Congenital Arhinia: A Rare Case Report and Review of Literature

Mondal, Uttam Kumar; Prasad, Rameshwar

doi:10.1007/s12070-016-1009-x

Cited by 11 publications

(11 citation statements)

References 8 publications

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“…At the age of one year, the child was feeding liquid and semisolid food and growing well. The case of Mondal and Prasad was most probably the reported case number fifty one [12].…”

Section: Discussionmentioning

confidence: 92%

Ruprecht Majewski-Bosma syndrome associated with atrial septal defect

Al-Mosawi¹

2019

IJCCR

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Background: Ruprecht Majewski-Bosma syndrome is an extremely rare dysmorphic syndrome results from severe hypoplasia of the nose and eyes occurring in association with palatal abnormalities.It is characterized by congenital complete nasal agenesis (Bilateral aplasia of the nose) , microphthalmia including clinical anophthalmia, hypertelorism and other eye defects, high arched palate, and other palatal defects. Materials and methods:A full term newborn female born to a consanguineous parents in their twenties with multiple congenital abnormalities was observed and studied at Children Teaching Hospital of Baghdad Medical City. The relevant literature was reviewed. Results: At birth the girl had the characteristic congenital abnormalities Ruprecht Majewski-Bosma syndrome of including complete nasal agenesis (Bilateral aplasia of the nose), bilateral microphthalmia, high arched palate, hypertelorism, and secondum atrial septal defect. Literature review confirmed the extreme rarity of the condition. Becerra-Solano et al ( 2016) reported the fifteenth case of the syndrome in the literature. The case of Mondal and Prasad was most probably the reported case number fifty one. Congenital heart defects have not been reported in association with this syndrome. Conclusion:The paper reported the fifty two patients with Ruprecht Majewski-Bosma syndrome which is the first case of the syndrome to be associated with congenital heart defect, atrial septal defect.

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“…At the age of one year, the child was feeding liquid and semisolid food and growing well. The case of Mondal and Prasad was most probably the reported case number fifty one [12].…”

Section: Discussionmentioning

confidence: 92%

Ruprecht Majewski-Bosma syndrome associated with atrial septal defect

Al-Mosawi¹

2019

IJCCR

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“…At the age of one year, the child was feeding liquid and semisolid food and growing well. The case of Mondal and Prasad was most probably the reported case number fi ftyone [12].…”

Section: Methodsmentioning

confidence: 88%

The case number 52 of Ruprecht Majewski-Bosma syndrome associated with atrial septal defect

Al-Mosawi¹

2020

Glob J Rare Dis

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“…However, once this membrane ruptures, communication is established between the nasal and buccal cavities in the form of posterior choanae. The posterior choanae are quickly filled by epithelial plugs, and recanalization of these plugs forms the secondary posterior choanae by the 24th week of life (Albernaz et al, 1996;Mondal and Prasad, 2016).…”

Section: Discussionmentioning

confidence: 99%

“…Complete congenital absence of the nose, also known as arhinia, is a rare embryologic disorder of unknown etiology with less than 50 reported cases (Mondal and Prasad, 2016). Congenital arhinia is commonly associated with other congenital abnormalities such as the eyes, ears, and palate (Méndez-Gallart et al, 2009;Mondal and Prasad, 2016). These patients can have difficulties with breathing and feeding due to the absence of the nose and nasal cavities (Méndez-Gallart et al, 2009).…”

Section: Introductionmentioning

confidence: 99%

Reconstruction of Congenital Arhinia With Stereolithographic Modeling: Case Correlate and Literature Review

Harrison

Anderson

Spiller

et al. 2021

The Cleft Palate-Craniofacial Journal

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Complete congenital arhinia is a rare defect of embryogenesis leading to the absence of the external nose and airway. We report our novel multistaged reconstructive approach and literature review. Nasal methyl methacrylate prosthesis was created from a stereolithographic model for use as a temporary prosthesis and tissue expander. Lefort 1 with cannulization was utilized for midface advancement and airway formation. External framework was reconstructed with bilateral conchal bowl cartilage and rib osteocartilagenous grafts. Patient was pleased with the aesthetics and had safe decannulation with the ability to breathe through the nose and airway.

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Congenital Arhinia: A Rare Case Report and Review of Literature

Cited by 11 publications

References 8 publications

Ruprecht Majewski-Bosma syndrome associated with atrial septal defect

Ruprecht Majewski-Bosma syndrome associated with atrial septal defect

The case number 52 of Ruprecht Majewski-Bosma syndrome associated with atrial septal defect

Reconstruction of Congenital Arhinia With Stereolithographic Modeling: Case Correlate and Literature Review

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