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Nuklearmedizin 2019; 58(03): 265-271
DOI: 10.1055/a-0830-4425
Original Article
© Georg Thieme Verlag KG Stuttgart · New York

Thyroid hemiagenesis is combined with a variety of thyroid disorders

Hemiagenesis thyroidea findet sich in Kombination mit verschiedenen Schilddrüsenerkrankungen
Christina Schneider
1   Department of Nuclear Medicine, University Hospital of Cologne, 50937 Cologne, Germany
,
Markus Dietlein
1   Department of Nuclear Medicine, University Hospital of Cologne, 50937 Cologne, Germany
,
Michael Faust
2   Department of Clinical Endocrinology, University Hospital of Cologne, 50937 Cologne, Germany
,
Alexander Drzezga
1   Department of Nuclear Medicine, University Hospital of Cologne, 50937 Cologne, Germany
,
Matthias Schmidt
1   Department of Nuclear Medicine, University Hospital of Cologne, 50937 Cologne, Germany
› Author Affiliations
Further Information

Publication History

19 July 2018

04 January 2019

Publication Date:
11 April 2019 (online)

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Abstract

Aim Thyroid hemiagenesis (TH) is a rare congenital anomaly in which one thyroid lobe fails to develop. We describe our experience with at least 13 patients presenting with TH at our department.

Methods We retrospectively analysed patients with TH, who had been referred primarily to our clinic between 2004 and 2010. In patients with TH, thyroid function parameters and thyroid autoantibodies were examined. 99mTc-pertechnetate thyroid scintigraphy and sonography were performed in all patients and confirmed the diagnosis of TH.

Results We identified 13 patients (11 women, 2 men) with TH in our patient collective and calculated an estimated prevalence of TH of 0.08 %.

We found TH to occur more frequently in the left lobe and also more frequently in females than in males. 9 patients presented with a total absence of one thyroid lobe and 4 patients presented with severe hypoplasia of one thyroid lobe with an isthmus appearing as a “hockey stick sign” on scintigraphic imaging. Associated thyroid diseases could be observed in the remaining lobe in all patients and included hyperthyroidism, hypothyroidism, nodular goiter, toxic goiter, hypofunctioning nodules, Graves’ disease and Hashimoto-thyroiditis. The most frequent thyroid disease in our patients with TH was nodular goiter. We did not find any association of TH with malignancy.

Conclusion TH is mostly detected incidentally as the prevalence of TH is extraordinary low. The fact that all of our patients with TH were also affected by other forms of thyroid disease is reasonable since the patients were not referred to the diagnostic centre due to TH but rather due to the associated thyroid disease. Possibly there are different groups of TH: the symptomatic hypothyroid children, the lifelong euthyroid adults who are diagnosed incidentally through another thyroid disease and the patients with a molecular failure of proper thyroid development.

Zusammenfassung

Ziel Hemiagenesis thyroidea (HT) ist selten. In der Literatur gibt es hierzu überwiegend lediglich Fallberichte von einzelnen Patienten. Wir beschreiben insgesamt 13 Patienten mit HT aus unserem Patientenkollektiv.

Methodik: Wir haben retrospektiv unser Patientenkollektiv hinsichtlich HT untersucht. Bei den Patienten mit HT wurden Schilddrüsenfunktionsparameter und Schilddrüsenantikörper dokumentiert sowie Tc-99m-Pertechnetat-Szintigraphie und Sonographie durchgeführt.

Ergebnisse Über einen Zeitraum von 7 Jahren konnten wir 13 Patienten mit HT identifizieren (11 Frauen, 2 Männer). Es ergab sich eine Prävalenz von 0,08 %.

Der linke Schilddrüsenlappen war häufiger betroffen als der rechte. Zudem fand sich HT bei Frauen häufiger als bei Männern. 9 Patienten zeigten einen vollständig fehlenden Schilddrüsenlappen, während 4 Patienten eine schwere Hypoplasie eines Schilddrüsenlappens im Sinne eines „hockey stick sign“ zeigten.

Bei allen Patienten wies der verbliebene Schilddrüsenlappen eine begleitende Schilddrüsenerkrankung auf. Dabei stellten die nodösen Veränderungen die häufigste Veränderung dar.

Schlussfolgerung Die Prävalenz von HT ist sehr niedrig und HT wird meistens als Zufallsbefund erkannt. Wahrscheinlich gibt es verschiedene Gruppen von Patienten mit HT: [1] die symptomatischen hypothyreoten Kinder [2] Patienten, bei denen durch einen Gendefekt bedingt eine regelrechte Anlage von beiden Schilddrüsenlappen nicht erfolgt sowie [3] euthyreote Erwachsene, bei denen HT als Zufallsbefund im Zusammenhang mit einer Schilddrüsenerkrankung entdeckt wird. Im Unterschied zur Literatur können wir ein erhöhtes Malignitätsrisiko bei HT nicht bestätigen.

Key words

Thyroid hemiagenesis - thyroid disorder - nodular goiter - hyperthyroidism - hypothyroidism

Schlüsselwörter

Hemiagenesis thyroidea - Schilddrüsenerkrankung - Struma nodosa - Hyperthyreose - Hypothyreose
 

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