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Bilateral eyelid ecchymosis and subconjunctival hemorrhage associated with coughing paroxysms in pertussis infection
Bilateral Eyelid Ecchymosis and Subconjunctival Hemorrhage Associated With Coughing Paroxysms in Pertussis Infection E v e l y n A. Paysse, M D , a n d David K. Coats, M D
p
ertussis (whooping cough) has been uncommon in the United States during the past 45 years as a result of effective immunization programs. Since the early 1980s, however, a resurgence of the disease has been noted (Figure 1). The incidence of reported cases of pertussis in the United States has cyclically increased for unknown reasons every 3 to 4 years. The last peak was in 1993, in which 6586 cases were reported. 1 Even today whooping cough is responsible for a significant number of childhood deaths worldwide, especially in areas where poor immunization programs exist. Whooping cough is an acute respiratory illness that most often affects infants and young children. Premature infants are at higher risk of contracting the disease. The etiologic agent is usually Bordetella pertussis, occasionally Bordetella parapertussis, and rarely Bordetella bronchioseptica. Whooping cough is typically divided into three stages, each lasting about 14 days. The catarrhal stage begins after an incubation period of 7 to 16 days. Symptoms are initially indistinguishable from a mild upper respiratory infection. Sneezing, mild fever, and nocturnal cough are present. Seven to 14 days later the paroxysmal stage begins. The cough becomes the most prominent feature and is more frequent, diurnal, and paroxysmal during this stage. A typical coughing paroxysm starts with a series of 15 to 20 short coughs, each cough more intense than the last. The paroxysm is then followed by a deep inspiratory effort, producing the characteristic "whoop." A thick mucus plug may be expelled, not uncommonly followed by emesis. The convalescent stage follows 14 days later. Gradually the coughing paroxysms become less frequent and less
From the Cullen Eye Institute, Baylor College of MedMne, Texas Children's Hospital, Houston, Texas. Supported in part by an unrestrictedgrantfrom Research to Prevent Blindness, Inc., New York. Submitted February 27, 199Z Revision accepted October 13, 199Z J AAPOS 1998;2:116-9. Reprint requests: Evelyn A. Paysse, MD, Baylor College of Medicine, Texas Children's Hospital, 1102 Bates, #300, Houston, T X 77030. Copyright © 1998 by the American Assodationfor Pediatric Ophthalmology and Strabismus. 1091-8531/98 $5.00 + 0 75/1/87499
116
April 1998
intense until they cease altogether. In very young infants, the paroxysms and whoop are often not present, replaced instead by choking spells and apneic episodes. 2 Infants may develop seizures and an acute encephalopathy. 3 Treatment for whooping cough varies depending on the severity of the disease. Several antibiotics are effective, including systemic erythromycin and oxytetracycline (in children older than 8 years). The organism is killed within a few days of initiation of antibiotic treatment. Relapse, however, is frequent, and for this reason the antibiotic regimen should be continued for 14 days (erythromycin) or 14 to 21 days (oxytetracycline). Supportive care to maintain proper fluid and electrolyte balance, nutrition, and oxygenation are of obvious importance. Ophthalmologic complications in pertussis occur infrequently. The only reports of eye findings in pertussis are from the early 1900s and include retinal detachment, ophthalmoplegia, and hemorrhages of the subconjunctival space, retina, choroid, anterior chamber, and orbit. 4-6 Ocular and periocular hemorrhages therefore are generally not a well-known finding of the disease. CASE R E P O R T S Patient 1
A 5-year-old girl was referred by her pediatrician for evaluation of severe, progressive, painless right upper and lower eyelid ecchymoses and subconjunctival hemorrhage. She had had severe nocturnal coughing paroxysms for the last several weeks and had lost 2 pounds in the 2 weeks before our evaluation. There was no history of trauma, coagulopathy, blood dyscrasias, or other medical problems. Before onset of this illness, she had been in good health. The patient denied ocular pain, discharge, photophobia, headache, or other systemic symptoms. The recommended total of five doses of diphtheria-pertussis-tetanus (DPT) vaccine had been received on the recommended schedule. Her last D P T vaccine was at age 5 years. A complete ophthalmologic examination was performed. Findings included severe fight upper and lower eyelid ecchymoses and subconjunctival hemorrhage. Two millimeters of proptosis of the fight eye was measured. The remainder of ffournal of AAPOS
Journal of AAPOS Volume 2 Number 2 April 1998
eca
=.,I
REPORTED CASES OF PERTUSSIS United States, 1965-1995
8/
10
"O
Paysse and Coats 117
6
O
4 G)
O
2 0
t 1965
I
I
I
I
I
1970
1975
1980 Year
1965
1990
1995
FIG. 1. Reported cases of pertussis in United States, 1965 to 1995. Note cyclic increase in reported cases every 3 to 4 years. (From Centers for Disease Control and Prevention. MMWR Morbid Mortal Wkly Rep 1995;9:46.)
FIR. 3. Patient 2, with resolving subconjunctival hemorrhage and lid ecchymoses.
TABLE. Etiology of subconjunctival and periorbital hemorrhage in infants and children. Hemophilia Anticoagulant therapy Coagulopathies Thromboccytopenia Trauma (occult or known) Rhabdomyosarcoma Metastatic neuroblastoma Leukemia Pertussis
junctival hemorrhages cleared rapidly after resolution of the coughing paroxysms. Patient 2
FIB. 2. Patient 1, with severe lid ecchymoses and subconjunctival hemorrhage.
her ophthalmologic examination, including visual acuity, motility evaluation, pupillary testing, and indirect ophthalmoscopy, was normal. Two days after the initial ophthalmologic examination, severe upper and lower eyelid ecchymoses and subconjunctival hemorrhages developed in the uninvolved left eye (Figure 2). A sodal services consult was called to investigate the social situation, and no evidence of intentional trauma or abuse was found. Orbital computed tomography was negative for neoplasm. Orbital soft tissue swelling was noted, accounting for the proptosis of the right eye. The white blood cell count was elevated at 14,800/btl with 68% 1ymphocytes. The platelet count was normal at 313,000/btl. B. pertussis was cultured from a nasopharyngeal swab. The patient was treated with oral erythromycin for 2 weeks, and the illness quickly subsided. The lid ecchymoses and subcon-
A 9-year-old boy was referred by his pediatrician for a 1week history of severe bilateral lid ecchymoses and subconjunctival hemorrhage. The medical history was significant for a coughing illness of more than 3 weeks' duration. There was a prominent history of severe nocturnal coughing paroxysms. The patient denied a history of ocular discharge, photophobia, pain, decreased vision, diplopia, or other symptoms. Medical history was significant for sickle cell anemia. The patient was status post splenectomy. He had received all five scheduled pertussis vaccinations (DPT), including his most recent one at age 4 years. Comprehensive ophthalmologic examination was notable for moderate bilateral upper and lower eyelid ecchymoses and severe bilateral subconjunctival hemorrhages (Figure 3). The remainder of the ophthalmologic examination was normal. A nasopharyngeal pertussis culture was negative. The pertussis immunoglobulin G (IgG) antibody level was elevated at 81 (normal <10) and the permssis immunoglobulin A (IgA) antibody level was elevated at 41 (normal <15), both highly suggestive of a recent permssis infection. Pertussis immunoglobulin M was not detected. Complete blood cell count revealed
118
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an elevated total white blood cell count of 14,800/gl with 52% lymphocytes. The platelet count was 307,000/gl. The patient was treated with oral erythromycin for 2 weeks and the cough, lid ecchymoses, and subconjunctival hemorrhages quickly resolved.
DISCUSSION Pertussis was a major cause of morbidity and mortality among infants and children in the United States in the prevaccine era (before the 1940s). The highest incidence of pertussis cases in this country was approximately 260,000 reported cases in 1934.1 Nine thousand pertussisrelated deaths were reported in 1923, the highest number ever reported. Since the introduction of the pertussis vaccine in 1949, the number of pertussis cases has markedly declined. In 1976 only 1010 cases were reported to the Centers for Disease Control and Prevention (CDC). Since the early 1980s, however, the reported incidence of pertussis has cyclically increased every 3 to 4 years for unknown reasons. The last peak was in 1993, when 6586 cases were reported, a 61% increase from 1992. Twentythree patients died between 1992 to 1993 from complications of pertussis.1 W h y has there been an increase in the incidence of pertussis in the United States despite an effective immunization program? The CDC is unsure. The answer may lie in the fact that the pertussis vaccine is not 100% effective. In fact, the vaccine's effectiveness between 1992 and 1994 among children aged 7 to 18 months was only 85% after three doses of vaccine, and among children aged 19 to 47 months it was 94% after four or more doses. Even these estimates are overstated because they assume that all children received the D P T vaccination. However, an estimated 6.7% of children receive the diphtheria-tetanus vaccination instead of the D P T vaccine, and some children are not vaccinated at all. So in reality the vaccine's overall effectiveness is really only 64% and 82% after three or four doses, respectively. 1 The current pertussis vaccination schedule recommended by both the American Academy of Pediatrics and the Advisory Committee on Immunization Practices is for D T P vaccines to be administered at 2, 4, and 6 months of age. Additional doses of D T P should be given at 12 to 18 months of age and again at 4 to 6 years of age. 7 Pertussis is defined by the World Health Organization (WHO) as a paroxysmal coughing illness of 21 or more days in duration with at least one of the following additional criteria: (1) positive nasopharyngeal culture in the patient or in a close family contact, (2) seroconversion or a significant rise in IgG or IgA antibody against P T and FHA antigens, or (3) contact with a culture-confirmed infected household member or other close contact. 8 Both our patients met the W H O criteria for diagnosis of pertussis. Because pertussis appears to be making a comeback, ophthalmologists need to be aware of the potential eye findings of this disease. To our knowledge, the only reports
Journal of AAPOS Volume 2 Number 2 April 1998
of ocular findings of pertussis are from the early 1900s, when the disease was common. Duke-Elder, 4 in the 1920s, reported that ocular complications from pertussis infection were rare. These complications were usually mechanical in nature, as a result of the coughing paroxysms, and included hemorrhages of the subconjunctival space, orbit, anterior chamber, and rarely of the retina and ocular adnexa. Detachment of the retina was a very unusual complication but was reported. Even blindness, cerebral hemorrhage, and a variety of ophthalmoplegias were rarely reported), 5, 6 The characteristic forceful coughing paroxysms with a strong Valsalva effect are enough to explain all the known associated ophthalmic findings. Proptosis has not been previously reported as a sequela of pertussis infection and should be added to the list of possible ophthalmologic complications. Increased venous congestion and venous pressure resulting from the Valsalva effect are thought to be the cause of the hemorrhages and the orbital soft tissue edema. Simultaneous periorbital and subconjunctival hemorrhages occur in relatively few disease entities. Coagulopathies, thrombocytopenia, and trauma (occult or known) are the most common causes. But simultaneous subconjunctival and eyelid hemorrhages have also been reported in patients with metastatic neuroblastoma, rhabdomyosarcoma, and leukemia (Table). 9-13 Pertussis infection should be added to this differential diagnosis. Negative culture results are not uncommon in pertussis because many patients are seen in the late paroxysmal or early convalescent stages when 50% of nasopharyngeal cultures for pertussis are negative. Positive culture results therefore are not required to diagnose the disease. Patient 2 was seen late in the disease process and had negative culture results. Pertussis serologic studies were then performed to confirm the diagnosis. Pertussis serologic tests, however, are difficult to interpret and therefore are not routinely used. Elevated IgG could be attributed to the previous immunization. IgA, on the other hand, is an antibody found primarily in secretions and in mucous membranes. IgA is only elevated in an acute infection and when elevated lends support to the diagnosis of pertussis. Proptosis was a prominent feature in Patient 1. The possibility of an orbital tumor such as rhabdomyosarcoma or metastatic neuroblastoma was entertained and ruled out with neuroimaging. Nonaccidental trauma is also an important cause of lid and subconjunctival hemorrhages. This possible etiology was entertained in Patient 1, and no evidence was found to support it. It is important to recognize that the presence of pertussis infection does not rule out the possibility of child abuse resulting in covert injury, and appropriate investigation should still be undertaken in suspicious cases. In conclusion, pertussis should be considered in the differential diagnosis of eyelid ecchymosis, proptosis, or subconjunctival hemorrhage. Ophthalmologists should be aware that the incidence of pertussis infection is increasing.
Journal of M P O S Volume 2 Number 2 April 1998
Paysse and Coats 119
References 1. Centers for Disease Control and Prevention. Pertussis--United States, January 1992-June 1995. MMWR Morbid Mortal Wkly Rep 1995;44:525-9. 2. Katz SL. Pertussis. In: Wyngaarden JB, Smith LH, editors. Cecil textbook of medicine. 17th ed. Philadelphia: WB Saunders; 1985. p. 1568-70. 3. Words N, Strebel PM, Wharton M, et al. Pertussis deaths: report of 23 cases in the United States, 1992-1993. Pediatrics 1996;97:607-12. 4. Duke-Elder S. System of ophthalmology. Vol 15. St. Louis: CV Mosby; 1973. 5. Batten FE. Complete ophthalmoplegia externa with blindness of acute onset. BMJ 1903;1:249. 6. Gowring BW. A case of complete ophthalmoplegia occurring during whooping-cough. BMJ 1903;2:1638. 7. Centers for Disease Control and Prevention. Recommended child-
8.
9. 10. 11. 12.
13.
hood immunization United States, 1989-1991. MMWR Morbid Mortal Wldy Rep 1992;41:11-9. Committee on Infectious Diseases, Acellular Pertussis Vaccine. Recommendations for use as the initial series in infants and children. Pediatrics 1997;99:282-8. Eyster, ME, Bill FM, Blaff PM. Central nervous system bleeding in hemophiliacs. Blood 1978;51:1179-88. Levine MN, Hirsch J. Hemorrhagic complications of anticoagulant therapy. Semin Thromb Hemost 1986;12:39-57. Kincaid MC, Green WR. Ocular and orbital involvement in leukemia. Surv Ophthalmol 1983;27:211-32. Leonardy NJ, Rupani M, Dent G, Klintworth GK. Analysis of 135 autopsy eyes for ocular involvement in leukemia. Am J Ophthalmol 1990;109:436-44. Porterfield JT, Zimmerman LE. Rhabdomyosarcoma of the orbit: a clinicopathologic study of 55 cases. Virchows Arch Path Anat 1962;335:329-44.
p
ertussis (whooping cough) has been uncommon in the United States during the past 45 years as a result of effective immunization programs. Since the early 1980s, however, a resurgence of the disease has been noted (Figure 1). The incidence of reported cases of pertussis in the United States has cyclically increased for unknown reasons every 3 to 4 years. The last peak was in 1993, in which 6586 cases were reported. 1 Even today whooping cough is responsible for a significant number of childhood deaths worldwide, especially in areas where poor immunization programs exist. Whooping cough is an acute respiratory illness that most often affects infants and young children. Premature infants are at higher risk of contracting the disease. The etiologic agent is usually Bordetella pertussis, occasionally Bordetella parapertussis, and rarely Bordetella bronchioseptica. Whooping cough is typically divided into three stages, each lasting about 14 days. The catarrhal stage begins after an incubation period of 7 to 16 days. Symptoms are initially indistinguishable from a mild upper respiratory infection. Sneezing, mild fever, and nocturnal cough are present. Seven to 14 days later the paroxysmal stage begins. The cough becomes the most prominent feature and is more frequent, diurnal, and paroxysmal during this stage. A typical coughing paroxysm starts with a series of 15 to 20 short coughs, each cough more intense than the last. The paroxysm is then followed by a deep inspiratory effort, producing the characteristic "whoop." A thick mucus plug may be expelled, not uncommonly followed by emesis. The convalescent stage follows 14 days later. Gradually the coughing paroxysms become less frequent and less
From the Cullen Eye Institute, Baylor College of MedMne, Texas Children's Hospital, Houston, Texas. Supported in part by an unrestrictedgrantfrom Research to Prevent Blindness, Inc., New York. Submitted February 27, 199Z Revision accepted October 13, 199Z J AAPOS 1998;2:116-9. Reprint requests: Evelyn A. Paysse, MD, Baylor College of Medicine, Texas Children's Hospital, 1102 Bates, #300, Houston, T X 77030. Copyright © 1998 by the American Assodationfor Pediatric Ophthalmology and Strabismus. 1091-8531/98 $5.00 + 0 75/1/87499
116
April 1998
intense until they cease altogether. In very young infants, the paroxysms and whoop are often not present, replaced instead by choking spells and apneic episodes. 2 Infants may develop seizures and an acute encephalopathy. 3 Treatment for whooping cough varies depending on the severity of the disease. Several antibiotics are effective, including systemic erythromycin and oxytetracycline (in children older than 8 years). The organism is killed within a few days of initiation of antibiotic treatment. Relapse, however, is frequent, and for this reason the antibiotic regimen should be continued for 14 days (erythromycin) or 14 to 21 days (oxytetracycline). Supportive care to maintain proper fluid and electrolyte balance, nutrition, and oxygenation are of obvious importance. Ophthalmologic complications in pertussis occur infrequently. The only reports of eye findings in pertussis are from the early 1900s and include retinal detachment, ophthalmoplegia, and hemorrhages of the subconjunctival space, retina, choroid, anterior chamber, and orbit. 4-6 Ocular and periocular hemorrhages therefore are generally not a well-known finding of the disease. CASE R E P O R T S Patient 1
A 5-year-old girl was referred by her pediatrician for evaluation of severe, progressive, painless right upper and lower eyelid ecchymoses and subconjunctival hemorrhage. She had had severe nocturnal coughing paroxysms for the last several weeks and had lost 2 pounds in the 2 weeks before our evaluation. There was no history of trauma, coagulopathy, blood dyscrasias, or other medical problems. Before onset of this illness, she had been in good health. The patient denied ocular pain, discharge, photophobia, headache, or other systemic symptoms. The recommended total of five doses of diphtheria-pertussis-tetanus (DPT) vaccine had been received on the recommended schedule. Her last D P T vaccine was at age 5 years. A complete ophthalmologic examination was performed. Findings included severe fight upper and lower eyelid ecchymoses and subconjunctival hemorrhage. Two millimeters of proptosis of the fight eye was measured. The remainder of ffournal of AAPOS
Journal of AAPOS Volume 2 Number 2 April 1998
eca
=.,I
REPORTED CASES OF PERTUSSIS United States, 1965-1995
8/
10
"O
Paysse and Coats 117
6
O
4 G)
O
2 0
t 1965
I
I
I
I
I
1970
1975
1980 Year
1965
1990
1995
FIG. 1. Reported cases of pertussis in United States, 1965 to 1995. Note cyclic increase in reported cases every 3 to 4 years. (From Centers for Disease Control and Prevention. MMWR Morbid Mortal Wkly Rep 1995;9:46.)
FIR. 3. Patient 2, with resolving subconjunctival hemorrhage and lid ecchymoses.
TABLE. Etiology of subconjunctival and periorbital hemorrhage in infants and children. Hemophilia Anticoagulant therapy Coagulopathies Thromboccytopenia Trauma (occult or known) Rhabdomyosarcoma Metastatic neuroblastoma Leukemia Pertussis
junctival hemorrhages cleared rapidly after resolution of the coughing paroxysms. Patient 2
FIB. 2. Patient 1, with severe lid ecchymoses and subconjunctival hemorrhage.
her ophthalmologic examination, including visual acuity, motility evaluation, pupillary testing, and indirect ophthalmoscopy, was normal. Two days after the initial ophthalmologic examination, severe upper and lower eyelid ecchymoses and subconjunctival hemorrhages developed in the uninvolved left eye (Figure 2). A sodal services consult was called to investigate the social situation, and no evidence of intentional trauma or abuse was found. Orbital computed tomography was negative for neoplasm. Orbital soft tissue swelling was noted, accounting for the proptosis of the right eye. The white blood cell count was elevated at 14,800/btl with 68% 1ymphocytes. The platelet count was normal at 313,000/btl. B. pertussis was cultured from a nasopharyngeal swab. The patient was treated with oral erythromycin for 2 weeks, and the illness quickly subsided. The lid ecchymoses and subcon-
A 9-year-old boy was referred by his pediatrician for a 1week history of severe bilateral lid ecchymoses and subconjunctival hemorrhage. The medical history was significant for a coughing illness of more than 3 weeks' duration. There was a prominent history of severe nocturnal coughing paroxysms. The patient denied a history of ocular discharge, photophobia, pain, decreased vision, diplopia, or other symptoms. Medical history was significant for sickle cell anemia. The patient was status post splenectomy. He had received all five scheduled pertussis vaccinations (DPT), including his most recent one at age 4 years. Comprehensive ophthalmologic examination was notable for moderate bilateral upper and lower eyelid ecchymoses and severe bilateral subconjunctival hemorrhages (Figure 3). The remainder of the ophthalmologic examination was normal. A nasopharyngeal pertussis culture was negative. The pertussis immunoglobulin G (IgG) antibody level was elevated at 81 (normal <10) and the permssis immunoglobulin A (IgA) antibody level was elevated at 41 (normal <15), both highly suggestive of a recent permssis infection. Pertussis immunoglobulin M was not detected. Complete blood cell count revealed
118
Paysseand Coats
an elevated total white blood cell count of 14,800/gl with 52% lymphocytes. The platelet count was 307,000/gl. The patient was treated with oral erythromycin for 2 weeks and the cough, lid ecchymoses, and subconjunctival hemorrhages quickly resolved.
DISCUSSION Pertussis was a major cause of morbidity and mortality among infants and children in the United States in the prevaccine era (before the 1940s). The highest incidence of pertussis cases in this country was approximately 260,000 reported cases in 1934.1 Nine thousand pertussisrelated deaths were reported in 1923, the highest number ever reported. Since the introduction of the pertussis vaccine in 1949, the number of pertussis cases has markedly declined. In 1976 only 1010 cases were reported to the Centers for Disease Control and Prevention (CDC). Since the early 1980s, however, the reported incidence of pertussis has cyclically increased every 3 to 4 years for unknown reasons. The last peak was in 1993, when 6586 cases were reported, a 61% increase from 1992. Twentythree patients died between 1992 to 1993 from complications of pertussis.1 W h y has there been an increase in the incidence of pertussis in the United States despite an effective immunization program? The CDC is unsure. The answer may lie in the fact that the pertussis vaccine is not 100% effective. In fact, the vaccine's effectiveness between 1992 and 1994 among children aged 7 to 18 months was only 85% after three doses of vaccine, and among children aged 19 to 47 months it was 94% after four or more doses. Even these estimates are overstated because they assume that all children received the D P T vaccination. However, an estimated 6.7% of children receive the diphtheria-tetanus vaccination instead of the D P T vaccine, and some children are not vaccinated at all. So in reality the vaccine's overall effectiveness is really only 64% and 82% after three or four doses, respectively. 1 The current pertussis vaccination schedule recommended by both the American Academy of Pediatrics and the Advisory Committee on Immunization Practices is for D T P vaccines to be administered at 2, 4, and 6 months of age. Additional doses of D T P should be given at 12 to 18 months of age and again at 4 to 6 years of age. 7 Pertussis is defined by the World Health Organization (WHO) as a paroxysmal coughing illness of 21 or more days in duration with at least one of the following additional criteria: (1) positive nasopharyngeal culture in the patient or in a close family contact, (2) seroconversion or a significant rise in IgG or IgA antibody against P T and FHA antigens, or (3) contact with a culture-confirmed infected household member or other close contact. 8 Both our patients met the W H O criteria for diagnosis of pertussis. Because pertussis appears to be making a comeback, ophthalmologists need to be aware of the potential eye findings of this disease. To our knowledge, the only reports
Journal of AAPOS Volume 2 Number 2 April 1998
of ocular findings of pertussis are from the early 1900s, when the disease was common. Duke-Elder, 4 in the 1920s, reported that ocular complications from pertussis infection were rare. These complications were usually mechanical in nature, as a result of the coughing paroxysms, and included hemorrhages of the subconjunctival space, orbit, anterior chamber, and rarely of the retina and ocular adnexa. Detachment of the retina was a very unusual complication but was reported. Even blindness, cerebral hemorrhage, and a variety of ophthalmoplegias were rarely reported), 5, 6 The characteristic forceful coughing paroxysms with a strong Valsalva effect are enough to explain all the known associated ophthalmic findings. Proptosis has not been previously reported as a sequela of pertussis infection and should be added to the list of possible ophthalmologic complications. Increased venous congestion and venous pressure resulting from the Valsalva effect are thought to be the cause of the hemorrhages and the orbital soft tissue edema. Simultaneous periorbital and subconjunctival hemorrhages occur in relatively few disease entities. Coagulopathies, thrombocytopenia, and trauma (occult or known) are the most common causes. But simultaneous subconjunctival and eyelid hemorrhages have also been reported in patients with metastatic neuroblastoma, rhabdomyosarcoma, and leukemia (Table). 9-13 Pertussis infection should be added to this differential diagnosis. Negative culture results are not uncommon in pertussis because many patients are seen in the late paroxysmal or early convalescent stages when 50% of nasopharyngeal cultures for pertussis are negative. Positive culture results therefore are not required to diagnose the disease. Patient 2 was seen late in the disease process and had negative culture results. Pertussis serologic studies were then performed to confirm the diagnosis. Pertussis serologic tests, however, are difficult to interpret and therefore are not routinely used. Elevated IgG could be attributed to the previous immunization. IgA, on the other hand, is an antibody found primarily in secretions and in mucous membranes. IgA is only elevated in an acute infection and when elevated lends support to the diagnosis of pertussis. Proptosis was a prominent feature in Patient 1. The possibility of an orbital tumor such as rhabdomyosarcoma or metastatic neuroblastoma was entertained and ruled out with neuroimaging. Nonaccidental trauma is also an important cause of lid and subconjunctival hemorrhages. This possible etiology was entertained in Patient 1, and no evidence was found to support it. It is important to recognize that the presence of pertussis infection does not rule out the possibility of child abuse resulting in covert injury, and appropriate investigation should still be undertaken in suspicious cases. In conclusion, pertussis should be considered in the differential diagnosis of eyelid ecchymosis, proptosis, or subconjunctival hemorrhage. Ophthalmologists should be aware that the incidence of pertussis infection is increasing.
Journal of M P O S Volume 2 Number 2 April 1998
Paysse and Coats 119
References 1. Centers for Disease Control and Prevention. Pertussis--United States, January 1992-June 1995. MMWR Morbid Mortal Wkly Rep 1995;44:525-9. 2. Katz SL. Pertussis. In: Wyngaarden JB, Smith LH, editors. Cecil textbook of medicine. 17th ed. Philadelphia: WB Saunders; 1985. p. 1568-70. 3. Words N, Strebel PM, Wharton M, et al. Pertussis deaths: report of 23 cases in the United States, 1992-1993. Pediatrics 1996;97:607-12. 4. Duke-Elder S. System of ophthalmology. Vol 15. St. Louis: CV Mosby; 1973. 5. Batten FE. Complete ophthalmoplegia externa with blindness of acute onset. BMJ 1903;1:249. 6. Gowring BW. A case of complete ophthalmoplegia occurring during whooping-cough. BMJ 1903;2:1638. 7. Centers for Disease Control and Prevention. Recommended child-
8.
9. 10. 11. 12.
13.
hood immunization United States, 1989-1991. MMWR Morbid Mortal Wldy Rep 1992;41:11-9. Committee on Infectious Diseases, Acellular Pertussis Vaccine. Recommendations for use as the initial series in infants and children. Pediatrics 1997;99:282-8. Eyster, ME, Bill FM, Blaff PM. Central nervous system bleeding in hemophiliacs. Blood 1978;51:1179-88. Levine MN, Hirsch J. Hemorrhagic complications of anticoagulant therapy. Semin Thromb Hemost 1986;12:39-57. Kincaid MC, Green WR. Ocular and orbital involvement in leukemia. Surv Ophthalmol 1983;27:211-32. Leonardy NJ, Rupani M, Dent G, Klintworth GK. Analysis of 135 autopsy eyes for ocular involvement in leukemia. Am J Ophthalmol 1990;109:436-44. Porterfield JT, Zimmerman LE. Rhabdomyosarcoma of the orbit: a clinicopathologic study of 55 cases. Virchows Arch Path Anat 1962;335:329-44.