancy associated with type IV Ehlers-Danlos syndrome. Am. J. Perinatol. 1996; 13: 181–3.
Kitazono, T., Imaizumi, T., Imayama, al. Two cases of myocardial infarction in type IV Ehlers-Danlos syndrome. Chest 1989; 95: 1274–7.
Price, C. M., Ford, S., Jones, John L.St & Murday, V.Myocardial ischaemia associated with Ehlers-Danlos syndrome. Br. J. Anaesth. 1996; 76: 464–6.
Paepe, A. & Malfait, F.Bleeding and bruising in patients with Ehlers-Danlos syndrome and other collagen vascular disorders. Br. J. Haematol. 2004; 127: 491–500.
Brighouse, D. & Guard, B.Anaesthesia for caesarean section in a patient with Ehlers-Danlos syndrome type IV. Br. J. Anaesth. 1992; 69: 517–19.
Dolan, P., Sisko, F. & Riley, E.Anesthetic considerations for Ehlers-Danlos syndrome. Anesthesiology 1980; 52; 266–9.
Goldstein, M. & Miller, R.Anesthesia for cesarean delivery in a patient with Ehlers-Danlos Syndrome. Type II. Reg. Anesth. 1997; 22: 280–3.
Glynn, J. C. & Yentis, S. M.Epidural analgesia in a parturient with classic type Ehlers-Danlos syndrome. Int. J. Obstet. Anesth. 2005; 14: 78–9.
Campbell, N. & Rosaeg, O. P.Anesthetic management of a parturient with Ehlers-Danlos syndrome type IV. Can. J. Anesth. 2002; 49: 493–6.
Dill-Russell, P. & Jones, John L.StAnaesthetic for caesarean section in a patient with Ehlers-Danlos syndrome and mitral valve prolapse. Int. J. Obstet. Anesth. 2001; 10: 192–7.
Kuczkowski, K. M. & Benumof, J. L.Cesarean section and Ehlers-Danlos syndrome: choice of anesthesia. Int. J. Obstet. Anesth. 2002; 11: 222–4.
Byers, P. H. & Steiner, R. D.Osteogenesis imperfecta. Ann. Rev. Med. 1992; 43: 269–82.
Rauch, F. & Glorieux, F. H.Osteogenesis imperfecta. Lancet 2004; 363: 1377–85.
McAllion, S. J. & Paterson, C. R.Musculo-skeletal problems associated with pregnancy in women with osteogenesis imperfecta. J. Obstet. Gynaecol. 2002; 22: 169–72.
Carlson, J. W. & Harlass, F. E.Management of osteogenesis imperfecta in pregnancy. J. Reprod. Med. 1993; 38: 228–32.
Sharma, A., George, L. & Erkine, K.Osteogenesis imperfecta in pregnancy: two case reports and review of literature. Obstet. Gynecol. Surv. 2001; 56: 563–6.
Cubert, R., Cheng, E. Y., Mack, S., Pepin, M. G. & Byers, P. H.Osteogenesis imperfecta: mode of delivery and neonatal outcome. Obstet. Gynecol. 2001; 97: 66–9.
Krishnamoorthy, U., Vausse, S. & Donnai, P.Management of pregnancy complicated by maternal osteogenesis imperfecta. Report of a case with uterine rupture. J. Obstet. Gynaecol. 2002; 22: 316.
Di Lieto, A., Pollio, F., Falco, al. Collagen content and growth factor immunoexpression in the uterine lower segment of type 1A osteogenesis imperfecta; relationship with recurrent uterine rupture in pregnancy. Am. J. Obstet. Gynecol. 2003; 189: 594–600.
Vogel, T. M., Ratner, E. F., Thomas, R. C. & Chitkara, U.Pregnancy complicated by severe osteogenesis imperfecta: a report of two cases. Anesth. Analg. 2002; 94: 1315–17.
Cho, E., Dayan, S. S. & Marx, G. F.Anaesthesia in a parturient with osteogenesis imperfecta. Br. J. Anaesth. 1992; 68: 422–3.
Yeo, S. T. & Paech, M. J.Regional anaesthesia for multiple caesarean sections in a parturient with osteogenesis imperfecta. Int. J. Obstet. Anesth. 1999; 8: 284–7.
Aly, E. E. & Harris, P.Spinal anesthesia in an obese patient with osteogenesis imperfecta. Can. J. Anaesth. 2003; 50: 421–2.
Porsborg, P., Astrup, G., Bendixen, D,Lund, A. M. & Ording, H.Osteogenesis imperfecta and malignant hyperthermia. Is there a relationship?Anaesthesia 1996; 51: 863–5.
Hathaway, W. E. & Solomons, C. C.Platelet function and pyrophosphates in osteogenesis imperfecta. Blood 1972; 39: 500–9.