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Thyroidal hemiagenesis
Thyroidal Herniagenesis
Juan Piera, MD, FACS, Barcelona, Spain Jorge Garriga, MD, Barcelona, Spain Rkado
Calabuig, MD, Barcelona, Spain
Domingo Bargallo, MD, Barcelona, Spain
Thyroidal herniagenesis is a rare congenital anomaly in which one of the thyroidal lobes fails to develop. In their exclusive bibliographic survey, Melnick and Stemkowski [1] encountered 94 cases, the vast majority reported in the last two decades. Their own personal experience added four new cases to the list. The first description of thyroidal herniagenesis was made in 1886 by Handfield-Jones [I]. In 1918, Dubs published the first surgical case and in 1965, Blahd obtained the first gammagraphic image [I]. Finally, in 1970, Hamburger and Hamburger [2] first used the stimulation test with thyroid stimulating hormone to reach a clinical diagnosis. Since 1981, we have found five more reported cases [3,4], bringing the total to just over 100. We have not found any case of thyroidal herniagenesis in the Spanish literature. The purpose of this report is to present three cases of adenomatous goiter in which agenesia of the contralateral lobe was found, one in the right and two in the left side. Case Reports Case 1. A 40 year old woman was seen for a mass in the anterior cervical region that was present for the previous 2 months. The physical examination revealed a painless elastic nodule 2 cm in diameter on the right thyroidal lobe. A technetium 99 scan of the thyroid showed low uptake on the lower half of the right lobe and no activity of the left lobe (Figure 1). The radioactive iodine uptake was 37 percent in 24 hours. The thyroxine level was 13 pmol/liter and the thyroid stimulating hormone level, less than 1 mu/liter. A thvroid stimulation test was performed (Figure 2) and the only change observed was an increase in the From the Department of Surgery, Hospital de la Santa Cruz y San Pablo and Hospital Militar, Universidad Autonoma, Barcelona, Spain. Requests for reprints should be addressed to Juan Piera. MD, Servicio de Cirug/a General, Hospital de la Santa Cruz y San Pablo, Avda. San Antonio M. Claret 167, 06025 Barcelona, Spain.
Volume 151, March 1966
Figure 1. Thyroid scintiscan reveallng right lobe with low uptake on the lower half, and tack ot activity on the left side.
radioactive iodine uptake to 83 percent after 24 hours. The preoperative diagnosis of a cold nodule on the right thvroidal lobe with hemiagenesis of the left lobe was confirmed at operation. The isthmus and left lobe were absent. The left recurrent laryngeal nerve appeared along its normal course and the parathyroid glands were not
419
Piera et al
Figure 2. Thyroid scintlscan after thyroid hormone stimulation. Left lobe shows no actlvity.
examined. A partial thyroidectomy was performed. The pathologic diagnosis was adenomatous nodular goiter. The postoperative scintiscan (Figure 3) showed a small remnant of the right lobe. The radioactive iodine uptake was 27 percent at 2 hours and 40 percent at 24 hours. The patient was discharged with a regimen of L-thyroxine. Case 2. A 54 year old woman complained of a lump in the neck of 15 years duration. Physical examination revealed a painless right nodule, and a thyroid scintiscan showed a large right lobe with a cold nodule 3 cm in diameter and a lack of activity on the left side. The thyroid stimulation test was not performed. At operation, the left lobe and isthmus were not found, and the left recurrent laryngeal nerve and parathyroid glands were in place. A subtotal right thyroidectomy was performed. Pathologic examination revealed a simple adenomatous goiter. The patient was discharged with Lthyroxine substitutive treatment. Case 3. A 53 year old woman presented with a left nodular goiter that was diagnosed as functionally active due to a lack of captation of iodine 131 in the right lobe and no changes in the thyroid stimulation test results. In 1976, she underwent a left thyroidectomy and the right side was not explored. The pathologic diagnosis was follicular adenoma. In 1983, the patient was seen at our center with recurrence of the left goiter which presented with the same clinical, including scintigraphic, features as in the past. At operation, we found a left nodular goiter with a small remnant of normal thyroidal tissue in the pyramidal area, and a complete absence of the right lobe and isthmus. A subtotal left thyroidectomy was performed. The patient was discharged with substitutive hormonal treatment. Comments
Thyroid herniagenesis is a rare anomaly. Hamburger and Hamburger [2] described 4 cases detected in 7,000 thyroid scanning examinations and Harada and Nishikawa [5] detected 7 cases in 12,456 pathologic thyroid sections. Among the observed cases, there is a female predominance of 3:1, and the frequency of agenesia is approximately four times 420
Figure 3. Postoperatlve scintlscan. Remaining normal port/on of the right lobe.
greater in the left lobe than in the right. The cause is unknown, but it has been suggested that the condition might only be an extreme degree of the asymmetry that is normal in all bilateral symmetric organs [3]. The isthmus is present in 50 percent of reported cases. The extremely high percentage of pathologic abnormalities associated with herniagenesis is noteworthy, but this is probably due to the fact that the search for such abnormalities is what leads to the discovery of the malformation which is asymptomatic by itself. There is a slight predominance of hyperthyroidism, but we also found follicular and papillar neoplasms and colloidal or adenomatous goiters in the literature, as seen in our three patients. There are several examples of herniagenesis with a normal contralateral lobe, but only four cases have been demonstrated histologically and in three others there has been scintigraphic evidence with functional exploration of the thyroid gland [6-81. In one case [9], ignorance of this anomaly led to an unnecessary operation. The fact that Letonturier and Hazard [IO] and Harada and Nishikawa [5] found eight and seven cases, respectively, has led us to suspect that this anomaly is not that rare and that there have probably been other cases which were either not published or incorrectly diagnosed. Summary
Three cases of thyroidal herniagenesis have been reported. The patients were euthyroid and presented with adenomatous goiters; one uninodular and the others multinodular in the contralateral lobe. In The American Journal of Surgery
Thyroidal Hemiagenesis
two cases the diagnosis was established by the thyroid stimulation test and confirmed at operation. In one case, this test was not performed and the hemiagenesia was discovered at routine surgical exploration of the scintigraphically absent lobe. From our cases and review of the literature we have concluded that the anomaly is usually discovered while searching for a contralateral pathologic abnormality, a thyroid stimulation test is essential for a preoperative suspicion, it occurs more frequently among women and in the left lobe, and ours are the first cases reported from Spain. References 1. Melnick JC, Stemkowski PE. Thyroid herniagenesis (hockey stick sign): a review of the world literature and report of four cases. J Clin Endocrinol Metab 1981;52:247-51.
Volume 151, March 1986
2. Hamburger JI, Hamburger SW. Thyroidal herniagenesis. Report of a case and comments on clinical implications. Arch Surg 1970;100:319-20, 3. Mortimer PS, Tomlinson IW, Rosenthal D. Hemiaplasia of the thyroid with thyrotoxicosis. J Clin Endocrinol Metab 1981; 52: 152-5. 4. Matsumara LK, Russo EMK, Dib SA, et al. Hemiagenesis of the thyroid gland and T3 hyperthyroidism. Postgrad Med J 1982;58:244-6. 5. Harada T, Nishikawa Y, Ito K. Aplasia of one thyroid lobe. Am J Surg 1972;124:617-9. 6. TashimaCK, Lee WY, Leong A. Agenesis of the thyroid. JAMA 1973;244:1761-2. 7. Russoto JA, Bayar JA. Thyroid hemiagenesis. J Nucl Med 1970;12:186-7. 8. Burman KD, Adler RA, Wartfsky L. Hemiagenesis of the thyroid gland. Am J Med 1975;58:143-6. 9. Goliger RC. Thyroid hemiagenesis with goiter. JAMA 1973; 224: 128. 10. Letontourier PH, Hazard J, Tourneur R, et al. Hemiagenesie thyroidienne (lobe thyroidienne unique). Presse Med 1979; 15:1227-g.
421
Juan Piera, MD, FACS, Barcelona, Spain Jorge Garriga, MD, Barcelona, Spain Rkado
Calabuig, MD, Barcelona, Spain
Domingo Bargallo, MD, Barcelona, Spain
Thyroidal herniagenesis is a rare congenital anomaly in which one of the thyroidal lobes fails to develop. In their exclusive bibliographic survey, Melnick and Stemkowski [1] encountered 94 cases, the vast majority reported in the last two decades. Their own personal experience added four new cases to the list. The first description of thyroidal herniagenesis was made in 1886 by Handfield-Jones [I]. In 1918, Dubs published the first surgical case and in 1965, Blahd obtained the first gammagraphic image [I]. Finally, in 1970, Hamburger and Hamburger [2] first used the stimulation test with thyroid stimulating hormone to reach a clinical diagnosis. Since 1981, we have found five more reported cases [3,4], bringing the total to just over 100. We have not found any case of thyroidal herniagenesis in the Spanish literature. The purpose of this report is to present three cases of adenomatous goiter in which agenesia of the contralateral lobe was found, one in the right and two in the left side. Case Reports Case 1. A 40 year old woman was seen for a mass in the anterior cervical region that was present for the previous 2 months. The physical examination revealed a painless elastic nodule 2 cm in diameter on the right thyroidal lobe. A technetium 99 scan of the thyroid showed low uptake on the lower half of the right lobe and no activity of the left lobe (Figure 1). The radioactive iodine uptake was 37 percent in 24 hours. The thyroxine level was 13 pmol/liter and the thyroid stimulating hormone level, less than 1 mu/liter. A thvroid stimulation test was performed (Figure 2) and the only change observed was an increase in the From the Department of Surgery, Hospital de la Santa Cruz y San Pablo and Hospital Militar, Universidad Autonoma, Barcelona, Spain. Requests for reprints should be addressed to Juan Piera. MD, Servicio de Cirug/a General, Hospital de la Santa Cruz y San Pablo, Avda. San Antonio M. Claret 167, 06025 Barcelona, Spain.
Volume 151, March 1966
Figure 1. Thyroid scintiscan reveallng right lobe with low uptake on the lower half, and tack ot activity on the left side.
radioactive iodine uptake to 83 percent after 24 hours. The preoperative diagnosis of a cold nodule on the right thvroidal lobe with hemiagenesis of the left lobe was confirmed at operation. The isthmus and left lobe were absent. The left recurrent laryngeal nerve appeared along its normal course and the parathyroid glands were not
419
Piera et al
Figure 2. Thyroid scintlscan after thyroid hormone stimulation. Left lobe shows no actlvity.
examined. A partial thyroidectomy was performed. The pathologic diagnosis was adenomatous nodular goiter. The postoperative scintiscan (Figure 3) showed a small remnant of the right lobe. The radioactive iodine uptake was 27 percent at 2 hours and 40 percent at 24 hours. The patient was discharged with a regimen of L-thyroxine. Case 2. A 54 year old woman complained of a lump in the neck of 15 years duration. Physical examination revealed a painless right nodule, and a thyroid scintiscan showed a large right lobe with a cold nodule 3 cm in diameter and a lack of activity on the left side. The thyroid stimulation test was not performed. At operation, the left lobe and isthmus were not found, and the left recurrent laryngeal nerve and parathyroid glands were in place. A subtotal right thyroidectomy was performed. Pathologic examination revealed a simple adenomatous goiter. The patient was discharged with Lthyroxine substitutive treatment. Case 3. A 53 year old woman presented with a left nodular goiter that was diagnosed as functionally active due to a lack of captation of iodine 131 in the right lobe and no changes in the thyroid stimulation test results. In 1976, she underwent a left thyroidectomy and the right side was not explored. The pathologic diagnosis was follicular adenoma. In 1983, the patient was seen at our center with recurrence of the left goiter which presented with the same clinical, including scintigraphic, features as in the past. At operation, we found a left nodular goiter with a small remnant of normal thyroidal tissue in the pyramidal area, and a complete absence of the right lobe and isthmus. A subtotal left thyroidectomy was performed. The patient was discharged with substitutive hormonal treatment. Comments
Thyroid herniagenesis is a rare anomaly. Hamburger and Hamburger [2] described 4 cases detected in 7,000 thyroid scanning examinations and Harada and Nishikawa [5] detected 7 cases in 12,456 pathologic thyroid sections. Among the observed cases, there is a female predominance of 3:1, and the frequency of agenesia is approximately four times 420
Figure 3. Postoperatlve scintlscan. Remaining normal port/on of the right lobe.
greater in the left lobe than in the right. The cause is unknown, but it has been suggested that the condition might only be an extreme degree of the asymmetry that is normal in all bilateral symmetric organs [3]. The isthmus is present in 50 percent of reported cases. The extremely high percentage of pathologic abnormalities associated with herniagenesis is noteworthy, but this is probably due to the fact that the search for such abnormalities is what leads to the discovery of the malformation which is asymptomatic by itself. There is a slight predominance of hyperthyroidism, but we also found follicular and papillar neoplasms and colloidal or adenomatous goiters in the literature, as seen in our three patients. There are several examples of herniagenesis with a normal contralateral lobe, but only four cases have been demonstrated histologically and in three others there has been scintigraphic evidence with functional exploration of the thyroid gland [6-81. In one case [9], ignorance of this anomaly led to an unnecessary operation. The fact that Letonturier and Hazard [IO] and Harada and Nishikawa [5] found eight and seven cases, respectively, has led us to suspect that this anomaly is not that rare and that there have probably been other cases which were either not published or incorrectly diagnosed. Summary
Three cases of thyroidal herniagenesis have been reported. The patients were euthyroid and presented with adenomatous goiters; one uninodular and the others multinodular in the contralateral lobe. In The American Journal of Surgery
Thyroidal Hemiagenesis
two cases the diagnosis was established by the thyroid stimulation test and confirmed at operation. In one case, this test was not performed and the hemiagenesia was discovered at routine surgical exploration of the scintigraphically absent lobe. From our cases and review of the literature we have concluded that the anomaly is usually discovered while searching for a contralateral pathologic abnormality, a thyroid stimulation test is essential for a preoperative suspicion, it occurs more frequently among women and in the left lobe, and ours are the first cases reported from Spain. References 1. Melnick JC, Stemkowski PE. Thyroid herniagenesis (hockey stick sign): a review of the world literature and report of four cases. J Clin Endocrinol Metab 1981;52:247-51.
Volume 151, March 1986
2. Hamburger JI, Hamburger SW. Thyroidal herniagenesis. Report of a case and comments on clinical implications. Arch Surg 1970;100:319-20, 3. Mortimer PS, Tomlinson IW, Rosenthal D. Hemiaplasia of the thyroid with thyrotoxicosis. J Clin Endocrinol Metab 1981; 52: 152-5. 4. Matsumara LK, Russo EMK, Dib SA, et al. Hemiagenesis of the thyroid gland and T3 hyperthyroidism. Postgrad Med J 1982;58:244-6. 5. Harada T, Nishikawa Y, Ito K. Aplasia of one thyroid lobe. Am J Surg 1972;124:617-9. 6. TashimaCK, Lee WY, Leong A. Agenesis of the thyroid. JAMA 1973;244:1761-2. 7. Russoto JA, Bayar JA. Thyroid hemiagenesis. J Nucl Med 1970;12:186-7. 8. Burman KD, Adler RA, Wartfsky L. Hemiagenesis of the thyroid gland. Am J Med 1975;58:143-6. 9. Goliger RC. Thyroid hemiagenesis with goiter. JAMA 1973; 224: 128. 10. Letontourier PH, Hazard J, Tourneur R, et al. Hemiagenesie thyroidienne (lobe thyroidienne unique). Presse Med 1979; 15:1227-g.
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