ILLUSTRATIVE CASE
The 2012 PEMpix Photograph Competition Award Winner Making a Case for Pringles-Flavored Carrots Cortney C. Braund, MD,* Genie E. Roosevelt, MD, MPH,* Emily A. McCourt, MD,† and Keith Weisz, MD* Abstract: This is a case report of a 14-year-old boy with autism who presented with photophobia. Physical examination was significant for bilateral corneal ulcers. Differential diagnosis of this chief complaint and the management of the suspected condition are discussed. This case was presented at the Section of Emergency Medicine Meeting at the National Conference and Exhibition of the American Academy of Pediatrics in 2012 and was awarded first place in the PEMpix photograph competition. Key Words: vitamin A deficiency, xerophthalmia, corneal ulcer (Pediatr Emer Care 2014;30: 900–901)
CASE J.H. is a 14-year-old nonverbal, autistic boy who presented with bilateral eye redness and photophobia for 2 months. The patient's vision seemed to deteriorate significantly before presentation, causing him to fall while exiting the school bus. At time of presentation to the Emergency Department, he refused to open his eyes. Review of symptoms was significant for 2 days of tactile fevers, decreased oral intake for 2 months, and weight below the fifth percentile. There was no history of vomiting, rash, sick contacts, trauma, or travel. On physical examination, the boy was alert but pale, and walking into walls with his eyes covered by his hands. His vital signs were significant for a pulse of 132 beats per minute and a temperature of 38.5°C. An eye examination under sedation demonstrated bilateral scleral injection, a 4 4-mm central corneal ulcer with mild amount of thinning in the right eye (Fig. 1), and a complete ulcer from limbus to limbus with bulging appearance consistent with a descemetocele in the left eye (Fig. 2). The anterior chamber was formed on the right, but the left was not visualized. The ophthalmologists were consulted and the patient was admitted for intensive antibiotic therapy with q1h ophthalmic antibiotic drops. The differential diagnosis for the severe bilateral corneal ulcers in this patient included bacterial keratitis, herpes simplex keratitis, mechanical trauma with presumptive bacterial superinfection, or rheumatologic disease. As additional history revealed that the patient had eaten only Pringles and crackers for the last 2 years, nutritional deficiency became a concern as well. Examination under anesthesia on day 3 of hospitalization revealed severe xerophthalmia. Additionally, vitamin A levels were low (5 μg/dL with a normal range of 19–77 μg/dL), and herpes simplex virus and bacterial cultures from corneal scrapings
From the *Department of Pediatrics, Section of Emergency Medicine, and †Department of Pediatric Ophthalmology, University of Colorado Denver, Children's Hospital Colorado, Aurora, CO. Disclosure: The authors declare no conflict of interest. Reprints: Cortney C. Braund, MD, Department of Pediatrics, Section of Emergency Medicine, University of Colorado Denver, Children's Hospital Colorado, 13123 East 16th Ave, Box 251, Aurora, CO 80045 (e‐mail: [email protected]). Copyright © 2014 by Lippincott Williams & Wilkins ISSN: 0749-5161
900
www.pec-online.com
FIGURE 1. Right eye upon presentation demonstrating central corneal ulcer.
FIGURE 2. Left eye upon presentation demonstrating complete ulcer from limbus to limbus with bulging appearance consistent with a descemetocele.
FIGURE 3. Right eye 3 months after initiation of therapy showing mild residual corneal scarring. Pediatric Emergency Care • Volume 30, Number 12, December 2014
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Pediatric Emergency Care • Volume 30, Number 12, December 2014
2012 PEMpix Photograph Competition Award Winner
eye to aid in reepithelialization, and vitamin A replacement was initiated. Examination under anesthesia 3 months after initial presentation revealed overall improvement, with mild scarring on the right (Fig. 3) and significant corneal scarring on the left (Fig. 4). With improvement in his vision, he was able to independently ambulate and perform activities of daily living. Furthermore, he became involved with a nutritionist and gained 35 lb in that time frame.
REFERENCES 1. Congdon NG, Friedman DS, Lietman T. Important causes of visual impairment in the world today. JAMA. 2003;290:2057–2060.
FIGURE 4. Left eye 3 months after initiation of therapy showing residual corneal scarring.
were negative. Therefore, the diagnosis of vitamin A deficiency and xerophthalmia was made.
DISCUSSION Vitamin A deficiency is a leading cause of preventable blindness in children worldwide, but is an uncommon diagnosis in developed nations.1,2 Xerophthalmia is the spectrum of ocular disease arising from vitamin A deficiency. Ocular changes often begin with night blindness, and range from conjunctival and corneal xerosis to corneal ulceration, melting (keratomalacia), and lastly retinopathy.3 There have been reports describing cases of xerophthalmia in patients with cystic fibrosis,4 gastrointestinal malabsorptive diseases,5 and psychiatric-induced dietary restrictions.6 Patients with autism are at high risk for vitamin A deficiency and its ocular manifestations because of their extreme food selectivity practices.7–10 As the prevalence of autism has increased an estimated 78% since 2002,11 clinicians should have a high index of suspicion for the diagnosis given the relatively inexpensive nature of the treatment and the possible reversal of ocular disease if initiated early.12 In our patient, a ProKera-processed amniotic membrane patch (ProKera; Bio-Tissue, Inc, Miami, FL) was placed in the left
© 2014 Lippincott Williams & Wilkins
2. Sommer A. Nutritional factors in corneal xerophthalmia and keratomalacia. Arch Ophthalmol. 1982;100:399–403. 3. Smith J, Steinemann T. Vitamin A deficiency and the eye. Int Ophthalmol Clin. 2000;40:83–91. 4. Brooks HL Jr, Driebe WT Jr, Schemmer GG. Xerophthalmia and cystic fibrosis. Arch Ophthalmol. 1990;108:354–357. 5. Lange AP, Maloney G, Sheldon CA, et al. Bilateral corneal ulceration caused by vitamin A deficiency in eosinophilic gastroenteropathy. Case Rep Ophthalmol. 2011;2:302–306. 6. Cooney TM, Johnson CS, Elner VM. Keratomalacia caused by psychiatric-induced dietary restrictions. Cornea. 2007;26:995–997. 7. Steinemann T, Christiansen S. Vitamin A deficiency and xerophthalmia in an autistic child. Arch Ophthalmol. 1998;116:392–395. 8. Basti S, Schmidt C. Vitamin A deficiency [letter to the editor]. Cornea. 2008;27:973. 9. Tanoue K, Matsui K, Takamasu T. Fried potato diet causes vitamin A deficiency in an autistic child. JPEN J Parenter Enteral Nutr. 2012; 36(6):753–755. 10. Kuehn BM. Data on autism prevalence, trajectories illuminate socioeconomic disparities. JAMA. 2012;307:2137–2138. 11. Schreck K, Williams K, Smith A. A comparison of eating behaviors between children with and without autism. J Autism Dev Disord. 2004; 34:433–438. 12. Sommer A, Tarwotjo I. Protein deficiency and treatment of xerophthalmia. Arch Ophthalmol. 1982;100:785–787.
www.pec-online.com
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
901
The 2012 PEMpix Photograph Competition Award Winner Making a Case for Pringles-Flavored Carrots Cortney C. Braund, MD,* Genie E. Roosevelt, MD, MPH,* Emily A. McCourt, MD,† and Keith Weisz, MD* Abstract: This is a case report of a 14-year-old boy with autism who presented with photophobia. Physical examination was significant for bilateral corneal ulcers. Differential diagnosis of this chief complaint and the management of the suspected condition are discussed. This case was presented at the Section of Emergency Medicine Meeting at the National Conference and Exhibition of the American Academy of Pediatrics in 2012 and was awarded first place in the PEMpix photograph competition. Key Words: vitamin A deficiency, xerophthalmia, corneal ulcer (Pediatr Emer Care 2014;30: 900–901)
CASE J.H. is a 14-year-old nonverbal, autistic boy who presented with bilateral eye redness and photophobia for 2 months. The patient's vision seemed to deteriorate significantly before presentation, causing him to fall while exiting the school bus. At time of presentation to the Emergency Department, he refused to open his eyes. Review of symptoms was significant for 2 days of tactile fevers, decreased oral intake for 2 months, and weight below the fifth percentile. There was no history of vomiting, rash, sick contacts, trauma, or travel. On physical examination, the boy was alert but pale, and walking into walls with his eyes covered by his hands. His vital signs were significant for a pulse of 132 beats per minute and a temperature of 38.5°C. An eye examination under sedation demonstrated bilateral scleral injection, a 4 4-mm central corneal ulcer with mild amount of thinning in the right eye (Fig. 1), and a complete ulcer from limbus to limbus with bulging appearance consistent with a descemetocele in the left eye (Fig. 2). The anterior chamber was formed on the right, but the left was not visualized. The ophthalmologists were consulted and the patient was admitted for intensive antibiotic therapy with q1h ophthalmic antibiotic drops. The differential diagnosis for the severe bilateral corneal ulcers in this patient included bacterial keratitis, herpes simplex keratitis, mechanical trauma with presumptive bacterial superinfection, or rheumatologic disease. As additional history revealed that the patient had eaten only Pringles and crackers for the last 2 years, nutritional deficiency became a concern as well. Examination under anesthesia on day 3 of hospitalization revealed severe xerophthalmia. Additionally, vitamin A levels were low (5 μg/dL with a normal range of 19–77 μg/dL), and herpes simplex virus and bacterial cultures from corneal scrapings
From the *Department of Pediatrics, Section of Emergency Medicine, and †Department of Pediatric Ophthalmology, University of Colorado Denver, Children's Hospital Colorado, Aurora, CO. Disclosure: The authors declare no conflict of interest. Reprints: Cortney C. Braund, MD, Department of Pediatrics, Section of Emergency Medicine, University of Colorado Denver, Children's Hospital Colorado, 13123 East 16th Ave, Box 251, Aurora, CO 80045 (e‐mail: [email protected]). Copyright © 2014 by Lippincott Williams & Wilkins ISSN: 0749-5161
900
www.pec-online.com
FIGURE 1. Right eye upon presentation demonstrating central corneal ulcer.
FIGURE 2. Left eye upon presentation demonstrating complete ulcer from limbus to limbus with bulging appearance consistent with a descemetocele.
FIGURE 3. Right eye 3 months after initiation of therapy showing mild residual corneal scarring. Pediatric Emergency Care • Volume 30, Number 12, December 2014
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Pediatric Emergency Care • Volume 30, Number 12, December 2014
2012 PEMpix Photograph Competition Award Winner
eye to aid in reepithelialization, and vitamin A replacement was initiated. Examination under anesthesia 3 months after initial presentation revealed overall improvement, with mild scarring on the right (Fig. 3) and significant corneal scarring on the left (Fig. 4). With improvement in his vision, he was able to independently ambulate and perform activities of daily living. Furthermore, he became involved with a nutritionist and gained 35 lb in that time frame.
REFERENCES 1. Congdon NG, Friedman DS, Lietman T. Important causes of visual impairment in the world today. JAMA. 2003;290:2057–2060.
FIGURE 4. Left eye 3 months after initiation of therapy showing residual corneal scarring.
were negative. Therefore, the diagnosis of vitamin A deficiency and xerophthalmia was made.
DISCUSSION Vitamin A deficiency is a leading cause of preventable blindness in children worldwide, but is an uncommon diagnosis in developed nations.1,2 Xerophthalmia is the spectrum of ocular disease arising from vitamin A deficiency. Ocular changes often begin with night blindness, and range from conjunctival and corneal xerosis to corneal ulceration, melting (keratomalacia), and lastly retinopathy.3 There have been reports describing cases of xerophthalmia in patients with cystic fibrosis,4 gastrointestinal malabsorptive diseases,5 and psychiatric-induced dietary restrictions.6 Patients with autism are at high risk for vitamin A deficiency and its ocular manifestations because of their extreme food selectivity practices.7–10 As the prevalence of autism has increased an estimated 78% since 2002,11 clinicians should have a high index of suspicion for the diagnosis given the relatively inexpensive nature of the treatment and the possible reversal of ocular disease if initiated early.12 In our patient, a ProKera-processed amniotic membrane patch (ProKera; Bio-Tissue, Inc, Miami, FL) was placed in the left
© 2014 Lippincott Williams & Wilkins
2. Sommer A. Nutritional factors in corneal xerophthalmia and keratomalacia. Arch Ophthalmol. 1982;100:399–403. 3. Smith J, Steinemann T. Vitamin A deficiency and the eye. Int Ophthalmol Clin. 2000;40:83–91. 4. Brooks HL Jr, Driebe WT Jr, Schemmer GG. Xerophthalmia and cystic fibrosis. Arch Ophthalmol. 1990;108:354–357. 5. Lange AP, Maloney G, Sheldon CA, et al. Bilateral corneal ulceration caused by vitamin A deficiency in eosinophilic gastroenteropathy. Case Rep Ophthalmol. 2011;2:302–306. 6. Cooney TM, Johnson CS, Elner VM. Keratomalacia caused by psychiatric-induced dietary restrictions. Cornea. 2007;26:995–997. 7. Steinemann T, Christiansen S. Vitamin A deficiency and xerophthalmia in an autistic child. Arch Ophthalmol. 1998;116:392–395. 8. Basti S, Schmidt C. Vitamin A deficiency [letter to the editor]. Cornea. 2008;27:973. 9. Tanoue K, Matsui K, Takamasu T. Fried potato diet causes vitamin A deficiency in an autistic child. JPEN J Parenter Enteral Nutr. 2012; 36(6):753–755. 10. Kuehn BM. Data on autism prevalence, trajectories illuminate socioeconomic disparities. JAMA. 2012;307:2137–2138. 11. Schreck K, Williams K, Smith A. A comparison of eating behaviors between children with and without autism. J Autism Dev Disord. 2004; 34:433–438. 12. Sommer A, Tarwotjo I. Protein deficiency and treatment of xerophthalmia. Arch Ophthalmol. 1982;100:785–787.
www.pec-online.com
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
901
