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Vol 43 # 2 June 2011 - Kma.org.kw

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<strong>June</strong> <strong>2011</strong><br />

KUWAIT MEDICAL JOURNAL 149<br />

readily demonstrate uptake in the case of suppression<br />

of involved lobe in an autonomously functioning<br />

nodule. Thyroid scan in a patient with hemiagenesis<br />

is quite characteristic and a hockey stick sign may be<br />

apparent. The increased functional burden caused by<br />

the hemiagenetic gland would promote neoplasia.<br />

However, the relevance of this is questionable since<br />

all the patients with thyroid hemiagenesis whose TSH<br />

levels were measured in Mariani’s series had normal<br />

TSH value [2] . Even then it is impossible to avoid the<br />

suggestion that long-standing elevated levels that can<br />

lead to enlargement of the lobe might have played a<br />

role in the development of thyroid carcinoma. Marshall<br />

was among the first clinicians to describe numerous<br />

anatomic variations of the thyroid, including the<br />

clinical entity of hemiagenesis in 1895 [5] . Melnick and<br />

Stemkowski described the hockey stick sign by imaging<br />

study in patients with thyroid hemiagenesis [12] . They<br />

also reported four patients and reviewed the world<br />

literature on the subject of thyroid hemiagenesis which<br />

revealed a total of 90 cases; however, only 17 out of<br />

these were reported in the American literature [8] . None<br />

of the four patients had thyroid cancer. Sheridan et al<br />

reported a patient with hemiagenesis and Hashimoto’s<br />

disease [13] . The authors identified and preserved the<br />

parathyroid glands in normal position on the side of the<br />

enlarged thyroid lobe. However, they did not identify<br />

the parathyroid on the agenic side. The information<br />

regarding the parathyroid on the agenic side is not well<br />

documented in the literature. Piera et al reported three<br />

cases of thyroid hemiagenesis in 1986 [14] ; however, they<br />

documented the normal presence of parathyroid on the<br />

side of the enlarged thyroid lobe only in one case. It is<br />

important for the thyroid surgeon undertaking surgery<br />

on a hemiagenic thyroid to appreciate the position of<br />

the parathyroid and to make every effort to preserve<br />

the parathyroid on the side of the thyroid lobectomy.<br />

McHenry et al recently reported seven patients with<br />

thyroid hemiagenesis - a collected experience of five<br />

physicians [15] . They reported four female and three<br />

male patients ranging in age from 17 to 58 years. The<br />

pathologies included follicular adenoma, Graves’<br />

disease, and nodular goiter. One patient had follicular<br />

carcinoma of the thyroid. They emphasized the need<br />

for preoperative recognition of thyroid hemiagenesis<br />

in order to make critical decisions regarding surgical<br />

intervention. McHenry et al suggested that all<br />

patients with thyroid hemiagenesis who do not have<br />

indications for surgery should have monitoring of their<br />

thyrotrophic hormone levels, treatment of thyrotrophic<br />

elevation with thyroid hormone, and careful follow-up<br />

evaluation for the development of neoplastic disease [15] .<br />

Our case is a unique one compared with cases reported<br />

in the literature; the patient was an adult male with<br />

absent right thyroid lobe (and not the left which is<br />

more common) with severe hypothyroidism instead of<br />

hyperthyroidism (which is more usual).<br />

CONCLUSION<br />

Recognition of this rare congenital anomaly is<br />

important to avoid unnecessary contralateral neck<br />

exploration with its potential morbidity and also to<br />

make sure that patients receive careful follow-up and<br />

appropriate therapy when necessary.<br />

REFERENCES<br />

1. Rajmil HO, Rodriguez-Espinosa, Soldevila J,<br />

Ordonez-Llanos J. Thyroid hemiagenesis in two<br />

sisters. J Endocrinol Invest 1984; 7:393-394.<br />

2. Spencer SL, Thomas AC. Hemiaplasia of the thyroid<br />

gland. Med J Aust 1949; 2:97.<br />

3. Mariani G, Molea N, Toni MG, Bianchi R. Thyroid<br />

hemiagenesis: a review of thirteen consecutive cases.<br />

J Nucl Med Allied Sci 1980; 24:183-187.<br />

4. Mikosch P, Gallowitsch HJ, Kresnik E, Molnar<br />

M, Gomez I, Lind P. Thyroid hemiagenesis in an<br />

endemic goiter area diagnosed by ultrasonography:<br />

report of sixteen patients. Thyroid 1999; 11:1075-<br />

1084.<br />

5. Marshall CF. Variations in the form of thyroid gland<br />

in man. J Anat Physiol 1895; 29:234-239.<br />

6. Harada T, Nishikawa Y, Ito K. Aplasia of one thyroid<br />

lobe. Am J Surg 1972; 124:617-619.<br />

7. Hamburger JI, Hamburger SW. Thyroidal<br />

hemiagenesis. Report of a case and comments on<br />

clinical ramifications. Arch Surg 1970; 100:319-320.<br />

8. Maganini J, Narendran K, Hinsdale, Hines.<br />

Hyperparathyroidism in a patient with thyroidal<br />

hemiagenesis. IMJ 1977; 151:368-370.<br />

9. Maiorana R, Carta A, Floriddia G, et al. Thyroid<br />

hemiagenesis: prevalence in normal children and<br />

effect on thyroid function. J Clin Endocrinol Metab<br />

2003; 88:1534-1536.<br />

10. Korpal-Szczyrska M, Kosiak W, Swieton D. Prevalence<br />

of thyroid hemiagenesis in an asymptomatic school<br />

children population. Thyroid 2008; 18:637-639.<br />

11. Shabana W, Delange F, Freson M, Osteaux M, De<br />

Schepper J. Prevalence of thyroid hemiagenesis:<br />

ultrasound screening in normal children. Eur J<br />

Pediatr 2000; 159:456-458.<br />

12 . Melnick NC, Stemkowski PE. Thyroid hemiagenesis<br />

(hockey stick sign): a review of the world literature<br />

and a report of four cases. J Clin Endocrinol Metab<br />

1981; 52:247-251.<br />

13 . Sheridan MF, Bruns AD, Burgess LP. Hemiagenesis<br />

of thyroid gland. Otolaryngol Head Neck Surg 1995;<br />

112:621-623.<br />

14. Piera J, Garriga J, Calabuig R, Bargallo D. Thyroid<br />

hemiagenesis. Am J Surg 1986; 151:419-421.<br />

15. McHenry CR, Walfish PG, Rosen IB, Lawrence AM,<br />

Paloyan E. Congenital thyroid hemiagenesis. Am<br />

Surg1995; 61:634-638.

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