(* Ex Third Year Resident, ** Second year Resident, *** Additional Professor, **** Professor and Head, Department of Obstetrics and Gynecology, Seth GS Medical College and KEM Hospital, Mumbai, India.)
Lingual thyroid (LT) gland is a rare entity. Ectopic thyroid gland located at the base of tongue may present with dysphagia, dysphonia, airway obstruction or bleeding at any time from birth to adult life. We present a case of ulcerated lingual thyroid presenting as intractable hematemesis for the first time during third trimester of pregnancy.
Hematemesis may occur in pregnancy due to several causes. It may be caused by conditions ranging from gastritis to portal hypertension. Prevalence of ectopic thyroid is reported to be between 1 per 100,000-300,000. Gu et al in their case series of 42 patients found that 83% cases occured in females.
Yoon et al in their analysis of 49 cases of ectopic thyroid, found thyroid disorder in 61.9% cases. Lingual thyroid is an extremely rare cause of hematemesis in pregnancy and a diagnosis of exclusion. It may be suspected by absence of thyroid gland at its normal anatomical position and when other etiologies have been ruled out either by imaging or endoscopy.
A 20 years old primigravida with 34 weeks' gestation came in emergency ward with several bouts of bloody vomiting for a day. On examination, she was pale and had tachycardia. She was normotensive. Uterine size corresponded to the period of gestation; with a single fetus in cephalic presentation. Her uterus was relaxed. Fetal heart sounds were absent.
There were no clinical signs of bleeding disorder or portal hypertension. Her hemoglobin of 6.7 g/dl, white blood cell counts were normal. Coagulation studies were normal. Ultrasonography showed fetal demise and normal intraabdominal organs. She was admitted in intensive care unit. Gastrointestinal endoscopy showed a small prominent blood vessel at lesser curvature of stomach and it was clipped, but hematemesis continued. Blood transfusions were given to correct anemia. Repeat endoscopy was inconclusive. Indirect laryngoscopy done by otorhinolaryngologists showed an ulcerative bleeding mass of about 2X3 cm at the base of the tongue. A working diagnosis of hemangioma of tongue was made. The patient was intubated and oropharyngeal packing was done for persistent bleeding. Tracheostomy was done as the patient needed prolonged intubation. Labour was induced after cessation of hematemesis and correction of anemia. Patient uneventfully delivered a stillborn male of 2 kg. Bleeding stopped without any other intervention. Sonography of the neck, as well as computed tomogram of head and neck showed absent thyroid gland in the pretracheal area and a hypodense mass at the base of tongue in the embryological path of descent of thyroid gland. Technetium scan confirmed the diagnosis of ulcerated lingual thyroid. Thyroid function tests showed T3 level of 108 ng/dl, T4 7.08 ng/dl, TSH 15 µIU/ml, and TPO 1500 IU/ml. which confirmed autoimmune thyroiditis and she was discharged on therapy.
Hemangioma of the tongue is rarely located at the base of the tongue. It may present with dysphagia or bleeding. Lingual thyroid is also a known entity at the tongue base. Failure of descent of the medial anlage of thyroid during embryogenesis around seventh week leads to lingual thyroid. Ectopic thyroid is the presence of a functioning thyroid tissue found outside the location of the normal thyroid gland. Clinical presentations are varied, mostly related to oropharyngeal obstruction, and include dysphagia, dyspnea, dysphonia, throat fullness and sleep apnoea, hemoptysis or hematemesis.
Infants may have mental retardation and thyroid ectopy may be detected at screening. They may present with severe stridor, resulting in a medical emergency. There may be a clinically detected mass. Others present with slowly progressing symptoms of oropharyngeal obstruction before or during puberty and during pregnancy due to the increased metabolic demand for thyroid hormone. Dossing reported recurrent pregnancy related hypertrophy of a lingual thyroid operated during pregnancy to relieve obstructive symptoms. Although bleeding from an ectopic lingual thyroid is rare, it can be life threatening, since the surface of a lingual thyroid may be covered by engorged blood vessels. Chiu et al reported a case of exsanguinating bleeding from lingual thyroid in pregnancy that required embolization. Our patient had severe anemia due to bleeding from the ectopic gland that required five transfusions. Laryngoscopy shows a midline pink to red vascular mass at the base of tongue.
Investigations required include thyroid function tests, radionuclide scan with technetium 99 or iodine-131 confirms the presence of thyroid tissue in the lesion. Aspiration cytology aids in detection of malignancy and planning further treatment. Thyroiditis occurring in ectopic thyroid tissue has been reported. Our case too was detected to have autoimmune thyroiditis. Treatment of bleeding lingual thyroid includes endoscopic cauterization of bleeding vessels, embolization of lingual arteries or transoral excision followed by levothyroxine supplementation. Before extirpating ectopic thyroid, It is necessary to confirm that a eutopic thyroid gland exists so that iatrogenic hypothyroidism is avoided. The caution exercised by our otorhinolaryngologists saved the solitary ectopic thyroid in our patient.
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