A .A. Musa
Department. of Surgery, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria
Correspondence: A .A. Musa Department. of Surgery Usmanu
Danfodiyo University Teaching Hospital P.M.B. 2370, Sokoto, Nigeria E-mail:
agabamusa@yahoo.com
Code Number: hs06010
Abstract
Background:Tropical idiopathic lower limb gangrene (TILLG) is also known as Symmetrical gangrene in the African, Idiopathic gangrene in the African and Idiopathic peripheral gangrene of the tropics.The aetiopathogenesis of this clinical entity is a mystery.
Objective: To review methods of diagnosing tropical idiopathic lower limb gangrene (TILLG) and highlight its clinical variants.
Method: All Literature on idiopathic gangrene of the extremities was searched from libraries, colleagues and internet but only literature on TILLG (in Africans) from 1947 to date was scrutinised. Each case was studied to find out the basis of diagnosis.
Result:TILLG is not fully understood and not easy to recognise.Two sets of criteria are known to be helpful in establishing diagnosis. These criteria can be classified as major and minor criteria. Major criteria are those clinical data that can establish the diagnosis of TILLG. No devices are required to identify them. Minor criteria are pathological changes that are consistent with TILLG. Devices are required to identify them.Three pathomorphological types of TILLG were described in literature and are classified as types A, B and C.
Conclusions:This review is supposed to sensitise the clinician and make
diagnosis easier.This will also encourage more researches.As more information
becomes available, aetiopathogenesis of TILLG will be clearer and more clinical
variants of the disease may be reported.This additional information will
help in the prevention of gangrene, reducing the socioeconomic problems arising
from amputation.
Introduction
The first report of tropical idiopathic lower limb gangrene (TILLG) is credited
to M. Gelfand1 . The features of this first case were; gangrene of unknown
aetiology, which was bilateral and simultaneous. The gangrene was sudden
and mostly in males who were in the second and forth decade of life. The
first sign was oedema of both feet accompanied by pain. Febrile condition
was associated with TILLG. Many cases have been reported in East and Central
Africa but recently two variants of the disease have been seen in Nigeria2,3 . Two colleagues in Nigeria discussed cases of TILLG they could not report
with the author (personal communications). This suggests the incidence
of TILLG could be higher than previously thought. Diagnosis of the disease
has always been difficult. Below is a review of the methods of diagnosing
TIILG and a highlight of its clinical variants.
Diagnosis of TILLG
TILLG has always been diagnosed on the bases of the
similarity of history of the case in question and the first case reported1.
Amongst the features of the cases reviewed, the sign that was always present
was dry gangrene of any of the lower limbs of sudden onset. The index of
suspicion of the physician was very instrumental in establishing the diagnosis1,2,3,4.
Lower limb gangrene can result from necrotising fasciitis5,
but there is always history of trauma and most often the gangrene was wet.
Atherosclerosis can cause dry gangrene. The patient is usually elderly,
above fifty years of age and histology will reveal typical artherosclerotic
changes in the form of plaques obliterating vascular lumen. In the neonate,
gangrene can result from congenital syphilis.VDRL test (Venereal Diseases
Research Laboratory Test) in any of the parents will be positive.This is
the differential diagnosis of TIILG.
This method of diagnosis is based
only on clinical data and can be called the major criteria for establishing
the diagnosis of this disease. Recognition of the signs and symptoms of
TILLG is within the competence of most clinicians.
There are pathological changes that have been found to be consistent with TILLG.
In vivo there is equilibrium between coagulation and fibrinolysis. In TILLG
there is slight increase in the level of fibrinolytic activity 6,7.Thus
analysis of blood sample from a patient diagnosed as a case of TILLG, reveals
raised levels of plasma fibrinogen, factors V and VII, shortening of clotting
and thrombin times.
TILLG has a particular arteriographic pattern8 . The lumen of the
arteries of the involved lower limb is not uniformly narrowed and collateral
circulation develops whenever metatarsal or proximal digital arteries were
occluded. TILLG results in distinct microscopic vascular changes9.
Part or the whole of the circumference of the arteries were thickened as
a result of proliferation of the intima by loose tissue composed of basophilic,
mucoid, cystic material whose contents are fibroblast and smooth muscle cells.
These mucoid cysts are also present in the media and adventitia. In some
of the cases proliferation of the intima is accompanied by elastic degeneration
of the lamina. In cases that present late there are foci of calcification
in all the layers of the arterial wall. There is increase of collagen and
hyaline tissue in this layer. Intimal proliferation and mucoid swelling of
the media are either cushion like or diffuse. Frank arterial thrombosis is
rare and when present may undergo mucoid transformation.These pathohistological
changes are not limited to the arteries alone.The same type of intimal proliferation
and mucoid swelling of the media are also seen in bigger veins. These pathological
changes can be called the minor criteria for establishing the diagnosis of
TILLG. These investigations are not be possible in the rural hospitals where
these patients will first present.