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A Histopathologic and Morphometric Differentiation of Nerves in Optic Nerve Hypoplasia and Leber Hereditary Optic Neuropathy

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A Histopathologic and Morphometric Differentiation of Nerves in Optic Nerve Hypoplasia and Leber Hereditary Optic Neuropathy LABORATORY SCIENCES A Histopathologic and Morphometric Differentiation of Nerves in Optic Nerve Hypoplasia and Leber Hereditary Optic Neuropathy Hossein G. Saadati, MD; Hugo Y. Hsu, AB; Keith B. Heller, BS; Alfredo A. Sadun, MD, PhD Objectives: To characterize and quantitate optic nerve tion centrally, fibrocytic scarring, scattered “degenera- histopathologic and morphometric differences between tion dust,” and evidence of minimal inflammation, with optic nerve hypoplasia (ONH) as an early and congeni- residual axons limited to superior and temporal periph- tal form of intrinsic axonal loss and Leber hereditary op- eral clusters. Morphometric analysis revealed total fiber tic neuropathy (LHON) as a late and acquired form of populations of 98 000 in the ONH optic nerve and 48 000 intrinsic axonal loss. in the LHON optic nerve, representing 90% and 95% re- ductions, respectively, compared with the control optic Materials and Methods: Optic nerves from 3 sources nerve (1.2 million fibers). were examined: a 42-year-old healthy woman (control), a 53-year-old woman with ONH diagnosed postmortem, and Conclusions: Optic nerve hypoplasia and LHON pre- a 74-year-old woman with LHON. The optic nerves were sent 2 distinguishable and distinctive patterns of nerve processed, embedded, and stained with a 1% solution of fiber distribution and axonal dropout. The lack of de- paraphenylene diamine. Histopathologic and morphomet- generated axons in ONH indicates that any axonal death ric analyses were performed via light microscopy and a semi- probably occurred through apoptosis during develop- automatic computer image analysis system. ment. In LHON, degenerated axons and minimal grade of inflammation were obvious, implicating a more “ac- Results: The ONH showed severe axonal depletion with- tive” pathologic process. This study describes distinc- out degenerated profiles in an inferonasal sector, with only tions between these 2 optic neuropathies. a small superotemporal sector having a near normal appearance. The LHON revealed general axonal deple- Arch Ophthalmol. 1998;116:911-916 DEARTH of literature ex- nerve hypoplasia is usually idiopathic, al- ists regarding detailed his- though the use of several toxins, medica- topathologic or morpho- tions, and alcohol during pregnancy, as metric descriptions of well as maternal diabetes mellitus, all have optic nerve hypoplasia been associated with children born with A(ONH) and Leber hereditary optic neu- ONH.8,11 The characteristic clinical fea- ropathy (LHON), much less a compari- tures range from severely decreased vi- son of these early and late forms of intrin- sion usually discovered in infants7,12 to an sic axonal loss. incidental finding without visual loss with Optic nerve hypoplasia was first rec- or without mild visual field abnormali- ognized in 19151; however, detailed clini- ties and reduced pupillary response to light cal descriptions were not available until the in adults.11 Results of funduscopic exami- 1960s.2 Initially, ONH was thought to be nation reveal a small optic disc and often a rare anomaly; more recently, it has been the so-called double ring sign.8 regarded as a fairly common cause of blind- Unilateral or asymmetrical cases of ness in children.3 Optic nerve hypoplasia ONH may be misdiagnosed as simple pri- is a nonprogressive congenital defect that mary strabismus and amblyopia.10 Bilat- can occur unilaterally or bilaterally,4-7 ei- eral cases may be erroneously diagnosed ther in isolation or in association with as being one of the hereditary congenital other central nervous system malforma- forms of optic atrophy8 but should be pri- 3,4,6-8 From the Doheny Eye Institute, tions and endocrine malfunctions. The marily differentiated from Leber congen- University of Southern incidence of ONH is equal in males and ital amaurosis and achromatopsia, which 4,9 10 California School of Medicine, females. It can manifest in complete, may have similar clinical features. Leber Los Angeles. incomplete, or segmental forms.10 Optic congenital amaurosis is not to be confused ARCH OPHTHALMOL / VOL 116, JULY 1998 911 ©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/02/2021 MATERIALS AND METHODS In this study, 3 optic nerves were carefully exam- ined. The first was obtained from a 42-year-old woman without a history of any neurologic or ophthalmic problems who had died of hepatic failure. The sec- ond optic nerve was obtained from a 53-year-old woman with ONH who had been blind since birth. Her medical records from 1964 to 1973 indicated that she was initially misdiagnosed at age 20 years with “Leber disease” (some of the physician’s notes incor- rectly implied LHON rather than ONH). However, the same records revealed that her visual acuities had Figure 1. Cross-section of control retrobulbar optic nerve been fairly stable (20/100 OD and 20/200 OS) since (1% paraphenylene diamine solution, 3 80). early childhood. Her fundus examination results were described as bilateral optic nerve atrophy. However, results of a skull x-ray (performed in 1970) were nega- tive, and there was no family history of any neuro- and 14484, which leads to mitochondrial dysfunction. ophthalmologic disease. At age 53 years, the patient The clinical features of LHON include acute, bilateral loss died of extensive third-degree thermal injuries, and of central vision, which affects primarily men in the sec- the eyes, optic nerves, and brain were obtained and ond or third decade of life.15 Although the visual loss af- preserved in 20% formalin within 3 hours of death. fects both eyes and is roughly symmetrical, the onset is Gross and histoanatomical examination of the brain usually not absolutely simultaneous. and histopathologic examination of the eyes were 4,6,7,15,16 performed. Previous histopathologic reports of LHON The third optic nerve was obtained from a 74- have been largely limited to describing loss of the reti- year-old woman known to have the 11778 primary nal ganglion cells and an associated decreased optic nerve mutation of LHON. She experienced a painless loss diameter and axonal loss. of vision at age 38 years in 1 eye; then, within 3 weeks, In the present investigation, we examine and com- her vision declined to light perception in both eyes pare the histopathologic and morphometric findings in and remained at this level until she died of severe optic nerves from a healthy control patient, a congeni- chronic obstructive pulmonary disease. tally blind patient shown on pathologic examination All optic nerves were fixed, osmicated, dehy- results to have ONH, and a patient with the 11778 drated in ethanol and propylene oxide, and embed- ded in epoxy resin (Epon). The left optic nerves were primary mutation of LHON. We characterize and histo- sectioned on an ultramicrotome at a thickness of 1.0 logically compare these 2 optic neuropathies, which dif- to 1.5 mm and stained with a 1% solution of para- fer in that ONH reflects either underdevelopment or phenylene diamine, which stains for myelin and shows early apoptosis of retinal ganglion cells and their axons axonal degeneration.17,18 and LHON occurs in the second or third decade of life Stained cross-sections of optic nerves were ex- and may involve different pathophysiological mecha- amined with light microscopy for histopathologic nisms. comparisons, and morphometric analysis of fibers (axon with myelin sheath) was performed via a semi- automatic computer image analysis system with video RESULTS acquisition and digitalization as previously de- scribed.15,19 The total fiber population of the optic Light microscopic and morphometric analysis of the con- nerves was calculated from multiple sampling in each trol optic nerve revealed a diameter of 2.1 mm (Figure 1), of 16 sectors and in the total cross-sectional area of a normal nerve fiber size spectrum, and a total count of the optic nerve as described previously.15,19 Despite 1.2 million fibers (Table). the fact that the number of optic nerves was only 3, Results of gross examination of the brain of the there remained a statistically significant difference be- patient with ONH showed hypoplastic changes in the cause of the large population of fibers counted in each corpus callosum limited to the posterior portion (sple- nerve. nium). Results of gross examination of the eyes re- vealed (1) a peripheral cataract, (2) some atrophic changes in the ciliary bodies, and (3) flat and pale optic nerves with sclerotic vessels at the optic nerve head. with LHON, a disorder that usually manifests in adulthood. Results of light microscopy revealed severe axonal Leber hereditary optic neuropathy was first de- depletion largely limited to a large, inferonasal sector scribed by von Graefe in 1858 but was more clearly de- (Figure 2, left) that contained an anomalous small ar- fined and established by Leber in 1871 as a late-onset he- tery distinct from the central artery (Figure 2, right). Only reditary optic atrophy. Results of recent studies13,14 a small superotemporal sector contained axons com- demonstrate that LHON is inherited maternally and is pacted to a near normal appearance. The remaining sec- usually associated with point mutations in the mitochon- tors consisted of sparse scattering of axons with exten- drial DNA at nucleotide positions 11778, 3460, 15257, sive proliferation of astrocytes, with no apparent ARCH OPHTHALMOL / VOL 116, JULY 1998 912 ©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/02/2021 Histological and Morphometric Comparison of Control, Optic Nerve Hypoplasia (ONH), and Leber Hereditary Optic Neuropathy (LHON) Optic Nerves Optic Nerve Control ONH LHON Counted fiber population* 1 200 000 98 000 48 000 Total cross-sectional area, mm2 3.5 1.8 2.0 Pattern of axonal distribution Normal Mostly superotemporal and Peripheral clusters superiorly and temporally a thin sector inferiority Degeneration pattern No degeneration No degeneration Scattered “degeneration dust” and some 1% paraphenylene diamine solution–verified degeneration P (fiber population vs ...† ,.001 ,.001 normal optic nerve) *Axon + myelin.
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