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o o
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67. Pollard, B. J. & Young, T. M. Anaesthesia in myotonia dystrophica (letter). 96. Islander, G., Henriksson, K. G. & Ranklev-Twetman, E. Malignant
Anaesthesia 1989; 44: 699. hyperthermia susceptibility without central core disease (CCD) in a family
68. Mitchell, M. M., Ali, H. H. & Savarese, J. J. Myotonia and neuromuscular where CCD is diagnosed. Neuromuscul. Disord. 1995; 5: 125“7.
blocking agents. Anesthesiology 1978; 49: 44“8. 97. Gordon, C. P. & Litz, S. Multicore myopathy in a patient with anhidrotic
69. Castano, J. & Pares, N. Anaesthesia for major abdominal surgery in a patient ectodermal dysplasia. Can. J. Anaesth. 1992; 39: 966“8.
with myotonia dystrophica. Br. J. Anaesth. 1987; 59: 1629“31. 98. Heard, S. T. & Kaplan, R. F. Neuromuscular blockade in a patient with
70. Azar, I. The response of patients with neuromuscular disorders to muscle nemaline myopathy. Anesthesiology 1983; 59: 588“90.
relaxants: a review. Anesthesiology 1984; 61: 173“87. 99. Cunliffe, M. & Burrows, F. A. Anaesthetic implications of nemaline rod
71. Buzello, W., Krieg, N. & Schlickewei, A. Hazards of neostigmine in patients myopathy. Can. Anaesth. Soc. J. 1985; 32: 543“7.
with neuromuscular disorders. Report of two cases. Br. J. Anaesth. 1982; 54: 100. Asai, T., Fujise, K. & Uchida, M. Anaesthesia for cardiac surgery in children
529“34. with nemaline myopathy. Anaesthesia 1992; 47: 405“8.
72. Rosenberg, H. Neuromuscular diseases, myopathies and anesthesia. Curr. 101. Pourmand, R. & Azzarelli, B. Adult-onset of nemaline myopathy associated
Rev. Clin. Anesth. 1983; 3: 99“107. with cores and abnormal mitochondria. Muscle Nerve 1994; 17: 1218“20.
73. Britt, B. A. & Kalow, W. Malignant hyperthermia: a statistical review. Can. 102. Stackhouse, R., Chelmow, D. & Dattel, B. J. Anesthetic complications in a
Anaesth. Soc. J. 1970; 17: 293“315. pregnant patient with nemaline myopathy. Anesth. Analg. 1994; 79: 1195“7.
74. Brownell, A. K. W. Malignant hyperthermia: relationship to other diseases. 103. Gottschalk, A., Heiman-Patterson, T., Quevedo de, R. & Quinn, P. D.
Br. J. Anaesth. 1988; 60: 303“8. General anesthesia for a patient with centronuclear (myotubular) myo-
75. Lehmann-Horn, F. & Knorr-Held, S. Muscle diseases relevant to the anaes- pathy. Anesthesiology 1998; 89: 1018“20.
thetist. Acta Anaesthesiologica Belgica 1990; 41: 113“18. 104. Chitayat, D., Hodgkinson, K. A., Ginsburg, O., Dimmick, J. & Watters, G. V.
76. Wedel, D. J. Review article: malignant hyperthermia and neuromuscular King syndrome: a genetically heterogenous phenotype due to congenital
disease. Neuromusc. Disord. 1992; 2: 157“64. myopathies. A. J. Med. Genet. 1992; 43: 954“6.
77. Mudge. B. J., Taylor, P. B. & Vanderspek, A. F. Perioperative hazards in 105. Abel, D. E. & Grotegut, C. A. King syndrome in pregnancy. Obstet. Gynecol.
myotonic dystrophy. Anaesthesia 1980; 35: 492“5. 2003; 101 1146“8.
78. Moore, J. K. & Moore, A. P. Postoperative complications of dystrophia myo- 106. Habib, A. S., Millar, S., Deballi, P., 3rd & Muir, H. A. Anesthetic manage-
tonica. Anaesthesia 1987; 42: 529“33. ment of a ventilator-dependent parturient with the King-Deborough syn-
79. Branthwaite, M. A. Myotonic dystrophy and respiratory function (letter). drome. Can. J. Anesth. 2003; 50: 589“92.
Anaesthesia 1990; 45: 250“1. 107. Johnson, M. P., Compton, A., Drugan, A. & Evans, M. I. Metabolic control
80. Duncan, P. G. Neuromuscular diseases. In Katz, J. & Steward, D. J. (eds.), of Von Gierke disease (glycogen storage disease type 1A) in pregnancy:
Anesthesia and Uncommon Pediatric Diseases, 2nd edn. Philadelphia: W B maintenance of euglycemia with cornstarch. Obstet. Gynecol. 1990; 75:
Saunders Co, 1993; pp. 672“94. 507“10.

Chapter 5

108. Morgan-Hughes, J. A. The mitochondrial myopathies. In Engel, A. G. & 133. Lambert, C., Blanloeil, Y., Krivosic Horber, R. et al. Malignant hyperther-
Banker, B. Q. (eds.), Myology, Vol. II. New York: McGraw-Hill, 1986; mia in a patient with hypokalemic periodic paralysis. Anesth. Analg. 1994;
pp. 1709“43. 79: 1012“14.
109. Dubowitz, V. Metabolic myopathies II: lipids disorders mitochondrial 134. Dubowitz, V. Metabolic myopathies III: ion channel disorders. In Muscle
disorders. In Muscle Disorders in Childhood, 2nd edn. London: W B Disorders in Childhood, 2nd edn, London: W B Saunders Co, 1995;
Saunders Co, 1995; p. 211. pp. 266“314.
110. Schapira, A. H. Mitochondrial myopathies: mechanisms now better 135. Engel, A. G. Periodic paralysis. In Engel, A. G. & Banker, B. Q. (eds.),
understood (letter). Br. Med. J. 1989; 298: 1127“8. Myology, Vol. II, New York: McGraw-Hill, 1986; pp. 1843“70.
111. Boudin, G., Mikol, J., Guillard, A. et al. Fatal systemic carnitine deficiency 136. Bashford, A. C. Case report: anaesthesia in familial hypokalaemic periodic
with lipid storage in skeletal muscle, heart, liver and kidney. J. Neurol. Sci. paralysis. Anaesth. Intens. Care 1977; 5: 74“5.
1976; 30: 313“25. 137. Rooney, R. T., Shanahan, E. C., Sun, T. & Nally, B. Atracurium and hypoka-
112. Cornelio, F., Di Donato, S., Peluchetti, D. et al. Fatal cases of lipid storage lemic familial periodic paralysis. Anesth. Analg. 1988; 67: 782“3.
myopathy with carnitine deficiency. J. Neurol. Neurosurg. Psych. 1977; 40: 138. Fukuda, K., Ogawa, S., Yokozuka, H., Handa, S. & Nakamura, Y. Long-
170“8. standing bidirectional tachycardia in a patient with hypokalemic periodic
113. Angelini, C., Govoni, E., Bragaglia, M. M. & Vergani, L. Carnitine defi- paralysis. J. Electrocardiol. 1988; 21: 71.
ciency: acute postpartum crisis. Ann. Neurol. 1978; 4: 558“61. 139. Lema, G., Urzua, J., Moran, S. & Canessa, A. Successful anesthetic manage-
114. Rowe, R. W. & Helander, E. Anesthetic management of a patient with ment of a patient with hypokalemic familial periodic paralysis undergoing
systemic carnitine deficiency. Anesth. Analg. 1990; 71: 295“7. cardiac surgery. Anesthesiology 1991; 74: 373“5.
115. Beilin, B., Shulman, D. & Schiffman, Y. Anaesthesia in myopathy of carni- 140. Laurito, C. E., Becker, G. L. & Miller, P. E. Atracurium use in a patient with
tine deficiency. Anaesthesia 1986; 41: 92. familial periodic paralysis. J. Clin. Anesth. 1991; 3: 225“8.
116. Katsuya, H., Misumi, M., Ohtani, Y. & Miike, T. Postanesthetic acute renal 141. Neuman, G. G. & Kopman, A. F. P. Dyskalemic periodic paralysis and myo-
failure due to carnitine palmityl transferase deficiency. Anesthesiology tonia. Anesth. Analg. 1993; 76: 426“8.
1988; 68: 945“8. 142. Viscomi, C. M., Ptacek, L. J. & Dudley, D. Anesthetic management of famil-
117. Zierz, S. & Schmitt, U. Inhibition of carnitine palmitoyltransferase by ial hypokalemic periodic paralysis during parturition. Anesth. Analg. 1999;
Malonyl-CoA in human muscle is influenced by anesthesia. 88: 1081“2.
Anesthesiology 1989; 70: 373. 143. Johnstone, F. D. & Greer, I. A. Hyperkalaemic periodic paralysis and
118. Dreval, D., Bernstein, D. & Zakut, H. Carnitine palmitoyl transferase defi- HELLP syndrome: an unusual combination. Scot. Med. J. 1989; 34: 530“1.
ciency in pregnancy. A case report. Am. J. Obstet. Gynecol. 1994; 170: 1390“2. 144. Aarons, J. J., Moon, R. E. & Camporesi, E. M. General anesthesia and hyper-
119. Bonnefont, J. P., Gemaugre, F., Prip-Buus, C. et al. Carnitine palmitoyl- kalemic periodic paralysis. Anesthesiology 1989; 71: 303“4.
transferase deficiencies. Molecular Genetics and Metabolism 1990; 68: 145. Ashwood. E. M., Russell, W. J. & Burrow, D. D. Hyperkalaemic periodic
424“40. paralysis and anaesthesia. Anaesthesia 1992; 47: 579“84.
120. Moundras, J. M., Wattrisse, G., Leroy, B., Decocq, J. & Krivosic-Horber, R. 146. Farbu, E., Softeland, E. & Bindoff, L. A. Anaesthetic complications associated
Prise en charge anesth´ sique du travail obstetrical chez une parturiente
e with myotonia congenita: case study and comparison with other myotonic
atteinte d™un deficit musculaire en carnitine palmitoyl transf´ rase. Ann.
e disorders. Acta Anaesthesiol. Scand. 2003; 47: 630“4.
Fr. Anesth. Reanim. 2000; 19: 611“16. 147. Schwartz, I. L., Dingfelder, J. R., O™Tuama, L. & Swift, M. Recessive
121. Mardirosoff, C., Dumont, L., Cobin, L. & Massaut, J. Labour analgesia in a congenital myotonia and pregnancy. Int. J. Gynaecol. Obstet. 1979; 17:
patient with carnitine palmityl transferase deficiency and idiopathic 194“6.
thrombocytopenic purpura. Int. J. Obst. Anesth. 1997; 7: 134“6. 148. Ptacek, L. J., Ziter, F. A., Roberts, J. W. et al. Evidence of genetic
122. Stoelting, R. K. & Hillier, S. C. Pharmacology and Physiology in Anesthetic heterogeneity among the nondystrophic myotonias. Neurology 1992; 42:
Practice, 4th edn. Philadelphia, PA: Lippincott Williams and Wilkins, 2006, 1046“8.
p. 162. 149. Streib, E. W. Paramyotonia congenita. Semin. Neurol. 1991; 11: 249“57.
123. Salengos, J. C., Velghe-Lenelle, C. E., Bollens, R., Engelman, E. & Barvais, L. 150. De Silva, S. M., Kuncl, R. W., Griffin, J. W., Cornblath D. R. & Chavoustie, S.
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Anesthesiology 2004; 101: 241“3. electrophysiological features of each entity in one family. Muscle Nerve
124. Berkowitz, K., Monteagudo, A., Marks, F., Jackson, U. & Baxi, L. 1990; 13: 21“6.
Mitochondrial myopathy and preeclampsia associated with pregnancy. 151. Ptacek, J. J., Tarwil, R., Griggs, R. C., Storvick, M. S. & Leppert, M. Linkage of
Am. J. Obstet. Gynecol. 1990; 162: 146. atypical myotonia to a sodium channel locus. Neurology 1992; 42: 431“3.
125. Roseag, O. P., Morrison, S. & MacLeod, J. P. Clinical report: anaesthetic 152. Chitayat, D., Etchell, M. & Wilson, R. D. Cold-induced abortion in para-
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127. Russell, S. H. & Hirsch, N. P. Anaesthesia and myotonia. Br. J. Anaesth. nia congenita. Anaesth. Intensive Care 1999; 27: 534“7.
1994; 72: 210“16. 155. Catoggio, L. J. & Soriano, E. R. Inflammatory muscle disease: therapeutic
128. Allen, G. C. Malignant hyperthermia in musculoskeletal disorders. In aspects. Bailliere™s Clinical Rheumatology 2000; 14: 55“71.
Kirby, R. R. & Brown, D. L. (eds.), Problems in Anaesthesia, Vol. V, 156. Kofteridis, D. P., Malliotakis, P. I., Sotsiou, F. et al. Acute onset of derma-
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Andrea J. Fuller, Sheila E. Cohen, and Emily F. Ratner

Introduction It is possible for other hormonal deficiencies to be present
in conjunction with human growth hormone (hGH) deficiency
Dwarfism is defined as failure to achieve a height of 4 feet 10
(e.g. decreased levels of luteinizing hormone [LH], follicle stimu-
inches (148 cm) at adulthood.1 Short stature is a clinical entity
lating hormone [FSH], thyroid stimulating hormone [TSH], and
that has numerous etiologies. These conditions can be of genetic,
adrenocorticotropin hormone [ACTH] in panhypopituitary
constitutional, or metabolic origin. There are more than 100
patients), in which case puberty may not occur, making pregnancy
different types of dwarfism, none of which is very common. The
unlikely in these individuals.7 The other tropic hormones appear to
most common variety, achondroplasia, occurs in only 0.5 to 1.5 per
be normal in pregnant hGH-deficient patients.8
10 000 live births. 2,3 Two major classifications of dwarfism are
A common type of isolated growth hormone (GH) deficiency is
especially useful to the anesthesiologist: (1) patients with short
seen in ateliotic dwarfs, who carry an autosomal recessive gene.
stature who are proportionate and have normal trunk-to-limb ratio,
This gene causes a decrease in hGH production.9 Ateliotic dwarfs
and (2) patients who have disproportionate growth and have either
are typically of normal weight and length at birth, but over the
short trunks in relation to their limbs or short limbs in relation to
next few months of life have a much slower growth rate than
their trunks. (see Table 6.1).2 There are women at the lower extreme
unaffected infants. Their height rarely exceeds 130 cm, the height
of height in some cultures who would meet the definition of
of a normal eight-and-a-half year old.1 Those who achieve sexual
dwarfism but who have no medical pathology. Their treatment
maturation, known as sexual ateliotics, may not reach puberty
must be individualized bearing in mind that they may be similar
until the late second or third decade of life. In addition to having
in some respects to parturients with proportionate dwarfism.
short stature, these individuals have soft, prematurely wrinkled
Since the formation of the Little People of America, a society for
skin, high-pitched voices, and may have mild micrognathia.
people of short stature, and even dedicated online dating services,
Some patients also lack a normal lumbar lordosis.1,8
much greater opportunity exists for dwarfs to meet, socialize, and
Isolated GH deficiency is not usually diagnosed by random
eventually have children. In addition, increased use of assisted
blood samples of hGH alone, because these levels are normally
reproductive technology is enabling women of short stature who
low throughout most of the day. Provocative tests are more fre-
were otherwise infertile to achieve pregnancy and even multiple
quently used, including measurement of hGH after stimulation by
gestations. As a result, it is likely that more pregnant dwarfs will
exercise, L-dopa, insulin, arginine, clonidine, glucagon, or a com-
present for medical care in the future, often with comorbidities,
bination of these.7 Some GH-deficient dwarfs have abnormal glu-
high-risk multiple gestation pregnancies,4,5 and frequent need for
cose metabolism or responses to insulin, with glycosuria in early
cesarean delivery.
gestation progressing later to gestational diabetes.1 These patients
should be thoroughly investigated in the antenatal period to detect
and control any glucose perturbations during pregnancy.
Proportionate short stature
This category of dwarfism refers to those people who have short
Laron dwarfism
stature with proportionate trunk-to-limb length ratio. Individuals
may have proportionate short stature as a result of an endocrine This is an autosomal recessive disorder, which is clinically very
deficiency, metabolic disorder, or long-standing cardiac, renal, similar to isolated GH deficiency. However, Laron dwarfs have
neurologic, or gastrointestinal disease.6 Although some of these increased release but resistance to GH, very low serum levels of
patients are infertile or do not survive long enough to reach child- insulin-like growth factor (IGF-I), and an abnormal GH recep-
bearing age,6 those with an endocrinological etiology are fre- tor.10 In addition to having a normally proportioned trunk and
quently capable of becoming pregnant. Two endocrine disorders, extremities, these individuals may have frontal bossing, saddle
which cause proportionate short stature and may be seen in preg- nose, acromicria (hypoplasia of the extremities of the skeleton “
nancy, are isolated growth hormone deficiency and Laron dwarfism. the nose, jaws, fingers, and toes),11 a high-pitched voice, and slow
and sparse hair growth.12

Isolated growth hormone deficiency
Effects of pregnancy
Four types of this condition exist. These patients vary in terms
of mode of inheritance (autosomal dominant, autosomal reces- The normal physiologic changes of pregnancy can have serious
sive, X-linked) as well as in their degree of hormonal deficiency.7 implications for maternal health in proportionate dwarfs with the

Obstetric Anesthesia and Uncommon Disorders, eds. David R. Gambling, M. Joanne Douglas and Robert S. F. McKay. Published by Cambridge University Press.
# Cambridge University Press 2008.
2 Musculoskeletal disorders

Table 6.1 Classification of dwarfism

I. Proportionate short stature
A. Endocrine etiology
1. Isolated growth hormone deficiency
2. Laron dwarfism
B. Constitutional
C. Chronic disease states
II. Disproportionate short stature
A. Osteochondrodysplasias “ abnormalities of cartilage þ/À bone
growth and development
1. Achondroplasia
2. Pseudoachondroplasia
3. Spondyloepiphyseal dysplasia congenita
4. Spondyloepiphyseal dysplasia tarda
5. Spondylometaphyseal dysplasia
6. Diastrophic dwarfism
7. Osteogenesis imperfecta
B. Primary metabolic abnormalities
1. Calcium derangements
2. Phosphorous derangements
3. Complex carbohydrate derangements

Table 6.2 Vertical and horizontal abdominal measurements in
nonpregnant individuals (cm)

Achondroplasia Ateliotic Diastrophic Normal

Xiphoid to 29 25 24 33
Intercristal 21 24 25 30

SED ¼ Spondyloepiphyseal dysplasia
Reprinted with permission from Tyson, J. E., Barnes, A. C., McKusick,
V. A. et al. Obstetric and gynecologic considerations of dwarfism.
Am. J. Obstet. Gynecol. 1970; 108: 688“703.

most concerning cardiorespiratory embarrassment. The abdom- Figure 6.1 Patient L. L. An ateliotic dwarf in the 28th week of gestation.
inal dimensions of these patients are markedly smaller than their Reprinted with permission from C. V. Mosby Co. from Tyson, J. E., Barnes, A. C.,
normal-sized counterparts. For example, the average distance McKusick, V. A. et al. Obstetric and gynecologic considerations of dwarfism.
in nongravid individuals from xiphoid to symphysis is significantly Am. J. Obstet. Gynecol. 1970; 108: 688“703.
less in ateliotic dwarfs than in normal-sized patients (see Table 6.2).
The uterus therefore becomes an intra-abdominal organ earlier in
gestation (see Figure 6.1). These factors may cause significant
Obstetric management
mechanical diaphragmatic dysfunction and greater aortocaval
compression. The decrease in functional residual capacity (FRC) Some authors believe that almost all pregnant patients of short
stature should be delivered by cesarean section (C/S).14 Others
that normally occurs during pregnancy causes additional respira-
tory compromise.13 Pregnant dwarfs may suffer from decreased report ateliotic patients who have had successful vaginal deliver-
ies but no details are available.1 The pelvic diameters of such
vital capacity, hypoxia, and acidosis, and develop tachypnea and
tachycardia in order to compensate for these physiologic derange- patients are typically smaller than those of parturients of normal
ments. Deterioration of pulmonary status may warrant immediate stature and are frequently only sufficient for delivery of a prema-
delivery of the fetus in order to improve maternal condition.1 ture infant.1 Cesarean section is thus performed frequently for

Chapter 6

cephalopelvic disproportion as well as to relieve maternal cardio-
Table 6.3 Anesthetic considerations for proportionate dwarfs
pulmonary embarrassment.
Other complications of pregnancy include higher than normal Dwarfism characteristics Anesthetic implications
rates of spontaneous abortion, stillbirth, and premature delivery.1
Upper airway
It is unclear whether these complications result from a mechan-
Micrognathia possible Possible difficult intubation
ical, genetic, or hormonal problem; however, premature delivery
Smaller airway May need smaller ETT
is more likely when maternal respiratory embarrassment is pre-
Cervical spine High incidence of spinal stenosis
sent.1 It has been suggested that preterm delivery16 may be due to
Possible atlantoaxial instability
some unknown physiological actions of GH in the first half of
Short stature Decreased dosage of local
pregnancy, prior to placental production of a variant type of
anesthetic for regional
GH.15 Additionally, there is a higher incidence of premature
birth secondary to maternal indications.1
Continuous regional technique is
Anesthetic considerations (see Table 6.3) Small pelvis High rate of cesarean section
Uterine impingement on Respiratory compromise
One case report describes a patient with pituitary dwarfism who
intrathoracic structures May not tolerate supine position or
was 124 cm tall and weighed 35 kg at 36 weeks™ gestation.16
rapid i.v. fluid administration
Because of anticipated fetal weight of almost 3000 g at that time,
Uterine impingement on Risk of supine hypotension
she had a planned C/S under epidural anesthesia at 39 weeks™
abdominal structures Risk of aspiration
gestation. The epidural catheter was placed easily and she received
12 ml of 2% lidocaine with 1:200 000 epinephrine and 50 mg of
fentanyl to achieve a T3 sensory level. Her perioperative course
was uneventful and she received 2 mg epidural morphine for post- slow titration of local anesthetic to provide adequate neural block-

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