Rare case of Hoffmann's syndrome necessitating forearm fasciotomy: a case report

Dr Erling Aarsæther at Department of Urology and Endocrine Surgery, University Hospital of North Norway, Tromsø, Norway and colleagues have reported a rare case ofHoffmann's syndrome necessitating forearm fasciotomy. The case has appeared in the Medical Case Reports.

Hoffmann's syndrome is a rare form of hypothyroid myopathy. Only a few cases of fasciotomy in this setting have previously been reported.

A 41-year-old Caucasian man presented to the hospital with intense pain in his right forearm. The pain exhibited a gradual onset over 2 days but was abruptly worsened following the simple task of tightening a screw with a screwdriver. His background was from a middle-class family with no known risk of hereditary disease. He was in a stable relationship and the father of two children, both in their twenties, from a previous marriage. After completing high school he had earned a university degree in education of children with disabilities, which was also his current profession. His alcohol consumption was moderate and he did not smoke tobacco. Despite his relatively young age, his medical record contained a comprehensive list of previous diseases. At age 25 he underwent fundoplication because of gastric reflux. The procedure was repeated 3 years later due to persisting symptoms. At 29 he was diagnosed as having Hodgkin's lymphoma. He initially received combination chemotherapy of doxorubicin, bleomycin, vinblastine, and dacarbazine, but this was terminated due to development of leg weakness. To compensate for chemotherapy intolerance, he subsequently received radiotherapy toward lymph nodes in his neck with a total of 40 gray. Consequently, he developed hypothyroidism as a side effect.

He had previously been on thyroid hormone replacement therapy for 7 years. During these years he had been changing the thyroxine medication from synthetic levothyroxine to a preparation derived from porcine thyroid glands, before ending up with a combination of the two. The previous 6 months he had expressed frustration over a general lack of well-being, claiming that the current thyroid hormone replacement therapy did not improve his symptoms. During several visits to his endocrinologist, he had communicated a strong desire to discontinue thyroxine hormone replacement therapy completely, in order to see whether it would make him feel better. The idea was supported by his endocrinologist, provided that he, the patient, would be willing to control thyroid function every week, in collaboration with his general physician. In the months leading up to the decision to abandon levothyroxine therapy completely, our patient's medical record indicated that he was on a natural preparation derived from porcine thyroid glands corresponding to a daily dose of 19 μg of levothyroxine and 4.5 μg of liothyronine in addition to 50 μg of levothyroxine 4 days a week and 25 μg of levothyroxine the remaining 3 days. Approximately 3 months after he had in fact discontinued thyroxine hormone replacement therapy completely, he found himself being examined in the emergency unit because of acute severe pain in his right forearm. At the time of admission, his regular medication included esomeprazole 40 mg twice a day and 100 mg of ferrous sulfate once a day only.

During clinical examination in the emergency unit after midnight, severe pain was located on the dorsal side of his right forearm. His arm appeared swollen on examination, but the overlying skin was completely normal. A brief neurological examination revealed reduced sensibility to sensation on his right forearm compared to his left, especially on the ulnar side. Brachioradial reflexes were normal on both sides, whereas biceps and triceps reflexes were unsuccessfully elicited on either side. Reduced muscular power was described in his fingers and wrist on the right side, but our patient spontaneously disclosed that this was due to the pain being increased during the contraction of these muscles. His blood pressure was 165/102 mmHg, pulse 90 beats/minute, respiratory rate 22 per/minute, and rectal temperature 37.0 °C. He did not present any obvious symptoms or clinical signs frequently seen in hypothyroidism, such as lethargy, hair loss, cold intolerance, or myxedema. His creatinine kinase was elevated to 1659 IU/L (range 40–280). The results of a screening of blood tests including complete blood count, liver function tests, renal function tests, and C-reactive protein were all within the reference range. The resident surgeon ordered overnight elevation of our patient's arm and opioid analgesics. Despite repeated administration of orally administered analgesics (1 g acetaminophen every 6 hours, 50 mg tramadol hydrochloride once) and intravenously administered opioid analgesics (5 mg oxycodone every 2 hours for 8 hours, followed by 5 mg morphine every 2 hours for 8 hours), he continued to complain of intense pain in his right forearm. The next morning a clinical evaluation of our patient revealed no improvement in pain or edema. His creatinine kinase had increased slightly to 1722 IU/L, and the surgeon on call diagnosed our patient as having compartment syndrome and referred him for immediate surgery. A preoperative computed tomography (CT) scan was performed, which revealed edema in the extensor carpi ulnaris muscle (Fig. 1), but no signs of an underlying process such as bleeding, tumor, or abscess. Complementary blood tests revealed a thyroid-stimulating hormone of 30.5 μIU/ml (range 0.2–4.3) and free thyroxine of 7 μmol/L (range 9–22), indicating hypothyroidism. A summary of the blood samples and their timing is provided in Table 1. After induction of general anesthesia by a combination of remifentanil (1 μg/kg per minute), propofol (180 mg), succinylcholine (80 mg) and fentanyl (200 μg), a straightforward fasciotomy was performed. The diagnosis of compartment syndrome was subsequently confirmed by the bulging of the affected muscle following opening of the fascia.

The surgery successfully removed the intense pain of our patient's forearm. However, a new problem surfaced in the postoperative ward, where he started complaining of similar intense pain in both calves, only a few hours after recovering from the forearm fasciotomy. Upon examination of his legs 3 hours after the forearm fasciotomy had been completed, his calf muscles appeared swollen, but soft and there was no clinical sign of compartment syndrome or myxedema. An ultrasound examination of his lower extremities was performed, but showed open deep veins bilaterally, excluding venous thrombosis as a source of the pain. The pain subsequently increased over the next few hours, but responded to elevation and opioid analgesics (5 mg oxycodone intravenously administered every 2 hours for 6 hours). The next morning, 18 hours after the forearm fasciotomy, the pain in his calves was still present but less severe and thyroid hormone replacement therapy was initiated with a levothyroxine dose of 50 μg a day. Over the next few days the pain in his calves gradually subsided, before disappearing completely on day 3 after the surgery. The edema of his forearm muscle also diminished over the next few days and the skin was closed on day 6 after the fasciotomy.

Our patient was followed regularly by an endocrinologist after he was discharged from hospital. His thyroid function stabilized on a substitution regime of 10 μg of liothyronine a day, in addition to 150 μg of levothyroxine 4 days a week and levothyroxine 125 μg the remaining 3 days of the week. Eight months after discharge he was seen by a neurologist. A full neurological examination only revealed normal findings, specifically demonstrating symmetrical and normal power, and normal sensation and function of his hand and wrist.

For more details click on the link: https://doi.org/10.1186/s13256-020-2351-x