Herniagenesis of the Thyroid Gland
KENNETH D. BURMAN,
M.D.
ROBERT A. ADLER, M.D.* LEONARD WARTOFSKY,
M.D.
Washington, D.C.
Three pajients with herniagenesis of the thyroid gland are described. One was clinically euthyroid, whereas the other two were more unusual in that one had coincident Graves’ disease with thyrotoxicosis, and one had primary myxedema. In all three cases the diagnosis of herniagenesis was established by the administration of thyroid-stimulating hormone (TSH). The literature on herniagenetic thyroid glands with and without associated thyroid disease is reviewed. Although the anomaly is uncommon, awareness and recognition of its existence may clarify an otherwise puzzling clinical thyroid evaluation, and thus possibly avert an unnecessary surgical procedure. Thyroidal herniagenesis is a congenital anomaly in which one lobe of the thyroid gland fails to develop. This condition may be discovered because of the co-occurrence of hyperthyroidism, hypothyroidism, colloid goiter or thyroid carcinoma. Although hemiagenesis of the thyroid is a benign condition, unawareness of its existence may lead to an incorrect diagnosis and the performance of unnecessary surgery. Our purpose here is to describe three patients with herniagenetic thyroid glands who presented to our clinic coincidentally within a 3 month period, and to briefly review the scant literature on this abnormality. The multiple potential clinical presentations of a patient with hemiagenesis are well illustrated by the patients we describe; one had overt thyrotoxicosis, one was euthyroid, and the third had moderately severe myxedema. CASE REPORTS
From the Department of Eindocrinofogyand Metabolism, Wafter Reed Army Institute of Research, Washington, DC. 20012. Requests for reprints should be addressed to Dr. Leonard Wartofsky, Department of Endocrinology and Metabolism, Ward 30. Watter Reed Army Institute of Research, Washington, DC. 20012. Manuscript accepted March 27, 1974. Present address: Department of Endocrinology 8 Metabolism, Fitzsimmons General Hospital, 12101 E. Colfax Avenue, Denver, Colorado 80240. l
Case 1 (Grave’s Disaase and Herniagenesis). This 41 year old woman (A.H.), was in good health until May 1973 when she began to notice increased nervousness, anxiety, diarrhea and heat intolerance. She denied weight loss, enlarging neck, hair loss, palpitations or proptosis. Her mother had previously had hyper-thyroidismthat remitted after she took antithyroid medications for an unknown period of time. There was no history of allergies, excessive iodine intake or ingestion of medications. On physical examination, blood pressure was 130/70 mm Hg, pulse rate lOO/min and regular, and respirations 18/min. The patient appeared nervous and anxious, her skin was warm and moist, and there was a fine tremor of the hands. There was no lid lag, proptosis or conjunctival injection. Examination of the thyroid gland revealed an enlarged, smooth and easily palpable right lobe and connecting isthmus which abruptly terminated 1 ( *VIpast the middle on the left. No thyroid tissue was palpable where the lul lobe should have been. No bruits or venous hums were heard over the r!:yrt~idgland. Examination of the lungs and heart disclosed no abnormal,,:. : Achilles tendon reflexes were brisk. At the time of initial presentaticll, :‘:e patient was thought to be moderately hyperthyroid on clinical grow,,
January 1975
The American Jou: ,ral of Medicine
Volume 58
143
THYROIDAL HEMIAGENESIS-BURMAN
ET AL
palpable where the left lobe normally lies. Triiodothyronine resin uptake (Abbott, Triosorb) was 25 per cent (normal 25 to 35 per cent) and serum thyroxine (Abbott, Tetrasorb) was 5.4 pg/lOO ml (normal 4.5 to 8.5 pg/lOO ml). Serum TSH was
KENNETH D. BURMAN,
M.D.
ROBERT A. ADLER, M.D.* LEONARD WARTOFSKY,
M.D.
Washington, D.C.
Three pajients with herniagenesis of the thyroid gland are described. One was clinically euthyroid, whereas the other two were more unusual in that one had coincident Graves’ disease with thyrotoxicosis, and one had primary myxedema. In all three cases the diagnosis of herniagenesis was established by the administration of thyroid-stimulating hormone (TSH). The literature on herniagenetic thyroid glands with and without associated thyroid disease is reviewed. Although the anomaly is uncommon, awareness and recognition of its existence may clarify an otherwise puzzling clinical thyroid evaluation, and thus possibly avert an unnecessary surgical procedure. Thyroidal herniagenesis is a congenital anomaly in which one lobe of the thyroid gland fails to develop. This condition may be discovered because of the co-occurrence of hyperthyroidism, hypothyroidism, colloid goiter or thyroid carcinoma. Although hemiagenesis of the thyroid is a benign condition, unawareness of its existence may lead to an incorrect diagnosis and the performance of unnecessary surgery. Our purpose here is to describe three patients with herniagenetic thyroid glands who presented to our clinic coincidentally within a 3 month period, and to briefly review the scant literature on this abnormality. The multiple potential clinical presentations of a patient with hemiagenesis are well illustrated by the patients we describe; one had overt thyrotoxicosis, one was euthyroid, and the third had moderately severe myxedema. CASE REPORTS
From the Department of Eindocrinofogyand Metabolism, Wafter Reed Army Institute of Research, Washington, DC. 20012. Requests for reprints should be addressed to Dr. Leonard Wartofsky, Department of Endocrinology and Metabolism, Ward 30. Watter Reed Army Institute of Research, Washington, DC. 20012. Manuscript accepted March 27, 1974. Present address: Department of Endocrinology 8 Metabolism, Fitzsimmons General Hospital, 12101 E. Colfax Avenue, Denver, Colorado 80240. l
Case 1 (Grave’s Disaase and Herniagenesis). This 41 year old woman (A.H.), was in good health until May 1973 when she began to notice increased nervousness, anxiety, diarrhea and heat intolerance. She denied weight loss, enlarging neck, hair loss, palpitations or proptosis. Her mother had previously had hyper-thyroidismthat remitted after she took antithyroid medications for an unknown period of time. There was no history of allergies, excessive iodine intake or ingestion of medications. On physical examination, blood pressure was 130/70 mm Hg, pulse rate lOO/min and regular, and respirations 18/min. The patient appeared nervous and anxious, her skin was warm and moist, and there was a fine tremor of the hands. There was no lid lag, proptosis or conjunctival injection. Examination of the thyroid gland revealed an enlarged, smooth and easily palpable right lobe and connecting isthmus which abruptly terminated 1 ( *VIpast the middle on the left. No thyroid tissue was palpable where the lul lobe should have been. No bruits or venous hums were heard over the r!:yrt~idgland. Examination of the lungs and heart disclosed no abnormal,,:. : Achilles tendon reflexes were brisk. At the time of initial presentaticll, :‘:e patient was thought to be moderately hyperthyroid on clinical grow,,
January 1975
The American Jou: ,ral of Medicine
Volume 58
143
THYROIDAL HEMIAGENESIS-BURMAN
ET AL
palpable where the left lobe normally lies. Triiodothyronine resin uptake (Abbott, Triosorb) was 25 per cent (normal 25 to 35 per cent) and serum thyroxine (Abbott, Tetrasorb) was 5.4 pg/lOO ml (normal 4.5 to 8.5 pg/lOO ml). Serum TSH was
