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HEAD & NECK RADIOLOGY Table of Contents   
Year : 2008  |  Volume : 18  |  Issue : 4  |  Page : 313-315
Right-sided hemiagenesis of the thyroid lobe and isthmus: A case report


1 Department of Radiology, Manipal Teaching Hospital, PO Box: 341, Phulbari, Pokhara, Nepal
2 Department of Surgery, Manipal Teaching Hospital, PO Box: 341, Phulbari, Pokhara, Nepal

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   Abstract 

Unilateral or bilateral hypoplasia or agenesis of one or both thyroid lobes, with or without isthmic agenesis, is a rare developmental anomaly. Hemiagenesis of the left lobe is far commoner than of the right. Clinically, these patients may be euthyroid, hyperthyroid, or hypothyroid. Ultrasonography is usually able to diagnose this condition easily, as we demonstrate in this case report of a 37-year-old lady with an incidentally detected thyroid nodule who was found to have hemiagenesis of the right lobe and isthmus.

Keywords: Congenital anomalies; hemiagenesis; thyroid

How to cite this article:
Tiwari PK, Baxi M, Baxi J, Koirala D. Right-sided hemiagenesis of the thyroid lobe and isthmus: A case report. Indian J Radiol Imaging 2008;18:313-5

How to cite this URL:
Tiwari PK, Baxi M, Baxi J, Koirala D. Right-sided hemiagenesis of the thyroid lobe and isthmus: A case report. Indian J Radiol Imaging [serial online] 2008 [cited 2019 Nov 16];18:313-5. Available from: http://www.ijri.org/text.asp?2008/18/4/313/40958
Developmental anomalies of the thyroid are rare. They are usually a result of abnormal descent rather than of abnormal development. Hemiagenesis of either lobe, with or without agenesis of the isthmus, is very rare, with a prevalence rate of around 0.2% [1],[2] in asymptomatic children. The left side is the most commonly involved in hemiagenesis (80%). The isthmus may be absent in 40-50% of cases. [2],[3] Rarely, right-sided hemiagenesis may be encountered. The disorder is more common in females, the male : female ratio being 3:1. Thyroid function may be altered in 38-47% of patients. [3] USG plays an important role in diagnosis.

We present a rare case of hemiagenesis of the right lobe and isthmus of the thyroid.


   Case Report Top


A 37-year-old lady presented with bilateral cyclical mastalgia and benign breast disease, which was confirmed by USG and fine needle aspiration cytology. On clinical examination, there was an enlarged left lobe; a solitary thyroid nodule, measuring 1.5 × 1.0 cm in size was also found. The right lobe could not be felt. There was no past history of surgery in the region of the neck.

Free T 3 , free T 4 , and TSH on two occasions suggested a biochemically euthyroid state. USG showed multiple hypoechoic to isoechoic heterogeneous nodules [Figure 1] in the enlarged left lobe, the largest being 6.6 mm in diameter. Doppler showed increased vascularity in the left lobe. The isthmus and right lobe were not visualized [Figure 2]. CT scan of the neck and thyroid confirmed the agenetic right lobe and isthmus [Figure 3]. Fine needle cytology of the thyroid nodule showed a colloid nodule with cystic degeneration and hemorrhage.

The patient was reassured and was prescribed vitamin E supplementation for her mastalgia. Due to the mild symptoms and the increased vascularity of the left lobe, and in view of the possibility of developing hypothyroidism or hyperthyroidism in the future, 3-monthly follow-up with thyroid function tests and USG was advised.


   Discussion Top


Congenital thyroid anomalies are rare. They may be related to abnormal descent of the thyroid gland or to structural abnormalities in thyroid development, such as hypoplasia or hemiagenesis with or without isthmic agenesis.

A literature review in the year 2000 documented 256 cases of hemiagenesis. [4] We could find 51 new cases published between 2000 and 2007 in the English literature. Left-sided hemiagenesis is far commoner than right-sided hemiagenesis, [1],[2],[5],[6],[7],[8],[9],[10] with a left : right ratio of 4:1. The prevalence of this disorder, as documented in 2845 Belgian school children who were screened by USG for congenital thyroid anomalies, was found to be 0.2%. [11]

Though the etiology of hemiagenesis is not clearly known and most of the cases are sporadic, a few may be familial and there may be a genetic predisposition. [12],[13],[14],[15],[16] Although patients may have a normal thyroid lobe with euthyroidism, [1],[3] both hypothyroidism [13],[14] and hyperthyroidism, [4],[16],[17],[18] are known to occur. Other anomalies such as benign euthyroid adenoma, [19] thyroiditis, [20] multinodular goiter, [1],[2],[8],[21] papillary carcinoma, [7],[9],[22] and primary hyperparathyroidism [5] have also been reported. Our patient had a euthyroid colloid nodule. The associated cyclical mastalgia and bilateral fibroadenosis were most likely coincidental findings.

As in our case, most patients with hemiagenesis are diagnosed incidentally. [1],[2],[8],[11] USG is a useful modality to detect this anomaly. In endemic zones, there may be a high incidence of adenomatous nodules and colloid cysts in the single lobe. Malignancy always remains a cause for concern. Though a CT scan was performed in our patient, other modalities may not always be required to confirm the diagnosis of hemiagenesis, USG usually being diagnostic. [8],[11],[23],[24],[25],[26]

 
   References Top

1.Aslaner A, Aydin M, Ozdere A. Multinodular goiter with thyroid hemiagenesis: A case report and review of the literature. Acta Chir Belg 2005;105:528-30.  Back to cited text no. 1    
2.Karabay N, Comlekci A, Canda MS, Bayraktar F, Degirmenci B. Thyroid hemiagenesis with multinodular goiter: A case report and review of the literature. Endocr J 2003;50:409-13.  Back to cited text no. 2    
3.Vαzquez-Chαvez C, Acevedo-Rivera K, Sartorius C, Espinosa-Said L. Thyroid hemiagenesis: Report of 3 cases and review of the literature. Gac Med Mex 1989;125:395-9.  Back to cited text no. 3    
4.Veliz J, Pineda G. Thyroid hemiagenesis associated with Basedow-Graves disease: Report of a case. Rev Med Chil 2000;128:896-8.  Back to cited text no. 4    
5.Sakurai K, Amano S, Enomoto K, Matsuo S, Kitajima A. Primary hyperparathyroidism with thyroid hemiagenesis. Asian J Surg 2007;30:151-3.  Back to cited text no. 5    
6.Büyükdereli G, Guney IB, Kibar M, Kinaci C. Thyroid hemiagenesis: A report of three cases and review of the literature. Ann Nucl Med 2005;19:147-50.   Back to cited text no. 6    
7.Huang SM, Chen HD, Wen TY, Kun MS. Right thyroid hemiagenesis associated with papillary thyroid cancer and an ectopic prelaryngeal thyroid: A case report. J Formos Med Assoc 2002;101:368-71.  Back to cited text no. 7    
8.Mikosch P, Gallowitsch HJ, Kresnik E, Molnar M, Gomez I, Lind P. Thyroid hemiagenesis in an endemic goiter area diagnosed by ultrasonography: Report of sixteen patients. Thyroid 1999;9:1075-84.  Back to cited text no. 8    
9.Khatri VP, Espinosa MH, Harada WA. Papillary adenocarcinoma in thyroid hemiagenesis. Head Neck 1992;14:312-5.  Back to cited text no. 9    
10.Letonturier P, Hazard J, Tourneur R, Perlemuter L, Angel R. Thyroid hemiagenesis (single thyroid lobe): 8 cases. Nouv Presse Med 1979;8:1227-9.  Back to cited text no. 10    
11.Shabana W, Delange F, Freson M, Osteaux M, De Schepper J. Prevalence of thyroid hemiagenesis: Ultrasound screening in normal children. Eur J Pediatr 2000;159:456-8.  Back to cited text no. 11    
12.Castanet M, Leenhardt L, Lιger J, Simon-Carrι A, Lyonnet S, Pelet A, et al . Thyroid hemiagenesis is a rare variant of thyroid dysgenesis with a familial component but without Pax8 mutations in a cohort of 22 cases. Pediatr Res 2005;7:908-13.   Back to cited text no. 12    
13.Gaudino R, Garel C, Czernichow P, Lιger J. Proportion of various types of thyroid disorders among newborns with congenital hypothyroidism and normally located gland: A regional cohort study. Clin Endocrinol (Oxf) 2005;62:444-8.  Back to cited text no. 13    
14.Tonacchera M, Banco ME, Montanelli L, Di Cosmo C, Agretti P, De Marco G, et al . Genetic analysis of the PAX8 gene in children with congenital hypothyroidism and dysgenetic or eutopic thyroid glands: Identification of a novel sequence variant. Clin Endocrinol (Oxf) 2007;67:34-40.   Back to cited text no. 14    
15.Rajmil HO, Rodriguez-Espinosa J, Soldevila J, Ordonez-Llanos J. Thyroid hemiagenesis in two sisters. J Endocrinol Invest 1984;7:393-4.  Back to cited text no. 15    
16.Lee IT, Sheu WH, Lin SY. Familial form of thyroid dysgenesis: Report of thyroid hemiagenesis with accompanying Graves' disease in a woman whose daughter has thyroid agenesis. Horm Res 2003;59:47-9.   Back to cited text no. 16    
17.Baldini M, Orsatti A, Cantalamessa L. A singular case of Graves' disease in congenital thyroid hemiagenesis. Horm Res 2005;63:107-10.  Back to cited text no. 17    
18.Sasaki H, Futata T, Ninomiya H, Higashi Y, Okumura M. CT and MR imagings of single thyroid lobe (thyroid hemiagenesis) with Graves' disease. Postgrad Med J 1991;67:701.  Back to cited text no. 18    
19.Vaswani K, Vitellas KM, Bennett WF. Thyroid hemiagenesis with adenoma. AJR Am J Roentgenol 2000;175:895-8.  Back to cited text no. 19    
20.Sharma R, Mondal A, Popli M, Sahoo M, Malhotra N, Soni S. Hemiagenesis of the thyroid associated with chronic lymphocytic thyroiditis. Clin Nucl Med 2001;26:506-8.  Back to cited text no. 20    
21.Graber AL. Multinodular goiter in thyroid hemiagenesis. J Tenn Med Assoc 1983;76:174.  Back to cited text no. 21    
22.Pizzini AM, Papi G, Corrado S, Carani C, Roti E. Thyroid hemiagenesis and incidentally discovered papillary thyroid cancer: Case report and review of the literature. J Endocrinol Invest 2005;28:66-71.  Back to cited text no. 22    
23.Hydovitz JD, Winzelberg GA. Use of ultrasonography to eliminate hemiagenesis of a thyroid lobe in the differential diagnosis of a toxic adenoma (Plummer's disease) Clin Nucl Med 1988;3:144-5.  Back to cited text no. 23    
24.Leckie RG, Delaplain CB, Watabe JT, Darrow M. Thyroid hemiagenesis by I-123 thyroid scan with high-resolution real-time ultrasound confirmation Clin Nucl Med 1993;18:161-3.  Back to cited text no. 24    
25.De Remigis P, D'Angelo M, Bonaduce S, Di Giandomenico V, Sensi S. Comparison of ultrasonic scanning and scintiscanning in the evaluation of thyroid hemiagenesis. J Clin Ultrasound 1985;13:561-3.  Back to cited text no. 25    
26.Kreisner E, Camargo-Neto E, Maia CR, Gross JL. Accuracy of ultrasonography to establish the diagnosis and etiology of permanent primary congenital hypothyroidism. Clin Endocrinol (Oxf) 2003;59:361-5.  Back to cited text no. 26    

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Correspondence Address:
M Baxi
Department of Surgery, Manipal Teaching Hospital, PO Box: 341, Phulbari, Pokhara
Nepal
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.40958

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    Figures

  [Figure 1], [Figure 2], [Figure 3]

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