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Biomedical subjects

P Henkind

Publications and source records attributed to P Henkind.

At least 37 records · Page 2Linked to original sources

Ocular findings in the facioauriculovertebral sequence (Goldenhar-Gorlin syndrome).

We reviewed the ocular findings in 57 consecutive patients with the facioauriculovertebral sequence (Goldenhar-Gorlin syndrome). Epibulbar choristomas were detected in 18 cases (32%), a much lower occurrence than reported previously. Various motility disorders (11 cases, 19%), blepharoptosis or narrow palpebral fissures (seven cases, 12%), eyelid colobomas (six cases, 11%), and lacrimal drainage system anomalies (six cases, 11%) were more frequent than previously noted. These ocular findings were more common in the patients with epibulbar choristomas. Of the various features of the Goldenhar-Gorlin syndrome (skin tags, microtia, hemifacial microsomia, and vertebral anomalies), only skin tags correlated positively with the laterality of epibulbar choristomas. Preauricular and facial tags represent choristomas, explaining their association with epibulbar choristomas and the laterality they share.

Abnormalities, Multiple↗

Momentary fluctuations of intraocular pressure in normal and glaucomatous eyes.

To examine the range of momentary fluctuations of intraocular pressure caused by vascular pulsations in 42 glaucomatous and 18 normal eyes, we obtained consecutive intraocular pressure measurements at hourly intervals with a noncontact air tonometer. Glaucomatous eyes had a significantly (P less than .001) greater range of intraocular pressure measurements on consecutive readings than normal eyes (4.4 +/- 1.4 mm Hg vs 2.7 +/- 0.7 mm Hg). The frequency of consecutive intraocular pressure readings exceeding a spread of 4 mm Hg was significantly increased in glaucomatous eyes. The range of intraocular pressure measurements was greater even in those glaucomatous eyes with mean intraocular pressures within the normal range. There was a positive correlation between mean intraocular pressure and mean range in glaucomatous eyes, but not in normal eyes. In both glaucomatous and normal eyes, there was no significant correlation between age and mean range.

Adolescent↗

Uveal melanoma presenting after cataract extraction with intraocular lens implantation.

Four patients underwent cataract extraction with implantation of intraocular lenses. Preoperative ultrasonography did not include a comprehensive diagnostic B-scan analysis. Postoperative examination revealed uveal melanomas. The clinical presentations as well as the histopathologic findings of the two enucleated eyes are discussed. These cases emphasize the necessity for B-scan ultrasonography prior to cataract surgery when media opacity prevents adequate visualization of the fundus.

Aged↗

An experimental model of carotid artery occlusive disease.

To develop an animal model for acute carotid artery occlusion suitable for studying the retinal sequelae of this disease, we performed bilateral and unilateral common carotid artery ligations on mature pigmented rats. Bilateral ligation consistently resulted in a characteristic pathologic retinal appearance, paralleling the retinopathy of carotid artery occlusive disease in humans, demonstrated by fundus photography and fluorescein angiography. Any abnormalities found in the rats with unilateral ligations were inconsistent and less severe, although fluorescein angiography proved to be more sensitive than ophthalmoscopy for detecting these subtle changes. Pepsin-trypsin retinal digests disclosed extensive disruption of the microvasculature in the eyes of bilaterally ligated animals. This model should prove to be useful in examining the reversibility of the ocular findings as well as evaluating central nervous system abnormalities after carotid artery occlusion.

Animals↗

Choroidal neovascularization can digest Bruch's membrane. A prior break is not essential.

A novel form of retinal phototoxicity has facilitated investigation of the effect of retinal pigment epithelial (RPE) damage on the choriocapillaris of the rat. In response to isolated RPE injury, the choriocapillaris buds, digests the endothelial basement membrane, and projects pseudopodia that erode Bruch's membrane. The development of subretinal neovascularization in one of the rats demonstrates that breaks in Bruch's membrane may result from this lytic process and that neither degeneration of Bruch's membrane nor a preexisting break is essential for subretinal neovascularization to occur.

Animals↗

Experimental carotid occlusion: funduscopic and fluorescein angiographic findings.

A characteristic fundus picture was consistently produced following acute bilateral common carotid artery ligation in mature rats, reminiscent of human carotid occlusive disease. Two days after ligation it consisted of dilatation and tortuosity of retinal veins, blurring and swelling of the optic disc, retinal whitening primarily along the venous distribution, and straightening of retinal arteries. Fluorescein angiography showed hyperfluorescence of the disc, delay in the rate of retinal arterial and venous filling, venous dilatation, disc oedema, disruption of the retinal capillary bed pattern, and late peripapillary staining/leakage. This pattern was not seen in rats which underwent acute unilateral ligation, although some mild changes were seen on fluorescein angiography. The vascular alterations seemed to regress spontaneously within one week. A peripapillary 'halo' and a granular-appearing nerve fibre layer developed later, exclusively in bilaterally ligated animals.

Animals↗

RPE destruction causes choriocapillary atrophy.

The authors have obtained evidence that destruction of the retinal pigment epithelium (RPE) causes choriocapillaris (CC) atrophy. The observations led us to hypothesize that the RPE modulates CC structure and function. Rabbits received injections of sodium iodate, which selectively destroyed the RPE. The authors killed the rabbits at various times after iodate and examined the RPE and CC by fluorescein angiography, fundus photography, and light and electron microscopy. Fluorescein angiography and fundus photography revealed a pattern of retinopathy similar to that described by other investigators, eg, blood-retinal barrier breakdown and the patchy nature of the RPE/CC degeneration. One week after injection of iodate, the RPE transformed into a mixture of flattened, depigmented cells and plump, highly pigmented ones lying along Bruch's membrane. The CC appeared normal by light microscopy, but electron microscopy revealed changes indicating CC atrophy: degenerating endothelial cells (EC), EC that appeared normal but had reduced numbers of fenestrae, and pericapillary basal laminae that looped away from the endothelium, as if the latter had shrunk. One month after iodate, patches of Bruch's membrane were devoid of RPE, which was replaced by scar tissue. The CC was markedly atrophic over these patches, having reduced numbers of profiles and smaller lumina in those which remained. The CC appeared normal over areas where RPE remained. Eleven weeks after iodate, the light microscopic picture parallelled that seen 1 month after injection, but the patchy RPE degeneration was more extensive. By electron microscopy, the CC profiles over areas devoid of RPE showed severe atrophy. Degenerating EC were more numerous. EC adjacent to areas of RPE loss had few or no fenestrae. Here, capillaries were encased in dense, collagenous, connective tissue, unlike the CC of normal rabbits. These changes were not seen where the RPE still covered Bruch's membrane. These observations suggest that RPE modulates CC structure and function. The authors propose that a diffusible vascular modulating factor produced by RPE cells does this.

Animals↗

Technology: its role in our conception of glaucoma. The 35th Mark J. Schoenberg lecture.

Glaucoma is a medical notion encompassing many diverse entities. Our present conception of glaucoma is rooted in four major technological innovations: ophthalmoscopy, tonometry, perimetry, and gonioscopy. The results of our findings from these modalities determine how we diagnose and plan management strategies. In essence, we are bound to a series of numbers or values useful in clinical practice but which tell us little about the pathogenetic factor(s) causing glaucoma.

Glaucoma↗

The relationship between retinal pigment epithelium and the choriocapillaris.

Absence of the choriocapillaris (CC) was observed histopathologically in human eyes with retinitis pigmentosa (RP). It was noted in areas where the overlying retinal pigment epithelium (RPE) was absent. Analysis of our material, as well as a review of the literature dealing with RP and related entities, as well as a number of other fundus disorders, suggested that the disappearance of the CC is secondary to REP degeneration. An experimental model using intravenous sodium iodate produced a similar sequence of events in the rabbit. We suggest that the RPE modulates structure and function of the CC. There is possibly a diffusable biochemical vascular modulation factor (VMF) which is required for normal CC fenestrae formation and maintenance, which is liberated by the normal RPE, and acts upon the vascular endothelium. Lack of VME causes the endothelium to lose its fenestrae and the vessel to occlude. This RPE-CC interaction is a further facet of the RPE which requires exploration in depth.

Aged↗

Rhabdomyosarcoma and late malignant melanoma of the orbit.

Forty-five years following surgical excision and radiation for a childhood rhabdomyosarcoma of the left orbit, a patient with primary lymphedema developed an ipsilateral malignant melanoma of the anterior orbital tissue. This was excised, but a metastasis of the melanoma occurred in the contralateral upper lid. This is the first case report of treated rhabdomyosarcoma of the orbit followed by a second primary tumor occurring in the field of radiation.

Eyelid Neoplasms↗

Fluorescein angiographic patterns of iris melanocytic tumors.

Iris fluorescein angiography was performed on 23 patients with primary iris melanocytic tumors. Four angiographic patterns were identified, the first three of which were considered to be usually indicative of a benign lesion, based on clinical duration and follow-up, biopsy specimens, and stable patterns on repeated angiography. (1) Eleven moderately to heavily pigmented placoid lesions were angiographically silent, in that they failed to display either a tumor-associated vasculature or diffuse leakage. (2) Seven generally nonpigmented lesions demonstrated a quasi-geometric filigree vascular network, approximating the caliber of the normal radial vasculature. These tumor-associated vessels fluoresced early and in synchrony with the appearance of dye in the radial vasculature from which they probably were derived, but they diffusely and confluently leaked fluorescein in the late phases of the angiogram. (3) Three brown or variably pigmented lesions manifested a mixed angiographic pattern, combining features of groups 1 and 2. (4) The final angiographic pattern was comprised of two pathologically proved mixed spindle-epithelioid cell melanomas, each of which in part or in toto showed diffuse and eventually confluent fluorescence emanating from ill-defined vascular foci. One of these two lesions additionally exhibited a large area that was perfused late by irregular leashes of vessels. The ability to distinguish benign from malignant melanocytic lesions of the iris can be greatly enhanced by iris fluorescein angiography.

Adult↗

Pathology of retinitis pigmentosa.

Eyes from patients with retinitis pigmentosa were obtained at autopsy. They were processed in celloidin and examined by light microscopy. The earliest evidence of retinal degeneration occurred in the equatorial zone and then extended peripherally and centrally. In the eyes with the earliest involvement, a sequence could be demonstrated in the zone of transition from the less involved macula to the more degenerated retina at the equator. The probable order for the development of degenerative changes in our material appeared to be as follows: (1) migration of nuclei from the outer nuclear layer to the rod and cone layer and the outer plexiform layer; (2) degeneration and loss of photoreceptors and their nuclei in the outer nuclear layer; (3) loss of connecting fibers in the outer plexiform layer; (4) migration of the retinal pigment epithelium (RPE) into the retina, mainly around the blood vessels, but also as isolated balls and spots (this prominent feature, which characterizes the disease, is secondary and only follows the loss of the photoreceptors and their nuclei); (5) adhesion of the retinal to the retinal pigment epithelium or Bruch's membrane in spots or broad areas and; (6) possible transneuronal degeneration of some cells in the inner nuclear and ganglion cell layers. Gliosis of the disc was universal as was the presence of glial membranes from the disc extending over the posterior retina, especially prominent in the macular region. As the material was obtained from 16 to 35 years ago, we lack electrophysiologic and familial data and electron microscopy was not possible.

Adult↗

Vitelliform macular dystrophy and butterfly-shaped epithelial dystrophy: a continuum?

We report the case of 22-year-old white female who presented for a routine ocular examination and displayed Best's disease in her right eye and butterfly-shaped macular dystrophy in her left eye. These diagnoses were confirmed by fluorescein angiography, EOG, and ERG. The fact that these 2 disorders are present in one person suggests that they are only different manifestations of the same underlying disease process.

Adult↗

Bilateral corneal dermis-like choristomas; an X chromosome-linked disorder.

A male child was born with bilateral corneal opacification. His maternal uncle and a male first cousin of the patient's mother also had been born with identical bilateral corneal opacification that was pathologically proven to result from corneal dermoids. Histopathological examination of tissue removed from the present patient at the time of lamellar keratoplasty revealed thickened, keratinized epithelium, absent Bowman's membrane, and dense, irregular bands of highly vascularized collagenous connective tissue. No adnexal appendages were identified. The remainder of the ocular and general physical examination results was normal. In view of these clinical and histopathological similarities, the diagnosis of corneal dermis-like choristomas was made. To our knowledge, this report is the first to describe a family with X chromosome-linked recessive transmission of this condition.

Choristoma↗