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Year : 2022  |  Volume : 28  |  Issue : 1  |  Page : 91-92

A rare case of right thyroid hemiagenesis associated with papillary carcinoma in remnant lobe

1 Department of General Surgery, Aster Malabar Institute of Medical Sciences, Kozhikode, Kerala, India

Date of Submission21-Jan-2022
Date of Decision10-May-2022
Date of Acceptance11-May-2022
Date of Web Publication14-Jul-2022

Correspondence Address:
Dr. Amal George
Department of General Surgery, Aster Malabar Institute of Medical Sciences, Govindapuram P.O, Kozhikode - 673 016, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ksj.ksj_2_22

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Thyroid hemiagenesis is a rare congenital abnormality, in which one thyroid lobe fails to develop. The associated diseases in the remaining thyroid lobe include benign multinodular goiter, adenoma, hyperthyroidism, chronic thyroiditis and rarely carcinoma. The most common pathology involved in thyroid hemiagenesis is hyperthyroidism. The presence of carcinoma in a patient with hemiagenesis is quite rare and very few cases are reported in the world literature.

Keywords: Congenital thyroid anomalies, papillary carcinoma thyroid, thyroid hemiagenesis

How to cite this article:
George A, Rajan P, Akshay Viswanath, U V, Augustine JT, George T. A rare case of right thyroid hemiagenesis associated with papillary carcinoma in remnant lobe. Kerala Surg J 2022;28:91-2

How to cite this URL:
George A, Rajan P, Akshay Viswanath, U V, Augustine JT, George T. A rare case of right thyroid hemiagenesis associated with papillary carcinoma in remnant lobe. Kerala Surg J [serial online] 2022 [cited 2023 Mar 25];28:91-2. Available from: http://www.keralasurgj.com/text.asp?2022/28/1/91/350893

  Introduction Top

Thyroid hemiagenesis is a rare developmental anomaly, in which one of the thyroid lobes fails to develop. It is commonly seen in the left lobe[1] and occurs as a result of developmental defect of a thyroid lobe or failure of its precursor to migrate to the normal location.[2] The prevalence of thyroid hemiagenesis ranges from 0.02% to 0.2%.[3] Here, we are reporting a a female patient presented with left-sided thyroid nodule, diagnosed with papillary carcinoma thyroid and right thyroid hemiagenesis.

  Case Report Top

A 30-year-old female presented to our service with left-sided infrahyoid neck swelling of 1.5 cm × 1.5 cm size, 8 months duration, which was firm nodular and moving with deglutition. There were no inflammatory signs or features suggestive of hypothyroidism or hyperthyroidism or pressure symptoms. Systemic examination revealed no significant abnormalities. Thyroid function test were within the normal limits. Ultrasonography (USG) of the neck [Figure 1] reported a TIRADS IV lesion in the left lobe of thyroid with the right lobe and isthmus agenesis without any enlarged lymph nodes. USG-guided fine-needle aspiration cytology reported as papillary carcinoma thyroid. She underwent total thyroidectomy and intraoperatively we noted a firm nodule of 1 cm × 1.5 cm in size in the left lobe of thyroid with right lobe and isthmus agenesis [Figure 2]. Intra-operative and post-operative periods were uneventful. Final histopathology reported as classical type papillary carcinoma thyroid with thyroiditis.
Figure 1: Preoperative USG image. USG: Ultrasonography

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Figure 2: Intraoperative image showing absent right lobe

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  Discussion Top

The potential thyroid gland can be identified as a midline thickening in the endoderm of the floor of the pharynx at the foramen cecum by the 4th week of foetal life, from which the thyroglossal duct develops.[4] It descends and reaches the anatomical location by 7 weeks.[5] Any failure to this descend leads to ectopic thyroid which can be present anywhere from the floor of the mouth to the neck.[5] The normal adult thyroid gland is composed of two lateral lobes and an isthmus connecting the two lobes.[4] The absence of one lobe of the thyroid was first described by Hanfield Jones in 1852.[2] The pathophysiology of thyroid hemiagnesis is not well understood. The cause of hemiagenesis has been postulated to be a failure of the thyroid anlage to become bilobular, followed by unilateral migration of cells.[6] Thyroid descent and development has been found to be controlled by several genes. Thyroid transcription factors (TTF), TTF1, TTF2 and Pax-8 are involved in thyroid developmental defects, but these have not been investigated in thyroid hemiagenesis.[7] Thyroid hemiagenesis commonly involves the left lobe, followed by isthmus and rarely the right lobe.[8] About 75% of hemiagenesis cases were accounted by females in contrast to 25% by males, giving a female-to-male ratio of 3:1.[9] For diagnostic confirmation of thyroidal hemiagenesis USG, computerised tomography or magnetic resonance imaging could also be applied. USG is a very convenient means of showing the absence of the thyroid gland by transverse section over the trachea and both sides of the thyroid. The cases of thyroid hemiagenesis who are asymptomatic should have monitoring of their thyrotropin levels, if elevated treatment with thyroid hormone and regular follow-up for any development of thyroid neoplasms. In patients undergoing thyroidectomy, pre-operative recognition of thyroid hemiagenesis is important to avoid unnecessary contralateral neck exploration, which may increase the risk of recurrent laryngeal nerve injury and parathyroid injury.

  Conclusion Top

Thyroid hemiagenesis is a rare congenital anomaly which is usually symptomless. Any associated abnormality of the contralateral thyroid lobe makes them symptomatic. Carcinoma of the remnant lobe is quite uncommon, and management of such patients includes thyroidectomy and appropriate management similar to that of differentiated thyroid carcinoma and regular follow-up.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Wang J, Gao L, Song C. Thyroid hemiagenesis associated with medullary or papillary carcinoma: Report of cases. Head Neck 2014;36:E106-11.  Back to cited text no. 1
Henle J. Handbuch der Systematischen Anatomie des Menschen. Vol. 1, Ch. 1. Germany: Sohn Friedrich Viewig und Braunschweig; 1871. p. 1809-85.  Back to cited text no. 2
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. 3
Hamilton WJ, Boyd JD, Mossman HW. Human Embryology. 3rd ed. Cambridge: Heffer & Sons; 1962. p. 227-8.  Back to cited text no. 4
Pintar JE. Normal development of the hypothalamic pituitary thyroid axis. In: Braverman LE, Utiger RD, editors. Werner & Ingbar's The Thyroid: A Fundamental and Clinical Text. Philadelphia, USA: Lippincott Williams & Wilkins; 2013; 19.  Back to cited text no. 5
Greening WP, Sarker SK, Osborne MP. Hemiagenesis of the thyroid gland. Br J Surg 1980;67:446-8.  Back to cited text no. 6
Clifton-Bligh RJ, Wentworth JM, Heinz P, Crisp MS, John R, Lazarus JH, et al. Mutation of the gene encoding human TTF-2 associated with thyroid agenesis, cleft palate and choanal atresia. Nat Genet 1998;19:399-401.  Back to cited text no. 7
De Sanctis V, Soliman AT, Di Maio S, Elsedfy H, Soliman NA, Elalaily R. Thyroid hemiagenesis from childhood to adulthood: Review of literature and personal experience. Pediatr Endocrinol Rev 2016;13:612-9.  Back to cited text no. 8
Konno N, Kanaya A. Thyroid hemiagenesis associated with the right aortic arch. J Endocrinol Invest 1988;11:685-7.  Back to cited text no. 9


  [Figure 1], [Figure 2]


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