HIV Quadruple Limb Gangrene: An Unusual Presentation and Review of Literature

doi:10.4236/ss.2013.410080

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Surgical Science, 2013, 4, 411-414

http://dx.doi.org/10.4236/ss.2013.410080 Published Online October 2013 (http://www.scirp.org/journal/ss)

HIV Quadruple Limb Gangrene: An Unusual Presentation

and Review of Literature

Edwin Omon Edomwonyi*, John Enekele Onuminya, Alfred Oghogho Ogbemudia,

Osita Chizoba Nwokike, Emeka Blessius Kesieme

Department of Orthopaedics and Traumatology, Irrua Specialist Teaching Hospital, Irrua, Nigeria

Email: *[email protected]

Received August 7, 2013; revised August 28, 2013; accepted September 3, 2013

License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

ABSTRACT

Vasculitis is one of the less common but important cons equence in patients with Hu man Immunodeficiency Virus (HIV)

infections and presentation with multiple limb gangrene is rare. We therefore report a novel case of quadruple limb

gangrene in a patient with HIV infection.

Keywords: HIV; Quadruple Limb Gangrene; Presentation; Amputation; Irrua

1. Introduction

Quadruple limb gangrene associated with HIV infection

is rare. The common aetiologies of gangrene of th e limbs

are trauma, diabetes mellitus and artherosclerosis [1].

However, systemic lupus erythematosus, progressive

sclerosis, henoch scholein syndrome, takayasu arteritis,

antineutrophil cystoplasmic antibody (ANCA) associated

vasculitis, gangrene associated with procoagulant states

due to malignancy, and anticardiolipin antibody syn-

drome are some important causes of gangrene of the ex-

tremities. Rare causes of gangrene include heparin in-

duced thrombocytopenia (HIT), hemolytic uremic syn-

drome (HUS) and Human immunodeficiency virus (HIV)

infection [2].

HIV infection is multisystemic, and cardiovascular

involvement is not an exception. It may present with ar-

terial occlusive disease, aneurismal disease, aortic dis-

section or AV fistula [2,3]. Vasculitis is one of the less

common but important consequences of HIV. Peripheral

arterial disease (PAD) is more prevalent in the HIV-

infected population than in the general population. Wide-

spread digital ischaemic changes and gangrene of the

hands and feet are uncommon in patients with HIV in-

fection [2-7]. A few cases of one or two limb gangrene

associated with HIV infection have been reported from

other centres [2-10]. The only reported case of quadruple

limb amputation reported in our environment was due to

trauma [1].

Quadruple gangrene of the extremities associated with

HIV infection is therefore reported to highlight the rare

complication and challenges. Relevant literature is also

reviewed.

2. Case Report

A 28-year-old southern lady presented wounds in both

upper and lower limb of two months duration via the

Accident and Emergency department of Irrua Specialist

Teaching Hospital, Irrua. She was in her usual state of

health until 6 months prior to presentation when she de-

veloped pepperish/tingling sensations on both upper and

lower limbs. She wasn’t a known hypertensive or dia-

betic. Subsequently, she noticed that the feet became

swollen and later ulcerated. She sought help in a native

herbal home where topical herbal preparation was ap-

plied on scarification marks. Three months later, darken-

ing of the toes was noticed which gradually spread to

both feet with loss of sensation. A month later, a similar

process repeated itself in both upper limbs following the

same chronology.

The husband was a long distant driver who abandoned

her in the course of the ailment claiming he does not un-

derstand the nature of the ailment. She never smoked

cigarette, ingest tobacco. No past history of suggestive of

coronary disease or stroke. On examination, we saw an

emanciated young lady in mild painful distress. She was

pale with bilateral inguinal lymphadenopathy. Nil axil-

lary lymph node enlargement. Vitals were within normal

range. She was hemodynamically stable. Abdomen and

*Corresponding a uthor.

E. O. EDOMWONYI ET AL.

412

Respiratory systems were essentially normal.

Extremities were darkish in colour and withering of

both hands and feet. The clinical appearances of the

quadruple gangrene of the feet and hands are shown in

Figures 1((a)-(c)). Feet were almost severed at the ankle

joints exposing the ligament, tendons and bones with

offensive discharge. Sensory loss in both feet was up to

the distal third. Dorsalis pedis and posterior tibial arterial

pulsations were absent. Popliteal arterial pulsation felt

but faint.

(a)

(b)

(c)

Figure 1. Clinical appearances of the quadruple gangrene of

feet and hands: (a) Gangrene of the feet with autodisarticu-

lation at the ankle joints from a traditional surgeon’s home;

(b) Gangrene of the left hand; (c) Gangrene of the right

hand.

There was hyperpigmentation of both hands up to the

metacarpophalangeal joints , and the metacarpophanlangeal

joints were in fixed flexion deformity with sensory loss.

Radial arterial pulsation was present in both upper limbs.

RBS was 86 mg/dl, Pcv was 27%, total Wbc, 4600

c/mm3; Platelet, 397,000 c/mm3; Na 140 mmole/l, K 2.9

mmole/l, Cr 0.8, CD4 count was 324 cl/ul. While she

was being worked up for emergency surgery it was dis-

covered she was HIV positive.

Emergency bilateral BKA were done under GA. Two

weeks later bilateral BEA was done on both upper limbs.

Lower limbs wounds were discovered to be infected and

this was managed by regular povidone iodine wound

dressing and antibiotics. The medical team also assessed

her and commenced HAART, lamivudine, nevirapine,

zidovudine as well as hematinics.

Wound cleaned out and refashioning of stumps were

done at intervals and wounds healed satisfactorily. Post

operative period was largely uneventful. Majority of

other requested investigations (Doppler USS, lipids etc.)

were not done due to financial constraints. The Manage-

ment of the hospital wrote off the bill and was finally

discharged after 114 days on admission without prosthe-

ses owing to lack of fund.

3. Discussion

Peripheral vascular disease is a common indication for

amputation of patients in the developed world and the

rever se is the cas e in Nigeri a where tr aumas an d diabe tes

lead [1].

Peripheral artery disease is more prevalent in the HIV

infected population than in the general population and

there is a six fold increased risk for Peripheral artery

disease in the HIV infected individuals as well as an ear-

lier onset of the disease compared with HIV negative

patients [9].

Almost every pattern and type of vasculitis of small,

medium and large vessels has been encountered in HIV

setting [3]. Widespread digital ischemic changes and

gangrene of the hands and feet is one uncommon presen-

tation in patients with HIV infection [8], which makes

this case stand out.

HIV positive patients with vasculopathy are younger

with an average age of 40 yrs in comparison to 55 yrs in

patients with artherosclerotic disease [3]. This is in sup-

port of our case who was 28 yrs of age. HIV associated

vascular disease is a specific disease entity which differs

from artherosclerotic disease in many aspects. Risk fac-

tors for artherosclerosis like smoking, hypertension and

diabetes mellitus are lower in incidence among patients

with HIV than peripheral vascular disease. This patient

never sm oked. She was ne i ther hyp ertensive nor dia be t ic.

CD4 T cell count 200 cells/ul was found as a signifi-

cant predictor of peripheral artery disease in HIV [9]. As

E. O. EDOMWONYI ET AL. 413

shown in this case CD4 count of patients with HIV vas-

culopathy are less than normal in 90% and the CD4/CD8

ratio is usually reversed, indicated of advanced Immu-

nosuppression [3]. Infection, occlusive disease due to

hypercoagulable states, vasculitis is some of the mecha-

nisms suggested for gangrene of the extremities in HIV.

Psychiatric symptoms complicating amputation is not

out of place as noticed in our patient, the physical reality

of mutilation, bod y image changes and diagnos is of HIV

in a patient is strong enough stress.

Risk of HIV was obvious in this patient; the husband

being a long distance driver who even neglected the pa-

tient when she became ill. She was seriously handi-

capped financially. Hence, she was unable to do most

investigation and her bills had to be written off after sev-

eral weeks of neglect following discharge from hospital.

3.1. Etiology of Gangrene in HIV

Vasculitis, occlusive disease and infections, hypercoagu-

lable states. Deficiency of protein C and free protein S,

antiThrombin III. Anti phospholip id syndrome have been

implicated as mechanism. Antiretroviral medications

could lead to endothelial damage too.

3.2. Clinical Feature

1) Male preponderance for obscure reasons; 2) Rest pain

of the limbs from ischemia; 3) Intermittent claudication

Gangrene.

3.3. Diagnosis

HIV associated gangrene may be associated with a

known pathogen or trigger or may occur in the absence

of one. To determine this, either a serological test, stain-

ing of smears, light microscopy, culture, immunohisto-

chemistry testing and viral markers may be done de-

pending on the clin ical presentation.

Work up for protein C, S, antithro mbin III, antinuclear

antibodies, antiphospholipid antibodies. Ankle index

(AB1) testing would help to identify those with Periph-

eral vascular disease.

Doppler study of the vascular system is essential.

Spotting within the arterial wall test described as string

of pearl sign [3].

Diagnostic angiography would identify the location of

occlusive or aneurysm. Care should be taken to heparinize

these patients before the procedure because of the high

risk of thrombosis.

3.4. Management

HIV vasculopathy and gangrene represent an advanced

stage of the disease process. It is advisable to commence

HAART irrespective of the CD4 count.

Patients with unsalvageable limb should have primary

amputation. When limb is salvageable, treatment options

include endovascular procedure like thrombectomy,

thrombolysis or by-pass procedures. Limb salvage rate

has been in the region of 27% [3]. Widespread ischemic

necrosis and gangrene may require treatment with corti-

costeroids (in the event of vasculitis), thrombolytic

agents (for thrombotic component), or both. Vascular

surgical principles should be adhered to and management

should be individu alized. When patien ts present late with

gangrene as in the index case amputation is the option of

surgical treatment and in this case the morbidity was high.

The challenges were multiple in ev ery respect.

4. Conclusions

Quadruple limb gangrene associated with HIV infection

is a rare and novel report from Irrua. In the light of the

foregoing, this is a wakeup call for clinicians. Clinicians

should consider HIV as an important cause of limb gan-

grene and screen for presence of HIV antibodies while

evaluating a patient with non-traumatic limb gangrene.

Early detection and timely commencement of appro-

priate treatment may prevent vascular complication and

subsequent gangrene in asymptomatic patients.

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