Aortic dissection and rupture in Turner syndrome

1
6 7 0 Editorial correspondence The Journal of Pediatrics April 1993 2. Marchant CD, Shurin PA, Johnson CE, et al. A randomized controlled trial of amoxicillin plus clavulanate compared with cefaclor for treatment of acute otitis media. J PEDIATR 1986;109:891-5. 3. Johnson CE, Carlin SA, Super DM, et al. Cefixime compared with amoxicillin for treatment of acute otitis media. J PEDZA'rR 1991;119:117-22. Reply To the Editor: The outcome definitions used in our study, as stated in the Methods section, defined bacteriologic cure as eradication of initial pathogens by tympanocentesis or by resolution of symptoms and a return of the tympanic membrane to normal. These determinations were made in a blinded fashion. Thus, although it is correct to state that bacterial eradication was often a clinical diagnosis, it would seem difficult to defend performing a tympanocentesis at day 10 to 14 on a child with no symptoms (such as fever, irritability, or ear pain) and a normal-appearing tympanic membrane. The authors cited by Dr. Feder did perform a second tympano- centesis on day 3 to 6 of therapy, a time when the tympanic mem- brane would usually still be abnormal in appearance, with fluid present. A second tympanocentesis may be justifiable in a study protocol to determine bacteriologic efficacy when only 30% to 60% of the antibiotic therapy had been completed, ttowever, these two studies do not establish follow-up tympanocentesis as a standard for bacteriologic cure or failure after 10 days of therapy. Mark A. Del Beccaro, MD Paul )lL Mendelman, )liD Samuel E. McLinn, )liD Wesley 3L Todd, )lid Children's Hospital and Medical Center Seattle, IVA 98105 9/35/44793 Aortic dissection and rupture in Turner syndrome To the Editor: Two previous articles in THE JOURNAL I~ 2 indicated an increased risk of aortic dissection in Turner syndrome. These cases occur largely in patients with coarctation of the aorta, bicuspid aortic valve or regurgitation or both, aortic root dilation, and systemic hypertension. Of two cases reported that described none of these associated risk factors, documentation was inadequate. Here, I re- port a 20-year-old woman with 45, X Turner syndrome who died suddenly because of aortic dissection and in whom all these risk factors were adequately assessed but not found. Results of initial cardiac evaluation were normal at the time of diagnosis, at age I 1 years, and estrogen therapy was started at age 13 years. At 19 years of age, a two-dimensional echocardiogram and Doppler examination showed a normal aortic valve and aortic root size. The patient was normotensive despite progressive obesity. Seven days before her death, chest pain and dyspnea suddenly de~ veloped in association with mild diarrhea, diaphoresis, and pallor. She was observed for several hours in a local emergency department until she was stable, and then was discharged home with the diag- nosis of acute viral gastroenteritis. Seven days later, the patient awoke, complained of a headache, and died suddenly. Postmortem examination revealed a ruptured aorta 1 cm distal to a normal aor- tic valve, with dissection and cardiac tamponade. Histologie exam- ination of the ruptured aorta showed cystic medial necrosis. This additional case report underscores our obligation to alert all patients with Turner syndrome, their families, and physicians of the significance of chest pain as a possible manifestation of aortic dis- section. I propose the development of a national Turner syndrome registry for the reporting of medical events. The information obtained would enable us to develop appropriate guidelines for medical and cardiac follow-up care. Karen Rubin, )liD Department of Pediatrics University of Connecticut Health Center Farmington, CT 06030 9/35]44796 REFERENCES 1. Strader W J, Wachtel HL, Lundberg GD Jr. Hypertension and aortic rupture in gonadal dysgenesis. J PEDIATR 197 I;79:473-5. 2. Lin AE, Lippe BM, Geffner ME, et al. Aortic dilation, dissec- tion, and rupture in patients with Turner syndrome. J PEDIA'I'R 1986;109:820-6. Reply To the Editor: Dr. Rubin's patient represents another tragic example of dissec- tion of the aorta in an otherwise healthy woman with Turner syn- drome. Since our report we have had another patient with unrec- ognized aortic dissection. She was a 34-year-old 45,X individual who had mild hypertension as her only risk factor for dissection. At presentation in an emergency department, she had chest pain, cough, and diaphoresis. Because of a history of possible aspiration of a peanut several days previously, the diagnosis of possible pul- monary atelectasis was made and She was sent home. She died less than 24 hours later. Dr. Rubin's call for greater recognition of this possible compli- cation is appropriate, albeit not new; we made the same plea in 1972. I Now that the true incidence of Turner syn'drome is recog- nized to be as high as 1 in 2000 live female births and more girls with short stature are being examined with a karyotype, I agree that it is incumbent on all health care providers to recognize this poten- tially treatable acute event. Barbara Lippe, )liD Professor of Pediatrics Chief, Division of Endocrinology University of California )lIedieal Center Los Angeles, CA 90024-1752 9/35/44795 REFERENCE 1. Lippe BM, Kogut MD. Aortic rupture in gonadal dysgenesis [Letter]. J PEDIA'rR 1972;80;895.

Transcript of Aortic dissection and rupture in Turner syndrome

6 7 0 Editorial correspondence The Journal of Pediatrics April 1993

2. Marchant CD, Shurin PA, Johnson CE, et al. A randomized controlled trial of amoxicillin plus clavulanate compared with cefaclor for treatment of acute otitis media. J PEDIATR 1986;109:891-5.

3. Johnson CE, Carlin SA, Super DM, et al. Cefixime compared with amoxicillin for treatment of acute otitis media. J PEDZA'rR 1991;119:117-22.

Reply

To the Editor: The outcome definitions used in our study, as stated in the

Methods section, defined bacteriologic cure as eradication of initial pathogens by tympanocentesis or by resolution of symptoms and a return of the tympanic membrane to normal. These determinations were made in a blinded fashion. Thus, although it is correct to state that bacterial eradication was often a clinical diagnosis, it would seem difficult to defend performing a tympanocentesis at day 10 to 14 on a child with no symptoms (such as fever, irritability, or ear pain) and a normal-appearing tympanic membrane.

The authors cited by Dr. Feder did perform a second tympano- centesis on day 3 to 6 of therapy, a time when the tympanic mem- brane would usually still be abnormal in appearance, with fluid present. A second tympanocentesis may be justifiable in a study protocol to determine bacteriologic efficacy when only 30% to 60% of the antibiotic therapy had been completed, ttowever, these two studies do not establish follow-up tympanocentesis as a standard for bacteriologic cure or failure after 10 days of therapy.

Mark A. Del Beccaro, MD Paul )lL Mendelman, )liD

Samuel E. McLinn, )liD Wesley 3L Todd, )lid

Children's Hospital and Medical Center Seattle, IVA 98105

9/35/44793

Aortic dissection and rupture in Turner syndrome

To the Editor: Two previous articles in THE JOURNAL I~ 2 indicated an increased

risk of aortic dissection in Turner syndrome. These cases occur largely in patients with coarctation of the aorta, bicuspid aortic valve or regurgitation or both, aortic root dilation, and systemic hypertension. Of two cases reported that described none of these associated risk factors, documentation was inadequate. Here, I re- port a 20-year-old woman with 45, X Turner syndrome who died suddenly because of aortic dissection and in whom all these risk factors were adequately assessed but not found.

Results of initial cardiac evaluation were normal at the time of diagnosis, at age I 1 years, and estrogen therapy was started at age 13 years. At 19 years of age, a two-dimensional echocardiogram and Doppler examination showed a normal aortic valve and aortic root size. The patient was normotensive despite progressive obesity.

Seven days before her death, chest pain and dyspnea suddenly de~ veloped in association with mild diarrhea, diaphoresis, and pallor. She was observed for several hours in a local emergency department until she was stable, and then was discharged home with the diag- nosis of acute viral gastroenteritis. Seven days later, the patient awoke, complained of a headache, and died suddenly. Postmortem examination revealed a ruptured aorta 1 cm distal to a normal aor- tic valve, with dissection and cardiac tamponade. Histologie exam- ination of the ruptured aorta showed cystic medial necrosis.

This additional case report underscores our obligation to alert all patients with Turner syndrome, their families, and physicians of the significance of chest pain as a possible manifestation of aortic dis- section. I propose the development of a national Turner syndrome registry for the reporting of medical events. The information obtained would enable us to develop appropriate guidelines for medical and cardiac follow-up care.

Karen Rubin, )liD Department of Pediatrics

University of Connecticut Health Center Farmington, CT 06030

9/35]44796

REFERENCES

1. Strader W J, Wachtel HL, Lundberg GD Jr. Hypertension and aortic rupture in gonadal dysgenesis. J PEDIATR 197 I;79:473-5.

2. Lin AE, Lippe BM, Geffner ME, et al. Aortic dilation, dissec- tion, and rupture in patients with Turner syndrome. J PEDIA'I'R 1986;109:820-6.

Reply

To the Editor: Dr. Rubin's patient represents another tragic example of dissec-

tion of the aorta in an otherwise healthy woman with Turner syn- drome. Since our report we have had another patient with unrec- ognized aortic dissection. She was a 34-year-old 45,X individual who had mild hypertension as her only risk factor for dissection. At presentation in an emergency department, she had chest pain, cough, and diaphoresis. Because of a history of possible aspiration of a peanut several days previously, the diagnosis of possible pul- monary atelectasis was made and She was sent home. She died less than 24 hours later.

Dr. Rubin's call for greater recognition of this possible compli- cation is appropriate, albeit not new; we made the same plea in 1972. I Now that the true incidence of Turner syn'drome is recog- nized to be as high as 1 in 2000 live female births and more girls with short stature are being examined with a karyotype, I agree that it is incumbent on all health care providers to recognize this poten- tially treatable acute event.

Barbara Lippe, )liD Professor of Pediatrics

Chief, Division of Endocrinology University of California )lIedieal Center

Los Angeles, CA 90024-1752 9/35/44795

REFERENCE

1. Lippe BM, Kogut MD. Aortic rupture in gonadal dysgenesis [Letter]. J PEDIA'rR 1972;80;895.