Langerhans Cell Histiocytosis Presenting with Hypopigmented Macules.

Langerhans cell histiocytosis (LCH) is a complex disease characterized by proliferation of the Langerhans cells. The clinical course is variable and ranges from a solitary lytic bone lesion or skin lesion with complete remission to a multisystem disorder with possible lethal outcome. The diagnosis is suspected by clinical signs and symptoms and radiological features commonly in craniofacial bones and skin lesions. Diagnosis is confirmed by biopsy and immunohistochemical studies. We present case of a 8 year old child presenting with proptosis, diabetes insipidus and hypopigmented macules on chest and back showing bilateral distribution which is a rare presentation. Skin biopsy revealed the diagnosis of Langerhans cell histiocytosis.

L'exophtalmie de l'enfant a l'Institut d'ophtalmologie tropicale de l'Afrique (IOTA)

Introduction. L'exophtalmie est un signe peu frequent. Determiner les aspects epidemiologiques; cliniques et les possibilites de prise en charge de l'exophtalmie de l'enfant au Centre hospitalier universitaire Institut d'Ophtalmologie Tropicale de l'Afrique (CHU-IOTA) etait l'objectif assigne a cette etude. Patients etmethode. Cette etude prospective et descriptive s'est deroulee de juillet 2006 a juin 2007 au CHU-IOTA; avec la collaboration de l'unite d'hematologie et d'oncologie pediatrique du CHU Gabriel Toure. Tous les patients ages de 0 a 15 ans qui presentaient une exophtalmie uni ou bilaterale; mesurable ou non etaient inclus.Un examen clinique complet etait realise et des examens complementaires appropries demandes. Toutes les informations etaient recueillies sur un questionnaire. Resultats. L'etude a retenu 46 yeux de 38 enfants : 15 OD; 15 OG et 8 bilateraux. L'age variait de 2 jours a 15 ans et sa moyenne etait de 9;6 ans. Le sex-ratio etait de 1;9 garcons pour 1 fille. La frequence de l'exophtalmie retrouvee etait de 0;3. L'exophtalmie constituait le motif de consultation dans 68;4des cas; soit 26 enfants. Notre classification des groupes etiologiques etait faite de 36;9de pathologies inflammatoires et/ou infectieuses (14 cas); de 31;6de pathologies tumorales (12 cas); de 13;1de traumatismes (5 cas); et de 18;4de causes indeterminees (7 cas). L'exophtalmie avait subi une regression millimetrique au Hertel dans 23;9des cas soit 11 yeux; pendant que 4 yeux malades avaient gagnes des lignes d'acuite visuelle; soit 8;7. Discussion. La frequence de 0;3de l'exophtalmie dans un centre de troisieme reference est suffisante pour alerter les autorites sanitaires quand on sait que cette pathologie est peu frequente. La classification est comparable a celle de Crawford evoquee par Desjardins. Les cellulites (50) et les retinoblastomes (33;3) etaient les deux etiologies les plus frequentes de l'exophtalmie. Conclusion. L'exophtalmie est un risque de perte de la fonction visuelle et meme vitale qu'il faut savoir prendre en charge a temps chez l'enfant

Proptosis in normal Thai samples and thyroid patients.

OBJECTIVE: Find normal values of proptosis in Thai population and study proptosis in Graves' disease and Hashimoto's thyroiditis. MATERIAL AND METHOD: Measurement of proptosis was made with Luedde exophthalmometer by one examiner in 277 normal Thai subjects and 591 thyroid patients. RESULTS: The mean proptosis of 168 normal Thai female patients was 11.44 mm. The mean proptosis of 109 normal Thai male patients was 11.84 mm. The value of standard deviation in both groups was 2 mm. The 99% confidence limit for exophthalmoses in Thai males was 16.5 mm and 16.1 mm in Thai females. The maximum value was 17 mm in males and 16 mm in females and the minimum value was 8 mm in males and 7 mm in females. The difference between both eyes was not greater than 2 mm. CONCLUSION: The mean proptosis of normal Thai female is 11.44 mm while the mean proptosis of normal Thai male is 11.84 mm. The distribution in proptosis in Graves' disease patients was greater than the normal population by about 2 mm while in Hashimoto's thyroiditis patients was not different from the normal population.

Protosis: etiology and demographic patterns

To determine the demographic pattern of patients presenting with proptosis and its etiology. A cross-sectional descriptive study. Khyber Institute of Ophthalmic Medical Sciences [KIOMS], Hayatabad Medical Complex, Peshawar from July 2001- December 2002. Sixty cases of proptosis presenting at KIOMS were included. Thirtysix patients were male while 24 were female. They were divided into two age groups; 0-15 years [paediatric group] and above 15 years [adult group]. An elaborate work up plan was formulated for all patients, which included detailed history, clinical examination [ocular, orbital and systemic], radiological and histopathological investigations. Neoplasms [33%] were the most common causes of proptosis in all the patients followed by orbital inflammations [23%], orbital infections [20%], structural abnormalities [12%], vascular abnormalities [7%] and trauma [5%]. Neoplasms were the most common cause of proptosis in both paediatric and adult group. Proptosis was the cardinal feature and visual deterioration was present in more than half of the patients. Timely referral, early diagnosis and appropriate management can result in reduction of visual morbidity

Study of causes of proptosis in relation to demographic features among patients of El-Minya University Hospital

To analyse causes of proptosis in patients attending El-Minya University Hospital out-patient ophthalmology clinic as regards their frequency in relation to age, sex, and lateralitiy. Fifty six patients with proptosis were examined in the ophthalmology out-patient clinic of El-Minya University hospital between July 2003 and March 2005. All patients were subjected to history taking, orbital, ocular and general examination, orbital echography and axial and coronal orbital CT scans. MR imaging of the orbits, examination of the field of vision, color vision testing, excisional and incisional biopsies, T3 and T4 level, complete blood picture, sternal puncture, chest X ray, abdominal US, therapeutic steroid test and endoscopic examination of paranasal sinuses were done in some patients guided by the results of clinical evaluation and primary studies. The patients were fifty six with 29 males and 27 female. The age ranged between two months and 70 years. 23 patients were in the pediatric age group [

Exophthalmometry in mexican adults

Introducción. A la fecha no conocemos ningún informe acerca de los valores normales de proptosis en mexicanos adultos, lo que es de importancia para distinguir lo normal de los procesos patológicos orbitarios, además de que dichos valores pudiesen variar debido entre otros, a factores étnicos. Sujetos y métodos. Se midió el grado de protrusión ocular de ambos ojos utilizando el exoftalmómetro de Hertel en 301 adultos normales obtenidos al azar (185 mujeres y 116 hombres) con edad promedio de 36 ñ 8.6 años, sin historia conocida de trauma o cirugía oculares ni de padecimiento endocrinológico o de cualquier otro proceso patológico orbitario. Resultados. Los valores promedio de protrusión ocular fueron 15.18 ñ 2.16 mm en los hombres y 14.82 ñ 1.98 en las mujeres (p NS). Encontramos una diferencia estadísticamente signifiativa entre los valores observados en sujetos mexicanos adultos, sin la patología enunciada previamente y lo informado en americanos blancos y negros, con datos suficientes para llevar a cabo el análisis estadístico. Discusión. En el presente estudio establecimos los valores normales de proptosis ocular en adultos mexicanos utilizando el exoftalmómetro de Hertel. No encontramos diferencia de los valores de la exoftalmometría entre mexicanos de ambos sexos. Nuestros hallazgos mostraron valores de proptosis ocular menores que los informados en americanos negros y blancos. Proponemos que estas diferencias pueden ser explicadas por una estructura craneal y más específicamente orbitaria diferente debido a factores antropológicos y/o raciales