Frontonasal dysplasia

Sedano, Heddie O.; Cohen, Michael; Jirasek, Jan; Gorlin, Robert J.

doi:10.1016/s0022-3476(70)80374-2

Cited by 219 publications

(134 citation statements)

References 24 publications

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“…While the malformations due to the failure of the progress merging or apposition of structures along the median facial plane are rare, they are associated with the hypertelorism and various forms of median clefts. The latter cases are called the median cleft face syndrome or frontal dysplasia [3,4,19]. The present cases of median CLJ and CLJP with ocular hypertelorism (cases Nos.…”

Section: Discussionmentioning

confidence: 96%

Craniofacial Skeletal Abnormalities in Anomalous Calves with Clefts of the Face.

Moritomo

Tsuda

Miyamoto

1999

The Journal of Veterinary Medical Science

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ABSTRACT. Thirteen anomalous calves with clefts of the face were morphologically examined, and craniofacial skeletons were studied in detail. According to the type and site of the cleft, four groups could be distinguished: median cleft lip and jaw (CLJ); median cleft lip, jaw, and palate (CLJP); lateral CLJ; and cleft palate (CP), including unilateral and bilateral type. Craniofacial skeletal abnormalities were observed in several bones at the roof, wall, and floor of the nasal cavity and at the boundary portion between the nasal and cranial cavities. Fissure formation at the cranial sutures, partial absence of the nasal process of the incisive bone, and opening of the bony palate were characteristic changes in median CLJ and CLJP, lateral CLJ, and CP, respectively. Furthermore, various associated changes were recognized in the median and paramedian skeletal elements of the face and other organs. The morphological changes of craniofacial skeletons with various types of clefts of the face depended on the site and degree of the cleft formation and reflected developmental errors of the facial embryonic segments. These changes would suggest disorders of the correlated development of facial processes and of other fetal organs of the face. For these conditions, etiologically hereditary cases were negative.-KEY WORDS: anomalous calf, craniofacial abnormality, facial cleft.

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Section: Discussionmentioning

confidence: 96%

Craniofacial Skeletal Abnormalities in Anomalous Calves with Clefts of the Face.

Moritomo

Tsuda

Miyamoto

1999

The Journal of Veterinary Medical Science

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“…Frontonasal dysplasia (FD) is a rare group of disorders, characterized by ocular hypertelorism and frontonasal process anomalies [1][2][3] in which clinical and etiological heterogeneity have been recognized since the first review 4 . Several central nervous system (CNS) anomalies are mentioned in this condition, such as frontal encephalocele, myelomeningocele, Chiari's malformation, hydrocephalus and Corpus callosum anomalies [5][6][7][8][9] .…”

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confidence: 99%

Angular analysis of corpus callosum in 18 patients with frontonasal dysplasia

Giffoni

Gonçalves

Zanardi

et al. 2004

Arq. Neuro-Psiquiatr.

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-Considering the rarity of the frontonasal dysplasia (FD) and the few reports about it in a large casuistry using magnetic resonance image (MRI), we describe the results of the angular analysis of the corpus callosum of 18 individuals with FD (7 male, 11 female), using an easily-reproductive method. Group I had 12 individuals with isolated form and Group II had 6 individuals with FD syndromic with unknown etiology. The results are presented in set. Comparing with the control group, patients with FD presented alpha angle increase and beta and gamma angles reduction (p<0.05).Alpha and gamma angles express the relationship between the anterior portion of corpus callosum and the floor of 4 th ventricle. Considering the embryonary development, these findings would occur secondarily to failure during the development of nasal capsula. Thus, angular anomaly in corpus callosum would be a usual finding, and not fortuitous in patients with FD.KEY WORDS: corpus callosum, frontonasal dysplasia, magnetic resonance image, midline facial cleft. Análise angular do corpo caloso em 18 pacientes com displasia frontonasalRESUMO -Considerando a raridade da displasia frontonasal (DF) e os poucos estudos sobre esta condição clínica usando ressonân-cia magnética (RM), descrevemos os resultados da análise angular do corpo caloso em 18 indivíduos com DF (7 homens, 11 mulheres), usando um método de fácil reprodução. O Grupo I foi formado por 12 indivíduos com DF isolada e o Grupo II, por 6 portadores de DF sindrômica de etiologia desconhecida. Não houve diferença entre os grupos, e os dados são apresentados em conjunto. Comparando com o grupo controle, houve aumento significativo do ângulo alfa e redução dos ângulos beta e gama (p<0,05) Os ângulos alfa e gama expressam a relação entre a porção anterior do corpo caloso e do piso do 4º ventrículo. Esses achados radiológicos poderiam ocorrer secundariamente à falência do desenvolvimento da cápsula nasal. Assim, as anomalias angulares no corpo caloso poderiam ser um achado usual, e não fortuito, na DF. PALAVRAS-CHAVE: corpo caloso, displasia frontonasal, ressonancia magnética, fenda facial mediana.Frontonasal dysplasia (FD) is a rare group of disorders, characterized by ocular hypertelorism and frontonasal process anomalies [1][2][3] in which clinical and etiological heterogeneity have been recognized since the first review 4 . Several central nervous system (CNS) anomalies are mentioned in this condition, such as frontal encephalocele, myelomeningocele, Chiari's malformation, hydrocephalus and Corpus callosum anomalies [5][6][7][8][9] . A morphometric method based on measurement of five angle in order to perform a morphological analysis of the corpus callosum in craniofacial malformative syndromes was described 10 . This method was used in 34 patients with different condition, including one with FD.In this article, we describe the angular analysis of 18 individuals with FD. METHODWe evaluated 18 individuals (7 males and 11 females) affected by FD. Minimum inclusion criteria were ocul...

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“…Multiple terms have been used to describe the constellations of abnormalities observed with true median clefts, including median cleft face syndrome [4], frontonasal dysplasia [5], and Tessier #0 clefts [6]. True median clefts can be distinguished by a lack of forebrain abnormalities.…”

Section: Introductionmentioning

confidence: 99%

Repair of the Median Microform Cleft Lip Using Z-Plasty

Buonocore¹,

Walker²,

Steinbacher³

2012

MPS

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A median cleft lip is a rare central midline deficiency of the upper lip. Multiple surgical techniques are described in the literature to address this defect, though there is little consensus on the preferred surgical technique. We describe an intra-oral approach for repair of the median upper lip cleft using mucosal Z-plasty. This technique provides excellent access to the attenuated orbicularis oris muscle and the frenulum fibrosed to the labial margin. The tethered lip can be mobilized and the notch converted with appropriate mucosal length, lip height, and vermillion fullness. The contour of the free labial border immediately improves, all while avoiding a cutaneous scar. The midline cleft lip notch can be effectively treated by adhering to 3 major principles: 1) excision of the tight, constrictive labial band; 2) achieving midline orbicularis oris muscle approximation; and 3) establishing mucosal lengthening using a Z-plasty.

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Frontonasal dysplasia

Cited by 219 publications

References 24 publications

Craniofacial Skeletal Abnormalities in Anomalous Calves with Clefts of the Face.

Craniofacial Skeletal Abnormalities in Anomalous Calves with Clefts of the Face.

Angular analysis of corpus callosum in 18 patients with frontonasal dysplasia

Repair of the Median Microform Cleft Lip Using Z-Plasty

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