The internal myxoedema syndrome of Eskamilla, Lisser and Shepherdson (a case report).
N Rais, L Gandhi, J Gotur, G Rajani, SJ Shah, BT Kudva
Endocrine and Metabolic Section, c.m.c., III, Bombay.
Endocrine and Metabolic Section, c.m.c., III, Bombay.
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Rais N, Gandhi L, Gotur J, Rajani G, Shah S J, Kudva B T. The internal myxoedema syndrome of Eskamilla, Lisser and Shepherdson (a case report). J Postgrad Med 1992;38:32-3
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Rais N, Gandhi L, Gotur J, Rajani G, Shah S J, Kudva B T. The internal myxoedema syndrome of Eskamilla, Lisser and Shepherdson (a case report). J Postgrad Med [serial online] 1992 [cited 2022 Dec 7 ];38:32-3
Available from: https://www.jpgmonline.com/text.asp?1992/38/1/32/734
Internal myxoedema is said to exist when the internal or systemic manifestations overshadow the well-described external features of hypothyrodism. We report below a 54 year old man with primary hypothyroidism who had diminished hearing, refractory anaemia, pericardial effusion, ascites, hypo-cholesterolaemia, cholelithaisis and difficulty in achieving infiltration anaesthesia during cataract surgery.
On 16th June 1981, a 54 year old, married man, a teetotaler presented for the following reason: he had undergone cataract surgery in the right eye on 14th June, 1981 and had needed four times the usual quantity of lignocaine to produce retrobulbar and facial block.
FM had been ailing for about 5 years. He had been suffering from exertional dyspnoea, weakness, lethargy, drowsiness and diminished hearing. He had poor appetite, and had lost about 6 kg of weight in one year. He was sexually impotent.
In 1977, he had been investigated elsewhere for severe anaemia (Hb 3.0. gm%), and had been found to have hepatomegaly, ascites and hemorrhagic pericardial effusion. He had been treated for tuberculosis pericarditis with streptomycin for 3 months, isoniazid, para-aminosolicyclic acid, B-complex for 18 months, along with blood transfusions and iron supplements, despite which his health did not improve.
On examination, we noted the following findings - the patient was conscious and alert, 165 cm tall and weighed 65 kg. Pulse rate was 80/min. BP was 140/70 mm Hg in supine position and 110160 mm Hg on standing. Jugular venous pressure was elevated but there was no edema of the feet. He was pale, had dry skin and his auxiliary and public hair was sparse. The testes were of normal adult size. He had koilonychia, slow speech and a hoarse voice. The cardiac dullness was grossly enlarged and the heart sounds were feeble. There was no murmur in the heart nor any pericardial rub. The liver was enlarged 8 cm. below the costal margin and ascites was present. His ankle jerks were "hung-up". A clinical diagnosis of hypothyroidism with pericardial effusion, ascites and refractory iron deficiency anaemia was made.
Investigations was as follows - haemoglobin was 3.3 gm%, 7400/cmm with 68% polymorphs, 23% lymphocytes and 7% monocytes Platelets were normal. Red blood cells showed marked hypochromia, microcytosis, anisocytosis and poikillocytosis. Packed cell volume was 17% (Normal: 40 - 54%) : MCV, 100 mm (M76 - 96mm ), MCH, 19.4 pgm (N: 27-32 pgm): MCHQ 19.6% (M30 - 35%). Reticulocyte count was 5.5% (Normal 0-2%)
A myeloid: erythroid ratio of 4 : 1 with normoblastic hyperplasia, a preponderance of early and late normoblasts alongwith occasional megaloblasts was observed on bone marrow examination. Serum cholesterol levels on three successive occasions were 115%, 100 mg% and 156 mg% respectively. Serum triglyceride was 150 mg%. Postglucose blood sugar was 140 mg%, BUN 20 mg%, and serum creatinine 1.5 mg%. Serum albumin was 3.54 gm% and globulin 2.7 gm%. Plasma CPK was 56 sigma units (N: 0 -12 i.u./ml) and plasma LDH 78 i.u/1. (M70 - 240 i.u./1). Serum T3 was 10 ng% (N: 70 - 200 ng%), T4 0.4 ug%, (N: 4.8 - 11.5 ug%, serum TSH greater than 20 i.u./ml. (N : 0.5 - 4.0 i.u./ml.), the thyrotest (thyroid antibody) was positive and the Thymune-T (thyrogolobulin antibodies) was positive in a titre of 1 : 1280. The ascitic fluid was yellow in colour and showed a fibrin clot, the protein content was 1.25 gm%. The predominant cells in the fluid were lymphocytes. A pericardial tap on two occasion yielded hemorrhagic fluid with a fibrin clot. The protein content was 1.2 gm%. A few lymphocytes were seen. Intestinal absorption studies were normal. E.C.G. showed low voltage of QRS complexes in the limb leads and flattening of T waves in all the loads. Fluoroscopy of the chest revealed a large heart with diminished cardiac pulsations. X-ray of the chest showed an enlarged cardiac silhouette in the erect position, which further increased in the recumbent position. An echocardiogram confirmed the presence of pericardial effusion. Barium meal examination of stomach, duodenum and small bowel was normal. X-ray of the skull revealed no abnormality in the sella turcica. Plain X-Ray of the abdomen revealed multiple radioopague densities in the gall bladder region. Oral cholecystogram confirmed cholelithiasis and demonstrated a poorly functioning gall bladder. The final diagnosis was primary hypothyroidism with pericardial effusion, ascites, hypocholesterolaemia with cholelithiasis, and refractory anemia. The patient was prescribed 1-thyroxine sodium 0.1 mg daily, increasing gradually to 0.3 mg daily. He lost 9 kg. of weight over 5 months and was relieved of all his symptoms. The haemoglobin rose to 11.0 gm% suggesting the anaemia to be a thyroxine responsive anaemia. X-Ray of the chest on 30th Nov. 1981 showed normal heart size. The koilonychia, deafness and skin changes disappeared. The voice became normal and there was no clinically demonstrable ascites. When seen last on 12th Dec. 1982, the patient was found to be asymptomatic and was advised to take 1-thyroxine sodium regularly.
The clinical and morphological features of anaemia suggested that it was predominantly iron deficiency anaemia. The latter had failed to respond to blood transfusions as well as haematinics. This refractoriness to the treatment can be explained on the basis that the anaemia was essentially a thyroxine responsive anaemia, for on replacing 1-thyroxine sodium the haemoglobin dramatically rose from 3.3 gm to 10.0 gm in about four months.
The pericardial effusion was frankly hemorrhagic in 1977 as well as in 1981. This feature of the effusion must have prompted the previous physician to institute ntituberculous therapy, which however was of no avail. We should like to emphasise that hemorrhagic pericardial effusions are virtually unknown in hypothyroidism. Observation over the years suggest that this effusion did not behave like a tuberculous effusion, many of which, are known to progress to pericardia) constriction.
The hypocholesterolaemia of primary hypothyroidism can be due to diminished food intake, malabsorption and possible associated cholelithiasis. The last factor has been claimed to promote longevity in such patients by inducing hypocholesterolaemia and delaying the atheroscierotic vascular disease, "a process of selection".
An interesting feature in this case was the failure to achieve infiltration anaesthesia during cataract surgery with the usual dose of lignocaine. The effect might be attributed to the presence of myxedematous tissue in the orbit and the face, which prevented the local anaesthetic from achieving a retrobulbar and facial block. We have not found a reference to this phenomenon in the literature,.
We thank the Dean, Seth GS Medical College and King Edward Memorial Hospital for allowing us to publish the hospital data.
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