Journal of Pediatric Ophthalmology and Strabismus

Hallermann-Streiff Syndrome Diagnosed from a Painting

James G Ravin, BA; Gerald P Hodge, BFA

Abstract

SINCE ANCIENT TIMES artists have observed details of the human figure and have been fascinated with recording human maladies. Thus, artists have inadvertently produced a valuable history of disease. The first known record of poliomyelitis, for example, appears in a Nineteenth Dynasty Egyptian (1350-1205 B.C.) limestone carving. Of similar importance is the first known evidence of tuberculosis in the Western Hemisphere, which is found in Mayan ceramics of the seventh to the tenth centuries. The great artists of the Renaissance were among the leading anatomists of their time. Succeeding artists maintained their interest in the human figure; this is evident in many portraits that show diagnosable disease states. The portraits of aehondroplastic and pituitary dwarfs by the sixteenth century Spanish master Velazquez are among the most famous paintings of medicine in art.

Today artists incline less toward rigidly organized forms and more toward faddish styles such as Op, Pop, and LSD art. It is unusual to find a successful artist who is concerned only with realism. Instead, artists have altered their techniques and subject matter to adjust to the increasing complexities of twentieth century life. Psychological art received great impetus in the late nineteenth century from the Norwegian painter, Edvard Munch. His paintings of fear, anxiety, and despair are direct antecedents to twentieth century works concerned with psychologic and social questions. Artistic concepts now exceed the limits of naturalistic portrayal of the past.

One artist who has successfully combined the well-established realistic style with the newer psychologic approach is the contemporary Mexican artist, Rafael Coronel. He has a probing insight that has produced psychologic studies in depth to an extent seldom seen in realistic art. His subjects are frequently common people whose faces reflect a resignation to a life that holds little hope. John Canaday, the noted art critic of The New York Times states:

"... The people Coronel paints wait quietly on the canvas, regarding the spectator as if not yet willing to admit him into their worlds, but ready to examine him as a desirable entrant and hopeful of discovering a sympathetic response. The mood is at once one of isolation and hope, based on a conviction of goodness."1

Born in 1932, in Zacatecas, Mexico, Coronel is one of the outstanding young artists who follow the creative Mexican tradition. He is a versatile portraitist and is one of his country's best known exponents of figurative expressionism. Coronel is the son-in-law of Diego Rivera, Mexico's most famous artist; his older brother is also well known in the field of art. Coronel studied architecture at the college at Zacatecas, and also studied at the Instituto Nacional de Bellas Artes, Mexico City. His paintings are widely acclaimed and many are included in important collections throughout the world.

Several of Coronel's paintings of the early nineteen-sixties have dealt with people who have ocular abnormalities. La Niña (The Child, Fig. 1) is particularly interesting from a medical aspect; the "patient" may be identified as having the rather rare Hallermann-StreifE syndrome. This syndrome, also known as Dyscephalia Mandibulo-Oeulo-Facialis, is a developmental anomaly of the face and skull first described by Hallermann2 in 1948, and Streif!3 in 1950. Many of the features of the syndrome are evident in La Niña: 4·5 (1) Skull malformations evident in the girl include a relatively expansive cranium in comparison to the underdeveloped facial bones, and prominent frontal and parietal bones. Brachycephaly is also a typical feature, but this cannot be determined from the painting. (2) Congenital anomalies of the eyes seen in La Niña include microphthalmia, microcornea, strabismus, and an antimongoloid palpebral fissure. Congenital cataracts that may rupture spontaneously,…

SINCE ANCIENT TIMES artists have observed details of the human figure and have been fascinated with recording human maladies. Thus, artists have inadvertently produced a valuable history of disease. The first known record of poliomyelitis, for example, appears in a Nineteenth Dynasty Egyptian (1350-1205 B.C.) limestone carving. Of similar importance is the first known evidence of tuberculosis in the Western Hemisphere, which is found in Mayan ceramics of the seventh to the tenth centuries. The great artists of the Renaissance were among the leading anatomists of their time. Succeeding artists maintained their interest in the human figure; this is evident in many portraits that show diagnosable disease states. The portraits of aehondroplastic and pituitary dwarfs by the sixteenth century Spanish master Velazquez are among the most famous paintings of medicine in art.

Today artists incline less toward rigidly organized forms and more toward faddish styles such as Op, Pop, and LSD art. It is unusual to find a successful artist who is concerned only with realism. Instead, artists have altered their techniques and subject matter to adjust to the increasing complexities of twentieth century life. Psychological art received great impetus in the late nineteenth century from the Norwegian painter, Edvard Munch. His paintings of fear, anxiety, and despair are direct antecedents to twentieth century works concerned with psychologic and social questions. Artistic concepts now exceed the limits of naturalistic portrayal of the past.

One artist who has successfully combined the well-established realistic style with the newer psychologic approach is the contemporary Mexican artist, Rafael Coronel. He has a probing insight that has produced psychologic studies in depth to an extent seldom seen in realistic art. His subjects are frequently common people whose faces reflect a resignation to a life that holds little hope. John Canaday, the noted art critic of The New York Times states:

"... The people Coronel paints wait quietly on the canvas, regarding the spectator as if not yet willing to admit him into their worlds, but ready to examine him as a desirable entrant and hopeful of discovering a sympathetic response. The mood is at once one of isolation and hope, based on a conviction of goodness."1

Fig. 1: La Niña, Raphael Coronel (1932- ).

Fig. 1: La Niña, Raphael Coronel (1932- ).

Born in 1932, in Zacatecas, Mexico, Coronel is one of the outstanding young artists who follow the creative Mexican tradition. He is a versatile portraitist and is one of his country's best known exponents of figurative expressionism. Coronel is the son-in-law of Diego Rivera, Mexico's most famous artist; his older brother is also well known in the field of art. Coronel studied architecture at the college at Zacatecas, and also studied at the Instituto Nacional de Bellas Artes, Mexico City. His paintings are widely acclaimed and many are included in important collections throughout the world.

Several of Coronel's paintings of the early nineteen-sixties have dealt with people who have ocular abnormalities. La Niña (The Child, Fig. 1) is particularly interesting from a medical aspect; the "patient" may be identified as having the rather rare Hallermann-StreifE syndrome. This syndrome, also known as Dyscephalia Mandibulo-Oeulo-Facialis, is a developmental anomaly of the face and skull first described by Hallermann2 in 1948, and Streif!3 in 1950. Many of the features of the syndrome are evident in La Niña: 4·5 (1) Skull malformations evident in the girl include a relatively expansive cranium in comparison to the underdeveloped facial bones, and prominent frontal and parietal bones. Brachycephaly is also a typical feature, but this cannot be determined from the painting. (2) Congenital anomalies of the eyes seen in La Niña include microphthalmia, microcornea, strabismus, and an antimongoloid palpebral fissure. Congenital cataracts that may rupture spontaneously, glaucoma, and nystagmus have also been reported. (3) The facial malformations are striking. The. nose is small and beaked and the nasofrontal angle is sharp, creating the typical "bird-faced" appearance. The girl also has a deviated nasal septum.

The skin of affected individuals is thin and taut, with prominent blood vessels, especially over the nose. The mandible is hypoplastic and as a result the ears are low-set. The mouth is small and the lips are thin. Typically one expects to find that the teeth are irregular, often fully erupted at birth. The palate is highly arched (ogive) and the tongue is small. (4) Localized alopecia is a part of the syndrome. (La Niña has no eyebrows and there is a suggestion of frontal baldness.) (5) Retardation of mental and motor development are usual findings. La Niña is apparently restrained by a straightjacket and her countenance is dull. (6) Other abnormalities often found, but which cannot be determined to exist from this painting, include dwarfism, sexual immaturity, and hyperextensibility of the joints.

La Niña is but a single example of a disease state diagnosed from art. Certainly today clinical photography is much more common and easier than artistic representation, but the latter approach still has its place. In La Niña the diagnosis may be made while the personality of the subject is emphasized.

References

1. Cakady, J.: "Mexican Modernism," The New York Times, April 25, 1965.

2. Hallermann, W.: Vogelgesicht und Cataracta Congenita. Klin. Mbl. Augenheilk. 113:315, 1948.

3. Streift, ?. ?.: Dysmorphie Mandibulo-Faciale (Tête d'Oiseau) et Altérations Oculaires. OphthalmoL·gica. 120: 79, 1950.

4. Falls, H. F. and Schull, W. J.: HallermannStreifE Syndrome. A Dyscephaly with Congenital Cataracts and Hypotrichosis. Arch. Ophth. 63:409, 1960.

5. Hoefnagel, D. and Bentrschke, ?.: Dyscephalia Mandibulo - Oculo - Facialis (Hallermann-StreifE Syndrome). Arch. Lus. Child. 40:57, 1965.

10.3928/0191-3913-19680501-13

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