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Facial profile of the Russell dwarf
Volume 81 Number 5
Letters to the Editor
1 03 5
Editorial correspondence
"Editorial Correspondence" or letters to the Editor relative to articles published in the JOURNAL or to topics of current interest are subject to critical review and to current editorial policy in respect to publication in part or in full.
F, ial profile q the R., dl d aq To the Editor: I have a number of disagreements with the report of Fitch and Pinsky.1 They do not mention my own paper written in 1963, which was the first in the American pediatric literature on the subject of the Russell dwarf. What is more disturbing is that the authors use the term "pseudohydrocephalus" which I was the first to apply to the Russell dwarf. 2 I have already outlined my arguments against the use of the "Russell-Silver" eponym in a previous Letter to the Editor, 8 and I will not repeat myself. However, the lateral facial profile of the Russell dwarf is distinctive, as emphasized by Fitch and Pinsky, and shown by the lateral views of my Cases 1 and 3. Since this profile is characteristic of the Russell, but not the Silver, dwarf, surely this is another argument for separating the Russell and Silver syndromes. The uniqueness of the lateral, facial profile in the Russell dwarf is secondary to the craniofacial disproportion which gives the patient the appearance of a large head (hence the term "pseudohydroeephalus") whereas, in truth, he has a normalsized head sitting atop a miniature face and body. This unique craniofacial disproportion of the Russell dwarf may be a function of age 8 as it is lacking in my oldest patient3 In 1963, I stated that pneumoencephalography was unnecessary in these patients but still in 1967,4 and again in 1971,~ we read of this procedure being performed. The Russell dwarf should be easily recognized by his "large" head and low birth weight. My final complaint (over which Fitch and Pinsky have no control) is the authors' state-
ment, "we are not aware of any comment on the appearance of the eyes, which seem to be prominent due to relatively" wide palpebral fissures, and the smallness of the face." Unfortunately for the poor reader, two the four patients have their eyes covered in the photographs.
Glenn G. Szalay, M.D. Kaiser Foundation Hospitals Harbor City, Calif. REFERENCES
1. Fitch, N., and Pinsky, L.: The lateral facial profile of the Silver-Russell dwarf, J. PEDIATg. 80: 827, 1972. 2. Szalay, G. C.: Pseudohydrocephalus in dwarfs: The Russell dwarf, J. PEDIATR. 63" 622, 1963. 3. Szalay, G. C.: Russell dwarf versus Silver syndrome, ft. PEDIATR. 80: 1066, 1972. 4. Holden, J. D.: The Russell-Silver's dwarf, Dev. Med. Child~Neurol. 9: 457, 1967. 5. Gareis, F. J., Smith, D. W., and Summltt, R. L.: The Russell-Silver syndrome without asymmetry, J. PEDIATR. 79: 775, 1971.
Reply To the Editor: We can understand Dr. Szalay's reaction to the omission of his paper 1 from our list of references. Several comments are in order. First, he is entitled to know that his was the first paper we referred to after suspecting the diagnosis in our infant patient. Second, "pseudohydrocephalus" has become part of the standard description of Russell dwarfism. Therefore, it is an oblique compliment to him that we felt free to employ the term without documenting his initial application of it. We support Dr. Szalay's statement that as the patients age they lose the "profile." That is why we sought to emphasize its diagnostic value in
Letters to the Editor
1 03 5
Editorial correspondence
"Editorial Correspondence" or letters to the Editor relative to articles published in the JOURNAL or to topics of current interest are subject to critical review and to current editorial policy in respect to publication in part or in full.
F, ial profile q the R., dl d aq To the Editor: I have a number of disagreements with the report of Fitch and Pinsky.1 They do not mention my own paper written in 1963, which was the first in the American pediatric literature on the subject of the Russell dwarf. What is more disturbing is that the authors use the term "pseudohydrocephalus" which I was the first to apply to the Russell dwarf. 2 I have already outlined my arguments against the use of the "Russell-Silver" eponym in a previous Letter to the Editor, 8 and I will not repeat myself. However, the lateral facial profile of the Russell dwarf is distinctive, as emphasized by Fitch and Pinsky, and shown by the lateral views of my Cases 1 and 3. Since this profile is characteristic of the Russell, but not the Silver, dwarf, surely this is another argument for separating the Russell and Silver syndromes. The uniqueness of the lateral, facial profile in the Russell dwarf is secondary to the craniofacial disproportion which gives the patient the appearance of a large head (hence the term "pseudohydroeephalus") whereas, in truth, he has a normalsized head sitting atop a miniature face and body. This unique craniofacial disproportion of the Russell dwarf may be a function of age 8 as it is lacking in my oldest patient3 In 1963, I stated that pneumoencephalography was unnecessary in these patients but still in 1967,4 and again in 1971,~ we read of this procedure being performed. The Russell dwarf should be easily recognized by his "large" head and low birth weight. My final complaint (over which Fitch and Pinsky have no control) is the authors' state-
ment, "we are not aware of any comment on the appearance of the eyes, which seem to be prominent due to relatively" wide palpebral fissures, and the smallness of the face." Unfortunately for the poor reader, two the four patients have their eyes covered in the photographs.
Glenn G. Szalay, M.D. Kaiser Foundation Hospitals Harbor City, Calif. REFERENCES
1. Fitch, N., and Pinsky, L.: The lateral facial profile of the Silver-Russell dwarf, J. PEDIATg. 80: 827, 1972. 2. Szalay, G. C.: Pseudohydrocephalus in dwarfs: The Russell dwarf, J. PEDIATR. 63" 622, 1963. 3. Szalay, G. C.: Russell dwarf versus Silver syndrome, ft. PEDIATR. 80: 1066, 1972. 4. Holden, J. D.: The Russell-Silver's dwarf, Dev. Med. Child~Neurol. 9: 457, 1967. 5. Gareis, F. J., Smith, D. W., and Summltt, R. L.: The Russell-Silver syndrome without asymmetry, J. PEDIATR. 79: 775, 1971.
Reply To the Editor: We can understand Dr. Szalay's reaction to the omission of his paper 1 from our list of references. Several comments are in order. First, he is entitled to know that his was the first paper we referred to after suspecting the diagnosis in our infant patient. Second, "pseudohydrocephalus" has become part of the standard description of Russell dwarfism. Therefore, it is an oblique compliment to him that we felt free to employ the term without documenting his initial application of it. We support Dr. Szalay's statement that as the patients age they lose the "profile." That is why we sought to emphasize its diagnostic value in