A Case Report on Lateral Proboscis: A Rare Congenital Anomaly

Lateral proboscis is a rare congenital condition characterized by a cylindrical protuberance on the nasofrontal region accompanied by abnormal nasal development on the affected side. We aimed to describe the management of the lateral proboscis in staged repair. A 7-year-old girl came with a tube-like projection on the left medial canthal region and nasal agenesis on the ipsilateral side. She was diagnosed with lateral proboscis, left microphthalmia, lower eye lid coloboma, and asymmetry in the orbital region. The patient has undergone 3 major surgeries at our institution. The first surgery involved the deconstruction of the tube to form the left nasal body and nostril. The second operation involved trimming of the new nose form and the excision of the bony protrusion directly beneath the base of the pedicle through bifrontal craniotomy. The remaining bone defect was closed using a pericranial flap. The orbital floor was reconstructed using titanium mesh. The third operation involved nasal reconstruction using a costal cartilage graft to create a dorsal nasal and alar framework. The patient healed with no complications, had become less reserved and her grades improved significantly after the operation. Further appointments are being scheduled to evaluate growth distortion and the resulting facial asymmetry. Surgical correction will be planned thereafter to further reconstruct the facial features. Evaluation of patient is necessary to explore possible clinical outcomes and corresponding treatment options. Multidisciplinary management is highly recommended, involving plastic surgeons, neurosurgeons, ophthalmologists, pediatricians, and pediatric psychiatrists in order to improve patient's quality of life.

Methods of Analysis of the Nasal Profile: A Systematic Review with Meta-analysis

The nose is the most prominent structure of the face, influencing facial appearance and profile. Orthodontists have an awareness of facial structures, including nasal morphology, when diagnosing and treatment planning. Maxillofacial surgeons influence facial profile by bimaxillary surgery, improving facial aesthetics and harmony. The aim of this review was to summarize the available methods of analysing nasal morphology and profile, and to assess their complexity. A literature search was conducted in PubMed, Scopus, Web of Science, and Embase using the following search terms: “nasal profile analysis”, “nasolabial angle”, and “nasal profile cephalometric” in order to select studies providing knowledge on correlations between occlusion and nasal development, differences between skeletal classes, ethnic variability, and differences between the sexes. Studies concerning genetic disorders were excluded. Finally, 17 full-text papers were analysed, which pertained to nasolabial angle, or facial profile including the nose. Data concerning methods, ethnic group, reference landmarks used, and measurements made were extracted and placed in tables. Numerous methods of nasal profile analysis can be found in the literature. These methods describe various numbers of parameters, which have influence on facial aesthetic. Nasal parameters are correlated to skeletal class and nasolabial angle, positions of upper incisors, and maxillary inclination.

Pyriform aperture enlargement in all aspects

Background:The pyriform aperture comprises the central area of facial bone structure. It is formed by the free corners of the nasal bone and the frontal processes of the maxillae, which articulate with each other at the nasomaxillary suture lines. Congenital nasal pyriform aperture stenosis might be linked to various craniofacial problems. This review presents all aspects of pyriform aperture stenosis and enlargement.Methods:A literature search was conducted. Pyriform aperture definition, nasal development, congenital nasal pyriform aperture stenosis and pyriform aperture enlargement were reviewed.Results:One of the most common abnormalities is holoprosencephaly, which is a midline developmental deficiency that may also be present in combination with facial clefting. The aetiology of nasal pyriform aperture stenosis remains unclear. When diagnosed, the choice of treatment is between non-surgical and operative methods, depending on the seriousness of the problem. Provided the sufferer can maintain a secure air passage with the help of specialised medical procedures and respiratory tract adjuvants, operative therapy may be delayed.Conclusion:The operative outcomes are extremely good, and the prognosis relies mainly on coexisting neural and endocrine problems. This paper evaluates the nasal pyriform aperture in detail.

Proboscis Lateralis: A Rare Case

ABSTRACT Proboscis lateralis (PL) is a rare congenital anomaly with a characteristic appearance. We present such a case in 5-year-old child which was managed by excision. Complete surgical excision at the base of the proboscis is desirable as a primary procedure if there is adequate ipsilateral nasal development or as a delayed excision if the proboscis is to be used in nasal reconstruction. Definitive cosmetic reconstruction with bone or cartilage should be planned at a later age keeping in mind the possibility of further growth and development of the nasal complex, however no such reconstruction was done in the present case as there was no gross asymmetry. How to cite this article Kakkar V, Sharma C, Malik P, Ghanghas B, Bishnoi S, Gulati A. Proboscis Lateralis: A Rare Case. Clin Rhinol An Int J 2014;7(2):83-86.

Cardiovascular and Craniofacial Defects in Crk-Null Mice

ABSTRACT The Crk adaptor protein, which is encoded by two splice variants termed CrkI and CrkII, contains both SH2 and SH3 domains but no catalytic region. It is thought to function in signal transduction processes involved in growth regulation, cell transformation, cell migration, and cell adhesion. Although the function of Crk has been studied in considerable detail in cell culture, its biological role in vivo is still unclear, and no Crk-knockout mouse model has been available. Therefore, we generated a complete null allele of Crk in mice by using the Cre-loxP recombination approach. The majority of Crk-null mice die at late stages of embryonic development, and the remainder succumb shortly after birth. Embryos lacking both CrkI and CrkII exhibited edema, hemorrhage, and cardiac defects. Immunohistochemical examination suggested that defects in vascular smooth muscle caused dilation and rupturing of blood vessels. Problems in nasal development and cleft palate were also observed. These data indicate that Crk is involved in cardiac and craniofacial development and that it plays an essential role in maintaining vascular integrity during embryonic development.