Cesarean section by local anesthesia in patients with familial dysautonomia Joseph R. Leiberman, MD: Adolph Cohen, MD: Arnon Wiznitzer, MD: Channa Maayan, MD,b and Lev Greemberg, MDc Beer Sheva, Jerusalem, Israel We describe a 29-year-old patient with familial dysautonomia who underwent cesarean section because of severe intrauterine fetal growth retardation. The sCirgery was done after induction of local anesthesia to avoid the critical and sometimes fatal complications of general anesthesia known in patients with familial dysautonomia. Surgery was uneventful and almost painless. The postoperative period was without complications. Induction of local anesthesia for cesarean section may constitute a suitable alternative in patients with familial dysautonomia. (AM J OBSTET GVNECOL 1991 ;165:110-1.)
Key words: Familial dysautonomia, Riley-Day syndrome, anesthesia Familial dysautonomia or Riley-Day syndrome is a rare, autosomal recessive genetic disorder found in the Ashkenazi Jewish population that affects the development of the autonomic and sensory nervous systems. Characteristic signs and symptoms include profuse sweating, vomiting, lack of overflow tears, lung infections after frequent episodes of aspirative pneumonia, scoliosis, cardiovascular instability, impaired heat perception and taste, and decreased sensation of pain.' In the past few patients survived long enough to become pregnant; therefore there is little experience with delivery reported in the medical literature. I. 2 Anesthesia for surgical delivery in these patients presents particular problems. Increased morbidity and mortality were found in subjects with familial dysautonomia who required induction of general anesthesia, and dehydration, dysautonomic crisis, intraoperative severe hypotension, and postoperative atelectasis were frequently described! Extradural anesthesia or spinal block are not adequate for the blood pressure lability. We present a patient with familial dysautonomia who was abdominally delivered of an infant after induction of local anesthesia to avoid the risks of the other anesthetic techniques.
Case report A 29-year-old Jewish nulliparous patient with familial dysautonomia was first seen at 8 weeks' gestation. She From the Division of Obstetrics and Gynecologya and the Division of Anesthesia,' Soroka Medical Center, Ben Gurion University, and the Department of Pediatrics/ Hadassah Universi(v Hospital, Mount Scopus. Received for publication january 2, 1991; accepted january 28,
1991.
Reprint requests: joseph R. Leiberman, MD, Soroka Medical Center, Division of Obstetrics and Gynecology, POB 151, Beer Sheva, Israel 84101.
6/1/28312
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worked as a volunteer in one of the hospital offices. Her previous history and signs revealed convulsive disorders during the school years, growth retardation, vomiting, episodes of lung infection, lack of fungiform papillae on the tongue, and dry eyes, all of which indicated mild familial dysautonomia. Three years before pregnancy a chest roentgenogram was normal. The final diagnosis of familial dysautonomia was made by a pathologic histamine test. The patient was pale; height was 145 em and weight was 40 kg. She had speech difficulty, infrequent heavy coughing, and postural hypotension. Blood pressure measurements were 150/90 mm Hg while supine and 75/40 mm Hg while standing. Previous gynecologic anamnesis was unremarkable. Menses started at the age of 12 years and were regular (4 to 5 days every 30 days). Vomiting during pregnancy was infrequent. Successive control visits revealed a normally developed pregnancy. Hemoglobin level was 11 gm/ dl, and the patient took multivitamin and mineral capsules. Amniocentesis was done at 16 weeks' gestation and revealed normal fetal karyotype and amniotic fluid level of u-fetoprotein. Several episodes of hypotension occurred at home on hot days and prompted hospitalization. Biochemical tests and arterial blood gas values were in the normal range. Simple hydration was enough to restore blood pressure and a well feeling. At 34 weeks' gestational age, fetal growth retardation was established sonographically. Fetal weight was calculated as 1520 gm (less than the 10th percentile). Nonstress test revealed a normal fetal heart rate. At 36 weeks' gestation severe fetal deterioration was diagnosed and we decided to terminate the pregnancy abdominally. In light of the serious risk of induction of general anesthesia in these patients, cesarean section after induction of local anesthesia was suggested. A team of anesthetists was ready at the surgical theater to induce immediate general anesthesia if required. Local anesthesia was induced by 1% lidocaine hydrochloride solution. Subcutaneous infiltration was
Familial dysautonomia
Volume 165 Number 1
made at both sides of a longitudinal infraumbilical incision. Subsequently, every layer was infiltrated. The abdominal wall was opened almost without any complaints by the patient. The approach to the uterus was slightly painful. The anesthetic agent was injected into the myometrium before it was incised transversally in the low segment. At this time the patient's talk showed confusion for a few minutes. A living female infant was delivered through the placenta with Apgar scores of 8 at 5 minutes and 10 at 10 minutes. The infant birth weight was 1630 gm, and results of the physical examination were normal. After extraction of the placenta, suture of the uterine wall required administration of additional local anesthetic agent. Closure of the abdominal wall was performed as usual. For the skin suture lidocaine was added. At the end of the operation the patient was happy and content. The postoperative period was simple and uneventful.
Comment Familial dysautonomia (Riley-Day syndrome) is a rare disease. The experience with pregnant patients is sparse and only two deliveries by cesarean section were reported in the literature,2 both of which were performed after induction of general anesthesia. Familial dysautonomia constitutes an anesthetic hazard and increased morbidity and mortality were reported. Cardiovascular lability, respiratory depression, and atelectasis, are grave complications described in surgery with induction of general anesthesia. Adequate hydration and careful titration of anesthetic agents were recently recommended to minimize intraoperative and postoperative complications. 2 Although attractive, neither extradural nor spinal block is the prefered method
111
to induce anesthesia in patients with dysautonomia because of cardiovascular instability. In our patient the difficulties and risks of induction of general anesthesia were obviated by induction of local anesthesia. Induction of local anesthesia by infiltration is a useful technique that may be indicated in high-risk patients. Cesarean section was performed without trouble with lidocaine 1%, a rather high concentration, as a local anesthetic agent. Although lidocaine by intravenous route is commonly used at the dose of 1 to 5 mg/kg, higher doses may be considered when other means of administration are used. The main adverse effects of lidocaine are on the central nervous system, and it may be that the slight confusion of the patient during surgery was a result of the anesthetic agent. Because a large quantity of anesthetic solution is needed, it may be recommended in future cases to use lidocaine solution 0.5% with epinephrine to slow absorption and reduce untoward effects. Because of greater understanding of the disease and better treatment today, more patients with familial dysautonomia reach adulthood and can get pregnant. It is our conclusion that when cesarean section is required, induction of local anesthesia may constitute a prefered alternative in these patients. REFERENCES 1. Porges RF, Axelrod FB, Richards M. Pregnancy in familial dysautonomia. AM] OBSTET GYNECOL 1978;132;485-8. 2. Axelrod FB, Donenfeld RF, Danziger F, Turndorf H. Anesthesia in familial dysautonomia. Anesthesiology 1988;68: 631-5.
Key words: Familial dysautonomia, Riley-Day syndrome, anesthesia Familial dysautonomia or Riley-Day syndrome is a rare, autosomal recessive genetic disorder found in the Ashkenazi Jewish population that affects the development of the autonomic and sensory nervous systems. Characteristic signs and symptoms include profuse sweating, vomiting, lack of overflow tears, lung infections after frequent episodes of aspirative pneumonia, scoliosis, cardiovascular instability, impaired heat perception and taste, and decreased sensation of pain.' In the past few patients survived long enough to become pregnant; therefore there is little experience with delivery reported in the medical literature. I. 2 Anesthesia for surgical delivery in these patients presents particular problems. Increased morbidity and mortality were found in subjects with familial dysautonomia who required induction of general anesthesia, and dehydration, dysautonomic crisis, intraoperative severe hypotension, and postoperative atelectasis were frequently described! Extradural anesthesia or spinal block are not adequate for the blood pressure lability. We present a patient with familial dysautonomia who was abdominally delivered of an infant after induction of local anesthesia to avoid the risks of the other anesthetic techniques.
Case report A 29-year-old Jewish nulliparous patient with familial dysautonomia was first seen at 8 weeks' gestation. She From the Division of Obstetrics and Gynecologya and the Division of Anesthesia,' Soroka Medical Center, Ben Gurion University, and the Department of Pediatrics/ Hadassah Universi(v Hospital, Mount Scopus. Received for publication january 2, 1991; accepted january 28,
1991.
Reprint requests: joseph R. Leiberman, MD, Soroka Medical Center, Division of Obstetrics and Gynecology, POB 151, Beer Sheva, Israel 84101.
6/1/28312
110
worked as a volunteer in one of the hospital offices. Her previous history and signs revealed convulsive disorders during the school years, growth retardation, vomiting, episodes of lung infection, lack of fungiform papillae on the tongue, and dry eyes, all of which indicated mild familial dysautonomia. Three years before pregnancy a chest roentgenogram was normal. The final diagnosis of familial dysautonomia was made by a pathologic histamine test. The patient was pale; height was 145 em and weight was 40 kg. She had speech difficulty, infrequent heavy coughing, and postural hypotension. Blood pressure measurements were 150/90 mm Hg while supine and 75/40 mm Hg while standing. Previous gynecologic anamnesis was unremarkable. Menses started at the age of 12 years and were regular (4 to 5 days every 30 days). Vomiting during pregnancy was infrequent. Successive control visits revealed a normally developed pregnancy. Hemoglobin level was 11 gm/ dl, and the patient took multivitamin and mineral capsules. Amniocentesis was done at 16 weeks' gestation and revealed normal fetal karyotype and amniotic fluid level of u-fetoprotein. Several episodes of hypotension occurred at home on hot days and prompted hospitalization. Biochemical tests and arterial blood gas values were in the normal range. Simple hydration was enough to restore blood pressure and a well feeling. At 34 weeks' gestational age, fetal growth retardation was established sonographically. Fetal weight was calculated as 1520 gm (less than the 10th percentile). Nonstress test revealed a normal fetal heart rate. At 36 weeks' gestation severe fetal deterioration was diagnosed and we decided to terminate the pregnancy abdominally. In light of the serious risk of induction of general anesthesia in these patients, cesarean section after induction of local anesthesia was suggested. A team of anesthetists was ready at the surgical theater to induce immediate general anesthesia if required. Local anesthesia was induced by 1% lidocaine hydrochloride solution. Subcutaneous infiltration was
Familial dysautonomia
Volume 165 Number 1
made at both sides of a longitudinal infraumbilical incision. Subsequently, every layer was infiltrated. The abdominal wall was opened almost without any complaints by the patient. The approach to the uterus was slightly painful. The anesthetic agent was injected into the myometrium before it was incised transversally in the low segment. At this time the patient's talk showed confusion for a few minutes. A living female infant was delivered through the placenta with Apgar scores of 8 at 5 minutes and 10 at 10 minutes. The infant birth weight was 1630 gm, and results of the physical examination were normal. After extraction of the placenta, suture of the uterine wall required administration of additional local anesthetic agent. Closure of the abdominal wall was performed as usual. For the skin suture lidocaine was added. At the end of the operation the patient was happy and content. The postoperative period was simple and uneventful.
Comment Familial dysautonomia (Riley-Day syndrome) is a rare disease. The experience with pregnant patients is sparse and only two deliveries by cesarean section were reported in the literature,2 both of which were performed after induction of general anesthesia. Familial dysautonomia constitutes an anesthetic hazard and increased morbidity and mortality were reported. Cardiovascular lability, respiratory depression, and atelectasis, are grave complications described in surgery with induction of general anesthesia. Adequate hydration and careful titration of anesthetic agents were recently recommended to minimize intraoperative and postoperative complications. 2 Although attractive, neither extradural nor spinal block is the prefered method
111
to induce anesthesia in patients with dysautonomia because of cardiovascular instability. In our patient the difficulties and risks of induction of general anesthesia were obviated by induction of local anesthesia. Induction of local anesthesia by infiltration is a useful technique that may be indicated in high-risk patients. Cesarean section was performed without trouble with lidocaine 1%, a rather high concentration, as a local anesthetic agent. Although lidocaine by intravenous route is commonly used at the dose of 1 to 5 mg/kg, higher doses may be considered when other means of administration are used. The main adverse effects of lidocaine are on the central nervous system, and it may be that the slight confusion of the patient during surgery was a result of the anesthetic agent. Because a large quantity of anesthetic solution is needed, it may be recommended in future cases to use lidocaine solution 0.5% with epinephrine to slow absorption and reduce untoward effects. Because of greater understanding of the disease and better treatment today, more patients with familial dysautonomia reach adulthood and can get pregnant. It is our conclusion that when cesarean section is required, induction of local anesthesia may constitute a prefered alternative in these patients. REFERENCES 1. Porges RF, Axelrod FB, Richards M. Pregnancy in familial dysautonomia. AM] OBSTET GYNECOL 1978;132;485-8. 2. Axelrod FB, Donenfeld RF, Danziger F, Turndorf H. Anesthesia in familial dysautonomia. Anesthesiology 1988;68: 631-5.
