goundou
TRANSCRIPT
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A historical form of yawsBy Abhinay Sharma Bhugoo
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y Y aws (also known as framboesia tropica and pian) is aform of non-venereal treponematoses.
y Treponema pallidum subsp pertenue ismorphologically and serologically identical to T pallidum ssp pallidum (syphilis)
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Yaws has three stages.
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Primary stage (early) yaws
y usually begins in childhood as a spontaneously healinggranulomatous or macular lesion (mother yaws) on a
leg.
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Secondary stage (recent) yawsy is characterised by various diff use skin lesions, and
some patients develop osteoperiostitis.
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Third stage (late) yawsy features severe skin ulcers and sometimes bone
destruction causing
y severe disabling deformities and disfiguration. Latelesions
y include mutilating facial ulcer (gangosa) andgummatous skin lesions
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y Goundou is a manifestation of recent yaws, withproliferative exostoses.
y The first case was described in 1882y This disease was rare even when yaws was
hyperendemic in the early 1900s, and has nowdisappeared.
y Because of its highly stigmatising effect on thepatients appearance, goundou posed a nosologicaland clinical problem for doctors at the beginning of the 20th century
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Clinical presentationy In most cases, goundou presented as paranasal
swelling,
y characterised by two large hard tumours that did notadhere to the superficial skin layer.
y Tumours were round or oval, with an oblique long axisrunning from the base outward (figures 1 and 2)
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Figure 1: Typical bilateral goundou in a young boy Ivory Coast,
1916 (reproduced by permission of Institut de MédecineTropicaledu Service de Santé des Armées, Le Pharo, Marseille, France)
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Figure 2: Unilateral goundou in a young woman Ivory Coast, 1917
(reproduced by permission of Institut de MédecineTropicale du
Service de Santé des Armées, Le Pharo, Marseille, France).
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Clinical picture (ctd)y Pus formation and associated adenopathies were never
seen.
yThe tumours could be painf ul to touch and coulddevelop over several years, beginning with theappearance of diff use thickening or deposits on theascending branches of the maxilla.
y
Large lesions could cause blindness by reducing the visual field, sometimes in association with severeinfection of the eyeball, and with nasal obstruction.
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y Involvement of other bones of the face was generally associated with goundou and, in rare cases, was
exclu
sive.y In these forms of the disease, hypertrophy pushed the
palate into the buccal cavity and enlargement of thealveolar ridge caused wide spacing between teeth.
y
Sometimes tumour growth was so extensive that the whole rhinomaxillary region was pushed forward,resulting in impairment of both breathing andswallowing (figure 3).
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Figure 3: Diffuse maxillofacial goundou with protrusion of all the
maxillae and palate Ivory Coast, 1917 (reproduced by permission
of Institut de MédecineTropicale du
Service de
Santé des Armées,Le Pharo, Marseille, France).
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Epidemiologyy Goundou was reported in populations of black people
not only in regions of sub-Saharan Africasuch as theG
old Coast,I vory Coast, Angola, Zanzibar, and Central Africa but also in New World countries, such as
Jamaica, Brazil, Trinidad, and the southern USA. A fewcases were even reported in Asia.
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y Anàkhré and Henpuye (dog-nose).
y More limited use of the names Toupakié in the Baoule
language and Noukrou in the Bambara language wasalso reported.
y The term goundou comes from the dialect used by the Agni people in I vory Coast,
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y The geographic zones of goundou and yaws coincided.
y Goundou consistently occurred as a concurrent (44%),
short-term (49%), or long-term (7%) complication of yaws.
y In most cases, lesions were associated with diff useosteoperiostitis, mainly on the tibia, which resulted ina swordblade-like appearance. The bones of the
forearms, fingers, hands, and feet were also affected.y Postcranial lesions, which were often painf ul, could be
associated with skin lesions at the recent and latestages.
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Figure 4: Bilateral goundou on a skeleton of an adolescent
Left, the upper jaw and mandible are deformed by an osteoperiostitis but the
rest of the skull is intact. Right, the round excrescences caused a majorreduction of the visual field and nasal aperture.
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y Surgical resection was the mainstay of treatment forgoundou.
y In patients with limited forms of the disease with two
paranasal excrescences the operation was simple, becausethere was an easily identifiable subperiosteal cleavageplane.
y Such operations were done by H Strachan in Jamaica in1894,6 A J Chalmers in Africa in 1900,7 and P Mendes inBrazil in 1901.13 In the I vory Coast in 1912, Botreau-Rousseloperated successf ully on a patient with goundou, with theassistance of a Canadian surgeon named J N Roy who was visiting Abidjan from Montreal.
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y Surgery was not the only weapon against suspected yaws.
y At the beginning of the 20th century, early forms of the
disease cou
ld be treated with treponemicidal dru
gscontaining arsenic derivatives, and later antibiotics wereused.
y However, even without treponemicidal treatment,goundou did not recur after surgical excision. In patients
who presented with inoperable late forms, treatment withdrugs caused the disease to stabilise, mucosalinflammation to disappear, and facial deformity to regress,but new bone growth persisted
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Eradicationy Mass treatment campaigns in endemic areas have now
eradicated goundou.
y
Only three minor, nosologically debatable cases havebeen reported in the past 30 years
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Several questions remain unanswered.y Why does yaws seem to be the only treponematoses
associated with inflammatory hypertrophy?
y
What are the underlying physiopathologicalmechanisms?
y Why did these tumours develop on the internalbranches of one or both maxillary bones in some
patients, and over the whole face in others?y Why was the incidence higher in western Africa than
in other tropical areas?
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Source :THE LANCET Vol 360 October 12, 2002
www.thelancet.com