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. 2022 May 27;2(2):mtsi.v2i2.2022.247.
doi: 10.48327/mtsi.v2i2.2022.247. eCollection 2022 Jun 30.

[Clinical aspects of Neurofibromatosis type 1 seen in the Department of Dermatology at University Hospital Antananarivo, Madagascar]

[Article in French]
Fandresena Arilala Sendrasoa  1 , Aurélie Rasoarisata  1 , Lala Soavina Ramarozatovo  1 , Fahafahantsoa Rapelanoro Rabenja  1
Affiliations

[Clinical aspects of Neurofibromatosis type 1 seen in the Department of Dermatology at University Hospital Antananarivo, Madagascar]

[Article in French]
Fandresena Arilala Sendrasoa et al. Med Trop Sante Int. .

Abstract
in English, French

Introduction: Neurofibromatosis 1 (NF1) is an inherited disease, in an autosomal dominant manner, with complex multi-system involvements. Prevalence varies from one country to another. However, little is known about neurofibromatosis in African countries, particularly in Madagascar.

Methodology: A descriptive retrospective study from 2014 to 2019 was conducted at the service of dermatology at University Hospital Joseph Raseta Befelatanana in Antananarivo, including all patients with neurofibromatosis according to National Institutes of Health Consensus Conference criteria for whom genealogical investigation could be made.

Results: Among 32 cases of NF1 seen during 6 years, 28 cases were included with a sex ratio M/F of 0.87. The mean age was 24 years ranging from 11 to 54 years. Seventeen patients presented sporadic forms. All patients had "café au lait" spots and cutaneous neurofibromatosis. Three cases presented plexiform neurofibromas which cause significant cosmetic and functional problems by their size and their displayed topography. Fifteen patients had Lisch nodules but no case of optic glioma was identified. Neurological symptoms such as learning difficulties, epilepsy and headache were frequent in our case series. However, access to medical imaging was very limited. Scoliosis was the most common orthopedic complication.

Conclusion: The clinical manifestations of NF1 are extremely variable. Although the possibility of systemic complications seems to be low, patients must be followed up.

Introduction: La neurofibromatose type 1 (NF1) est une maladie autosomique dominante, à expression multisystémique. Sa prévalence varie selon les pays et peu de données sur la NF1 sont connues à Madagascar.

Méthodologie: Une étude transversale rétrospective a été réalisée au service de dermatologie du Centre hospitalier universitaire Joseph Raseta Befelatanana d'Antananarivo, Madagascar, sur une période de 6 ans (de janvier 2014 à décembre 2019). Ont été inclus dans cette étude les patients vus en consultation porteurs de NF1 dont le diagnostic a été confirmé et chez lesquels une enquête généalogique a pu être faite.

Résultats: Vingt-huit cas de neurofibromatose ont été inclus avec un sexe-ratio H/F de 0,87. L’âge moyen était de 24 ans avec des extrêmes de 11 et 54 ans. La tranche d’âge 16-35 ans était la plus touchée. Dix-sept patients présentaient la forme sporadique. Tous les patients présentaient des taches café au lait et des neurofibromes cutanés, 15 patients portaient des nodules iridiens de Lisch. Les difficultés d'apprentissage, l’épilepsie et la scoliose étaient les complications les plus représentées.

Conclusion: Les manifestations cliniques de la NF1 sont extrêmement variables. Même si les complications systémiques sont rares, le suivi des patients est indispensable.

Keywords: Antananarivo; Indian Ocean; Madagascar; Neurofibromatosis type 1; Sporadic forms.

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Figures

Figure 1
Figure 1
“Café au lait” spots and axillary lentigines Taches café au lait et lentigines axillaires
Figure 2
Figure 2
Cutaneous neurofibromas and a plexiform neurofibroma of the lumbar region Neurofibromes cutanés et un neurofibrome plexiforme de localisation lombaire
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