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Show Unilateral Straight Hair and Congenital Horner Syndrome Frederick M. Wang, MD, Christian Wertenbaker, MD, Hyung Cho, MD, Maury A. Marmor, MD, Sandra S. Ahn-Lee, MD, Bruno A. Bernard, PhD Abstract: Congenital Horner syndrome is a rare disorder that accounts for less than 5% of all cases of Horner syndrome. Like Horner syndrome in general, it consists pri-marily of ptosis, miosis, and anhidrosis. Congenital Horner syndrome may manifest some special features such as iris heterochromia since the sympathetic nervous system is an essential component for the development and maintenance of eye color. We present 3 cases of unilateral straight hair in association with congenital Horner syndrome in which the patients had straight hair ipsilateral to the Horner syn-drome, whereas on the contralateral side, it was curly, and we discuss possible mechanisms for this phenomenon. Journal of Neuro-Ophthalmology 2012;32:132-134 doi: 10.1097/WNO.0b013e318240c678 © 2012 by North American Neuro-Ophthalmology Society Congenital Horner syndrome, in addition to the classic triad of ptosis, miosis, and anhidrosis, may manifest some special features, including iris heterochromia (1). Since the sympathetic nervous system is essential for the develop-ment and maintenance of eye color, sympathetic denerva-tion during ocular development can result in heterochromia, with the lighter-colored iris on the affected side. Shewmon (2) reported a case of unilateral straight hair in association with congenital Horner syndrome. The patient had straight hair ipsilateral to the Horner syndrome, whereas on the contralateral side, it was curly. This finding has been described in only 1 other publication (3). We document 3 cases of ipsilateral straight hair in association with congenital Horner syndrome, including the case previously described (3), and discuss possible mechanisms for this phenomenon. CASE REPORTS Case 1 A 2-year-old boy was found to have a congenital right Horner syndrome. Examination revealed heterochromia of the right iris and 2 mm of right ptosis. In light, the pupils measured 2 mm, right eye, and 3 mm, left eye, while in dark, they were 3 mm, right eye, and 6 mm, left eye. Right dilation lag was noted, and there was anhidrosis of the right side of the face and scalp. The hair on the right side was straighter than on the left (Fig. 1A). Comprehensive patient evaluation including imaging of the chest failed to reveal an etiology of the patient's Horner syndrome. Case 2 A 3-week-old boy was referred for evaluation of ptosis of the left eye that had been present since birth following a prolonged labor. Medical history including growth and development was normal. Ophthalmic examination revealed 2 mm of left ptosis. The left pupil was significantly smaller than the right, and this difference was accentuated in the dark. In light, the pupils were 2.5 mm, right eye, and 2.0 mm, left eye. In dark, they were 5 mm, right eye, and 3.5 mm, left eye. Heterochromia was not present. A pediatric neurologist documented a left brachial plexus injury with left arm weakness. Subsequent examination revealed anhidrosis of the left side of the face and left shoulder. The hair on the left side of his head was straight, while on the right, it was curly (Fig. 1B). Case 3 A 3-month-old boy was referred for evaluation of ptosis of the left upper lid. His mother related that the ptosis began at 4 weeks of age. The boy had been in good health with no history of birth trauma and he moved both arms equally. Department of Ophthalmology and Visual Science (FMW, CW, HC), Albert Einstein College of Medicine of Yeshiva University, Bronx, New York; Department of Ophthalmology (FMW), New York Eye and Ear Infirmary, New York, New York; Department of Pediatric Ophthalmology (SSA-L), Manhattan Eye, Ear, and Throat Hospital, New York, New York; Department of Ophthalmology (MAM), SUNY Stony Brook, Stony Brook, New York; and L'Oréal Recherche (BAB), Clichy, France. Supported in part by an unrestricted grant from Research to Prevent Blindness, Inc, New York, New York. The authors report no conflicts of interest. Address correspondence to Frederick M. Wang, MD, 30 East 40th Street, New York, NY 10016; E-mail: fmwang17@gmail.com 132 Wang et al: J Neuro-Ophthalmol 2012; 32: 132-134 Original Contribution Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Examination revealed 2 mm of ptosis of the left eyelid. In light, pupils measured 3 mm, right eye, and 2.5 mm, left eye, while in dark, they were 6 mm, right eye, and 3 mm, left eye. Cocaine eyedrop testing led to 2 mm of dilation of the right pupil, while the left pupil did not dilate. Anhidrosis of the left side of the face was noted, and the patient exhibited asymmetrical hair that was straighter on the left side (Fig. 1C). A mediastinal neuroblastoma was subsequently diag-nosed and later resected successfully. DISCUSSION The etiology of congenital Horner syndrome in our patients varied. In the first patient, the etiology could not be determined. The second patient had a brachial plexus injury, most likely due to birth trauma, and the third had a mediastinal neuroblastoma. It is unknown at what stage of development a sympathetic pathway lesion would lead to a change in hair curliness. One might think that this must occur in utero, but the presumed birth injury causing our second patient's Horner syndrome would argue against this. We use the term "congenital" in the generally accepted sense of the disorder manifesting in the first month after birth (4). The exact mechanism of curly hair formation is still a subject of some debate. Shewmon (2) speculated that the mechanism of the straighter hair on the side of the Horner syndrome might be due to absence of a trophic effect, similar to the loss of the trophic effect of sympathetic stim-ulation on the melanocytes of the iris, which results in a lighter iris color. A study in sheep suggested that the arrectores pilorum muscles could influence crimp formation in wool by sequential rhythmic contractions (5). Since sym-pathetic neurons innervate the arrectores pilorum muscles, it would then be possible that sympathetic denervation is associated with straighter hair in those with a predisposition to curly hair. A more recent study suggests that hair curli-ness is programmed from the hair bulb and independent of the arrectores pilorum muscles, as curly hair bulbs, sepa-rated from the arrectores pilorum and sebaceous glands, still grew curly hairs in vitro (6). It appears that human hair shape is influenced by numerous factors, including genetic background (7,8), asym-metry of keratin distribution (9), effects of IGF-binding pro-tein 5 (10), epidermal growth factor receptors (11), the lysophosphatidic acid/P2RY5 pathway (12), and pharmaco-logic treatment (13), including adrenergic drugs (14). With loss of sympathetic innervation due to Horner syndrome, the diminished adrenergic signal to the hair follicles might lead to straighter hair. Since the sympathetic pathway to hair follows the external carotid vasculature, one would expect that asym-metry of hair curliness would occur only with preganglionic lesions. Such is the case in all reported patients to date. REFERENCES 1. Weinstein JM, Zweifel TJ, Thompson HS. Congenital Horner's syndrome. Arch Ophthalmol. 1980;98:1074. 2. Shewmon DA. Unilateral straight hair in congenital Horner's syndrome due to stellate ganglion tumor. Ann Neurol. 1983;3:345. 3. Wertenbaker C. Horner's Syndrome. Clinical Signs in Ophthalmology. Vol 1, No. 4. Alcon Laboratories, 1984. 4. Yanoff M, Duker JS. Corneal and external eye manifestations of systemic disease. In: Ophthalmology, 3rd edition. Philadelphia, PA: Mosby Elsevier, 2009:340. 5. Chapman RE. The ovine arrector pili musculature and crimp formation in wool. In: Lyne AG, Short BF, eds. Biology of the Skin and Hair Growth. New York, NY: American Elsevier Pub Co. Inc, 1965:201-232. 6. Thibaut O, Gaillard P, Bouhanna DW, Bernard BA. Human hair shape is programmed from the bulb. Br J Dermatol. 2005;152:632-638. 7. Kjaer KW, Hansen L, Eiberg H, Leichet P, Opitz JM, Tommerup N. Novel connexin 43 (GJA1) mutation causes oculo-dento-digital dysplasia with curly hair. Am J Med Genet. 2004;127A:152-157. 8. Medland ES, Nyholt DR, Painter JN, McEvoy BP, McRae AF, Zhu G, Gordon SD, Ferreira MA, Wright MJ, Henders AK, Campbell MJ, Duffy DL, Hansell NK, Macgregor S, Slutske WS, Heath AC, Montgomery GW, Martin NG. Common variants in the trichohyalin gene are associated with straight hair in Europeans. Am J Hum Genet. 2009;85: 750-755. FIG. 1. A. Case 1. B. Case 2. C. Case 3. All patients demonstrate straight hair ipsilateral to the side of the Horner syndrome. Wang et al: J Neuro-Ophthalmol 2012; 32: 132-134 133 Original Contribution Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. 9. Thibaut S, Barbarat P, Leroy F, Bernard BA. Human hair keratin network and curvature. Int J Dermatol. 2007; 46(suppl 1):6-9. 10. Sriwiriyanont P, Hachiya A, Pickens WL, Moriwaki S, Kitahara T, Visscher MO, Kitzmiller WJ, Bello A, Takema Y, Kobinger GP. Effects of IGF-binding protein 5 in dysregulating the shape of human hair. J Invest Dermatol. 2011;131:320-328. 11. Agero AL, Dusza SW, Benvenuto-Andrade C, Busam KJ, Myskowski P, Halpern AC. Dermatologic side effects associated with the epidermal growth factor receptor inhibitors. J Am Acad Dermatol. 2006;55:657-670. 12. Shimomura Y, Wajid M, Ishii Y, Shapiro L, Petukhova L, Gordon D, Christiano AM. Disruption of P2RY5, an orphan receptor G protein-coupled receptor, underlies autosomal recessive wooly hair. Nat Genet. 2008;40:265-266. 13. Bessis D, Luong MS, Blanc P, Chapoutot C, Larrey D, Guilhou JJ, Guillot B. Straight hair associated with interferon-alpha plus ribavirin in hepatitis C infection. Br J Dermatol. 2002;147:385-410. 14. Botchkarev VA, Peters EM, Bothckareva NV, Maurer M, Paus R. Hair cycle-dependent changes in adrenergic skin innervation, and hair growth modulation by adrenergic drugs. J Invest Dermatol. 1999;113:878-887. 134 Wang et al: J Neuro-Ophthalmol 2012; 32: 132-134 Original Contribution Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. |
