Simultaneous Construction of an Internal and External Nose in an Infant with Arhinia

Mühlbauer, W.; Schmidt, Andreas; Fairley, Jeffrey

doi:10.1097/00006534-199304000-00027

Cited by 45 publications

(29 citation statements)

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“…Some parents, however, have opted to have reconstructive craniofacial surgery performed at an earlier age because of the social implications of the physical malformation. 13,14 In conclusion, congenital arhinia is a unique condition that has been infrequently reported in the literature. Most prior publications involved case reports of arhinia diagnosed postnatally.…”

Section: Discussionmentioning

confidence: 92%

Second-Trimester Prenatal Diagnosis of Total Arhinia

Majewski

Donnenfeld

Kuhlman

et al. 2007

Journal of Ultrasound in Medicine

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rhinia is defined as the congenital absence of the nose. It is an extremely rare condition, which is usually associated with other craniofacial abnormalities. An extensive literature search revealed that the prenatal diagnosis of this anomaly has only been reported on 1 previous occasion at 29 weeks' gestation, and the diagnosis at birth has been reported only 29 times in the pediatric literature.1-29 To our knowledge, there has not been a previously reported case of congenital arhinia diagnosed during the second trimester. This report describes the 2-and 3-dimensional sonographic appearance of arhinia shown during second-trimester genetic sonography and reviews this exceedingly rare craniofacial defect. Early diagnosis of this defect allows the patient to consider all her options regarding pregnancy management. The detection of arhinia in the second trimester provides evidence that this rare disorder can be diagnosed through appropriate evaluation of craniofacial anatomy on a comprehensive second-trimester sonographic examination.Received August 28, 2006, Case ReportAn 18-year-old woman, gravida 1, para 0, was initially seen by her obstetrician at 8 weeks' gestation. Her medical history was notable for depression treated with escitalopram oxalate (Lexapro; Forest laboratories, St Louis, MO) at 20 mg daily. The medication was begun 3 months before conception. At this first obstetric visit, the patient was advised to taper her medication to 10 mg for 1 week, then to 5 mg for an additional week, and then to discontinue use of the medication. Subsequently, a firsttrimester sonographic examination and genetic consultation because of the medication use were performed at 9 weeks' gestation. The sonogram revealed a single, viable intrauterine pregnancy with fetal measurements appropriate for gestational age.

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Section: Discussionmentioning

confidence: 92%

Second-Trimester Prenatal Diagnosis of Total Arhinia

Majewski

Donnenfeld

Kuhlman

et al. 2007

Journal of Ultrasound in Medicine

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“…Algumas alterações anatômicas vistas em nosso paciente já haviam sido descritas anteriormente, principalmente estenose óssea anterior 2,7,11,12 , cavidade nasal hipoplásica 7 , palato em ogiva 2,7,12 e com fenda 6 , agenesia de ducto naso-lacrimal 12,13 , hipoplasia de seios paranasais 7,12 e microftalmia 6 . Outras características presentes na literatura médica, que no entanto, não se encontravam em nosso caso são: fenda óssea na placa cribiforme e plano esfenoidal 7 e presença de seios maxilares 2 .…”

Section: Discussão Discussão Discussão Discussão Discussãounclassified

“…Essa primeira abordagem em geral é realizada no primeiro mês de vida, com relatos variando entre 15 dias 7 e 2 meses 6 . A técnica cirúrgica ainda não está plenamente estabelecida e varia bastante, conforme podemos perceber nos relatos de Meyer 2 e Mühlbauer 12 . O primeiro autor realizou a reconstrução do nariz em três tempos cirúrgicos, sendo que o primeiro deles consistiu na criação de um nariz externo, através de um flap cutâneo de região frontal com base inferior, posicionado sobre a região da agenesia nasal, de maneira a recobrir um enxerto de cartilagem costal, que sustentava a estrutura como um neo-septo.…”

Section: Discussão Discussão Discussão Discussão Discussãounclassified

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Agenesia do nariz: relato de caso

Chung

Goto

Romano

et al. 2002

Rev. Bras. Otorrinolaringol.

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Agenesia do nariz (arrinia, atresia nasal congênita) é uma malformação rara, tendo sido relatados aproximadamente 20 casos na literatura de língua inglesa no último século. Acredita-se que a origem embriológica do defeito seja o subdesenvolvimento dos placódios nasais. As dificuldades respiratórias se evidenciam logo após o nascimento e requerem intubação orotraqueal imediata seguida de ventilação mecânica. Neste trabalho relatamos um caso de ausência congênita do nariz em uma criança do sexo masculino. Logo após o nascimento, apresentou desconforto respiratório, evoluindo rapidamente com cianose, sendo submetida a intubação orotraqueal e posteriormente traqueostomia. Ao exame físico apresentava ausência completa da pirâmide nasal, substituída por uma superfície plana e lisa de consistência endurecida, palato duro em forma de ogiva e microftalmia bilateral. A criança foi avaliada também através de exames de tomografia computadorizada (TC) e ressonância nuclear magnética (RNM). A TC revelou ausência do nariz, estenose óssea anterior, ausência de cavidade nasal e seios paranasais, palato arqueado em ogiva, microftalmia bilateral e mucocele bilateral de saco lacrimal. A RNM demonstrou configuração e densidade normal do tecido cerebral.

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“…Often these defects can be associated with other complex craniofacial and intra cranial anomalies which can range from agenesis of corpus-callosum to Holoprosencephaly [1], proboscis, etc., which can be incompatible with life. The primary aim of congenital nasal reconstruction is to reconstitute the skeletal framework with preservation of its physiologic function along with the correction of the craniofacial skeleton.…”

Section: Introductionmentioning

confidence: 99%

Reconstruction of Nasal Cleft Deformities Using Expanded Forehead Flaps: A Case Series

Ramanathan

Pendem

Parameswaran

et al. 2013

J. Maxillofac. Oral Surg.

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Introduction Reconstruction of the nasal clefts is a challenging task considering the nasal anatomic complexity and their possible association with craniofacial defects. The reconstruction of these defects needs extensive amounts of soft tissue that warrant the use of forehead flaps. Often presence of cranial defects and low hairline compromise the amount of tissue available for reconstruction warrenting tissue expansion. Aim To evaluate the efficacy of tissue expansion in reconstruction of congenital nasal clefts. Subjects and Methods 9 patients with congenital nasal clefts involving multiple sub units were taken up for nasal reconstruction with expanded forehead flaps. The average amount of expansion needed was 200 ml. The reconstruction was performed in 3 stages. Results Expanded forehead flaps proved to be best modality for reconstruction providing the skin cover needed for ala, columella and dorsum with minimal scarring at the donor site. Conclusion Expansion of the forehead flap is a viable option for multiple sub unit reconstruction in congenital nasal cleft deformities.

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Simultaneous Construction of an Internal and External Nose in an Infant with Arhinia

Cited by 45 publications

References 0 publications

Second-Trimester Prenatal Diagnosis of Total Arhinia

Second-Trimester Prenatal Diagnosis of Total Arhinia

Agenesia do nariz: relato de caso

Reconstruction of Nasal Cleft Deformities Using Expanded Forehead Flaps: A Case Series

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