Does the benefit of salvage amputation always outweigh disability in drug-failure mycetoma?: a tale of two cases.

It is popularly believed that eumycetoma cases should be dealt with using surgical amputation for a better chance of cure especially when chemotherapy has failed. However, amputation leads to disability on one hand and on the other it may also fail to be curative. We present two cases with contrasting treatment options and outcome. In the eumycetoma case reported here, a 40-year-old male presented with right foot swelling for 16 years, from which Scedosporium apiospermum was isolated. He responded poorly to antifungal therapy and refused below-knee amputation 12 years ago. With counseling and wound care his condition improved, and Foot and Ankle Ability Measure (FAAM) score remained almost stable at 90% for 16 years, which is much better than the average functional outcome after amputation. Another 46-year-old female underwent below-knee amputation after receiving incomplete courses of antibiotics and antifungals for mycetoma of unknown etiology. She presented to us after recurrence of mycetoma on an amputated stump and was successfully treated by proper courses of antibiotics after detecting the causal agent, Actinomadura madurae. Her post-amputation disability and depression could have been avoided if the hasty decision of amputation had not been taken. In our opinion, living with drug-non-responsive mycetoma, supported by symptomatic management, may be a better option than amputation and its associated morbidities. So before taking the path of salvage amputation, we must consider many aspects, including patient's livelihood, psychological aspects and chances of recurrence even after the procedure.

What was known?

Amputation is usually done for drug-failure mycetomas as a salvage procedure.

Amputation has psychological and socio-economical impacts on patients.

Introduction

Mycetoma is a localized, chronic, deforming pyo-granulomatous infection of skin and subcutaneous tissue commonly affecting the exposed areas following traumatic implantation of saprophytic fungi or filamentous bacteria from an exogenous source that produce grains within multiple sinus tracts.[1] The outcome of treatment depends mainly on the etiological agent and the extent of the disease.[2] Medical treatment of eumycetoma done with ketoconazole and itraconazole for 18-24 months or longer, and of actinomycetoma with several regimens,[3] is followed by a tolerable extent of discomfort. Newer broad-spectrum triazoles, for example, voriconazole,[4] have also shown high efficacy, but overall medical treatment is still unsatisfactory, with a high failure rate and complications necessitating surgical procedures from wide de-bulking excisions to amputations.[24] These amputations have many psychological[5] and socio-economic impacts on patients, communities and healthcare authorities. So the benefit of amputation should be critically judged considering the probable disability of the patient with and without amputation.

Case Reports

Case 1

A 40-year-old male patient presented with a nodular swelling in the right leg [Figure 1a] with discharge of white grains from sinuses for 25 years following a bamboo-prick injury. Sixteen years ago eumycetoma was diagnosed by microscopy and culture.

Figure 1

(a) Eumycetoma on the dorsal surface of the right foot. (b) Eumycetoma grain of (KOH preparation) (×400). (c) Culture of showing a typical whitish cottony fungal colony. (d) Fungal morphology of (lactophenol-cotton blue stain) (×400)

The causative agent was confirmed as Scedosporium apiospermum. The same grain was also shown by histopathology. He was treated with itraconazole, ketoconazole and de-bulking surgery, without definite clinical improvement, and was advised below-knee amputation as the last resort 12 years ago. He did not give consent, as he could still perform his daily activities and professional work with limited disability.

We counseled him to expel pus with white grains regularly from the impending sinuses, followed by antiseptic dressing. It gave symptomatic relief from pain and inflammation. He was treated with voriconazole but the swelling did not regress and he received itraconazole over the last 21 months, as advised by another institution. Recently the patient again came with an unchanged clinical condition. Several follow up X-rays showed very slow progression of bone damage, with chronic osteoarthritis, and altered bone density. Swelling was almost painless.

Again diagnosis was reaffirmed by demonstration of soft white grains in the discharged material. The grains were 1000 μm in size, lobulated [Figure 1b], with > 2-μm-thick prominent filaments at the periphery and minimum cement substance in the centre. Growth of whitish cottony fungal colony [Figure 1c] was noted in Sabouraud's Dextrose Agar (SDA) medium using chloramphenicol after incubation at 25°C for 7 days. Slide culture with lactophenol-cotton blue staining showed septate fungal hyphae and unicellular ovoid conidia with truncated bases formed singly or in clusters [Figure 1d], identified as S. apiospermum.[6]

At present, he is having disability due to swelling and bone involvement. His Foot and Ankle Ability Measurement (FAAM)[7] sub-scores of Activities of Daily Living (ADL) and Sports are 90% and 70%, respectively, which is quite satisfactory for personal and socio-occupational activities. However, the probable functional outcome after amputation will not be better than the present condition. According to Hamilton Depression[8] and Anxiety[9] Rating Scale, the patient had no depression or anxiety after averting amputation, which shows that post-amputation psychiatric morbidity due to anxiety and depression could at least be avoided.

Case 2

A 46-year-old female patient presented with recurrence of mycetoma on the amputated stump 3 years after below-knee amputation of a right limb [Figure 2]. Initially it started as a nodular swelling in the right foot, 6 months after traumatic implantation of the organism following a bamboo prick, which was clinically diagnosed as mycetoma, with no growth in culture. Treatment was done by short courses of several drug combinations, including co-trimoxazole, streptomycin, dapsone, doxycycline and tetracycline, with no clinical improvement for 9 years. Finally the affected leg was amputated for complete remission. But after 3 years she came to us with recurrence. White grains from sinuses were collected and 1000-1500-μm grains with irregular delicate filaments of < 1 μm diameter were demonstrated in KOH smears. The causal agent was isolated in neutral SDA and identified as Actinomadura madurae by microscopy, growth characteristics and biochemical properties.

Figure 2

Recurrence of actinomycetoma after amputation in the right leg

The patient was cured by conservative treatment with amikacin and minocycline for 6 months. This shows that amputation and its consequences could have been avoided during management of primary disease, but due to one hurried decision, this school teacher had to undergo much psychological stress following loss of a limb, leading to disability. According to Hamilton Depression Rating Scale,[8] the patient had developed moderate depression post amputation.

Discussion

Considering these two representative cases, the benefits of amputation in drug-non-responsive mycetomas appear to be highly debatable. So instead of counseling for amputation and promising rehabilitation, a patient could be counseled alternatively as done in the first case. Although mycetoma causes disfigurement, it is rarely fatal. The condition is usually painless, but becomes painful with secondary bacterial infection and bone involvement. This can be minimized by regular expulsion of discharged material by expressing all active or impending sinuses, followed by antiseptic dressing. By such simple measures, emergence of newer lesions can be delayed. If a patient is properly explained the disease course, prognosis and convinced that the disease will neither spread to other body parts nor will it infect other persons by contact, it may increase the confidence level of the patient. Overall treatment for eumycetoma is unsatisfactory, requiring long-duration treatment with antifungals with many side effects and a high relapse rate. Also it is expensive for patients and healthcare authorities, but still does not promise complete cure. Even amputation becomes unsuccessful with recurrence of mycetoma,[2] like in the second case. The decision of amputation should be taken only after confirming the type of mycetoma and assessing the disability due to the disease, as well as predicting disability after operation. Mycetoma in uncommon sites, such as scalp, is unsuitable for amputation, leaving symptomatic management and proper counseling as the only options.[10] There is high incidence of anxiety and depression among amputees, which is about 20%.[511] The socio-demographic factors associated are gender, marital status, social support, income, occupation, type and level of amputation. Socio-economic impacts have essentially remained unchanged over the last decades due lack of social support, unemployment and psychiatric disability. Considering all these, a rational approach for mycetoma management is to be made.

What is new?

Amputation for drug-failure mycetomas is not always beneficial as there is a significant chance of relapse

For select cases, amputation can be avoided and disease extension can be limited by regular expulsion of discharged material

The decision of amputation should be taken only after confirming the type of mycetoma and assessing the disability due to the disease, as well as predicting disability after operation.