We report a child with right-sided heminasal aplasia in combination with anomalies of the right eye and maxilla. Unilateral aplasia of the nose is a rare congenital malformation. It is often associated with other malformations of the facial region, including abnormalities of the eye and lacrimal system, proboscis lateralis, and facial bone malformations. The eye anomalies in our patient consisted of microphthalmia with blepharophimosis and coloboma of the iris, retina and upper eyelid. Also hypoplasia of the lacrimal apparatus and right maxilla, and a rudimentary alveolar cleft on the same side were present. The embryological development of the midface can explain this association of anomalies.
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BMJ Case Rep
January 2022
Ophthalmology, Dr. RP Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, Delhi, India
Heminasal agenesis is an exceedingly rare congenital anomaly which is frequently associated with disorders of eye, lacrimal drainage system and face. At times, a proboscis may also be noted with this condition. Herein, we present a case of a 13-year-old boy who presented to us with heminasal agenesis with concomitant microphthalmos, leucomatous corneal opacity, blepharoptosis, dacryocystocoele and maxillary hypoplasia.
View Article and Find Full Text PDFHeminasal aplasia is a rare congenital nasal anomaly in which there is unilateral deficiency in both the external nasal anatomy and nasal airway. Unilateral failure in development of a nasal placode in embryogenesis is thought to be the underlying cause of this anomaly. The authors describe the reconstruction of heminasal aplasia in a teenager utilizing a templated cartilaginous framework and tissue expansion.
View Article and Find Full Text PDFJ Craniomaxillofac Surg
September 2019
Milan, Italy.
Proboscis lateralis (PL) is a rare malformation, reported for the first time in 1861 by Forster in his monograph on congenital malformations of the human body. The abnormal side of the nose is represented by a tube-like rudimentary nasal structure, attached at any point along the embryonic fusion line between the anterior maxilla and the frontonasal processes. As clefts of the lip (and alveolus) are bilateral or unilateral, an arrhinia can be bilateral (total) or unilateral.
View Article and Find Full Text PDFJ Craniofac Surg
July 2019
Department of Surgery, Division of Plastic and Reconstructive Surgery, American University of Beirut Medical Center, Lebanon.
Proboscis Lateralis is a rare congenital anomaly composed of an accessory tubular appendage with possible associated craniofacial anomalies. Computed tomography scan is essential for evaluation of the anomaly and proposing a plan of management. Treatment is complex and should be individualized.
View Article and Find Full Text PDFJ Craniofac Surg
July 2019
Plastic Surgery Department, Faculty of Medicine, Sohag University, Sohag, Egypt.
Objective: This report presents an extremely rare case of heminasal aplasia or hemi-arhinia with only about 80 cases reported so far. The exact etiology and mechanism of development of heminasal aplasia are still unknown. Furthermore, the rarity of this anomaly makes its reconstruction a surgical challenge with a diversity and controversy on the timing and technique of such reconstructive procedures.
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