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Haberland syndrome with bilateral ocular involvement
Síndrome de Haberland con afectación ocular bilateral
F. Zamorano Martína, M. García Lorentea,
Corresponding author
glorentemaria@gmail.com

Corresponding author.
, C. Rocha de Lossadab, R. Rachwani Anila, A. Santos Ortegaa, J. Escudero Gómeza
a Servicio de Oftalmología, Hospital Regional Universitario de Málaga, Málaga, Spain
b Servicio de Oftalmología, Hospital Clínic de Barcelona, Barcelona, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">The Haberland syndrome or encephalocranial lipomatosis is a very rare syndrome characterized by the classic triad of skin&#44; eye &#40;in the form of unilateral choristoma and palpebral coloboma&#41; and central nervous system involvement&#46; It was first described in 1970 by Haberland and Perou&#44;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> and since then about 60 cases have been reported&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case report</span><p id="par0010" class="elsevierStylePara elsevierViewall">A 14-week male patient&#44; first child of non-blood parents&#46; Born at full term by Cesarean section&#46; On examination&#44; a 2<span class="elsevierStyleHsp" style=""></span>cm right temporal alopecia&#44; palpebral coloboma in the inner third of the upper eyelid of both eyes &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>A&#41;&#44; a pedunculated tumor dependent on the left upper palpebral tarsal conjunctiva &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>B&#41; and soft pink lesions on the lower bulbar conjunctiva of both eyes were observed invading the cornea without compromising the visual axis &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>C&#41;&#46; These lesions and the palpebral pedicle tumor were finally catalogued as complex choristomas by pathological anatomy&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">The patient exhibited good visual behaviour&#46; The preferential gaze test produced a visual acuity of 8 cycles per degree in binocular vision and 4 cycles per degree with each eye in monocular vision&#46; In fundus examination&#44; a papilla with 0&#46;4 excavation and lower depigmentation stood out&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">In view of the suspected neurocutaneous picture&#44; a cerebral magnetic resonance was requested in which an increase of the temporal subarachnoid spaces was appreciated bilaterally with arachnoid cysts &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#44; being more significant in the left side&#44; where it exhibited an approximate thickness of 3<span class="elsevierStyleHsp" style=""></span>cm&#46; An increase in the parietal subarachnoid spaces was also observed bilaterally&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">Serological tests for HIV and TORCH were negative&#46; The rest of the examination did not provide new data of interest&#46; The diagnosis of Haberland syndrome with bilateral ocular affectation was made&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0030" class="elsevierStylePara elsevierViewall">The Haberland syndrome or encephalocranial lipomatosis is a very rare syndrome characterized by the classic triad of skin&#44; eye and central nervous system involvement&#46; Its etiology is unknown&#44; although it is postulated that it may be caused by a mutation in a gene during embryogenesis&#44; being only compatible with life in the form of mosaicism&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#8211;7</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">The most typical ocular involvement is in the form of unilateral and ipsolateral coristoma to skin lesions&#44; which can cause amblyopia by inducing corneal opacities&#44; refractive defects or both&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;5</span></a> A less frequent form of presentation is palpebral coloboma1&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">At the central nervous system&#44; arachnoid cysts appear in the middle cranial fossa&#44; dysplasia or cortical atrophy with adjacent ventricular dilation and cortical calcifications&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;4&#44;8</span></a> 60&#37; of patients present epilepsy and 40&#37; psychomotor retardation&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">An atypical case of encephalocraneocutaneous lipomatosis diagnosed at a very early age is presented&#46; The case presents palpebral coloboma and bilateral choristoma&#44; differing from most published cases in which these findings are exhibited unilaterally&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;5</span></a> Associated clinic and imaging tests are essential for classifying this pathology&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">In summary&#44; since the Haberland syndrome or encephalocranial lipomatosis is a rare disease with great clinical heterogeneity&#44; the ophthalmologist may be the first specialist consulted to assess a patient with this pathology&#46; The presence of choristomas together with alopecia or other ipsolateral skin alteration should rule out a central nervous system condition by means of imaging tests&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflict of interest</span><p id="par0055" class="elsevierStylePara elsevierViewall">None of the authors have any conflict of interest&#46;</p></span></span>"
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        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Haberland syndrome or encephalocutaneous lipomatosis is a very uncommon syndrome that is characterised by changes in the skin&#44; eye&#44; and central nervous system&#46; It was first described in 1970 by Haberland and Perou&#44; with about 60 cases having been reported since then&#46; A case is reported of a 14-week-old male diagnosed with Haberland syndrome with bilateral ocular involvement in the form of palpebral coloboma and choristomas&#46;</p></span>"
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        "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">El s&#237;ndrome de Haberland o lipomatosis encefalocraneocut&#225;nea es un s&#237;ndrome muy infrecuente caracterizado por la triada cl&#225;sica de afectaci&#243;n cut&#225;nea&#44; ocular y del sistema nervioso central&#46; Fue descrito por primera vez en 1970 por Haberland y Perou&#44;<span class="elsevierStyleSup">1</span> habi&#233;ndose reportado unos 60 casos desde entonces&#46;<span class="elsevierStyleSup">2</span> Presentamos un caso de un var&#243;n de 14 semanas diagnosticado de s&#237;ndrome de Haberland con afectaci&#243;n bilateral ocular en forma de coloboma palpebral y coristomas&#46;</p></span>"
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Original language: English
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