Proboscis Lateralis Research Articles

Proboscis Lateralis of the Nose

Abstract Proboscis lateralis (PL) of the nose is a very uncommon craniofacial anomaly characterized by a rudimentary tubular nose-like structure seen a little away from the midline of the face. It may be associated with other anomalies such as heminasal aplasia, mental retardation, callosal agenesis, microphthalmia, heminasal hypoplasia, and atypical clefting syndrome. Patients with this congenital craniofacial malformation need proper evaluation with the help of a computed tomography scan to assess the growth of facial and skull bones. The esthetic aspects and psychological problems are often a concern for patient’s families. The cartilaginous part or bony support of the nose can be reconstructed following proper growth of the face. Here, we presented a male neonate of 15 days with PL of the nose. There was no evidence of cleft lip or cleft palate. Parents were counseled for staged repair at an early age to avoid anxiety and to allow proportionate growth of the nose and face.

Supernumerary Nostril, Proboscis Lateralis and Polyrhinia

Supernumerary nostrils can be divided into three categories: extranostrils added to normal pair of nostrils as simple supernumerary, Proboscis lateralis when only one nostril present and a new nostril develop later, Polyrhinia is described as double set of noses in a single face. GnRH required for growth and manifestation of sexual organs originated at nasal placodes and settles at hypothalamus later. Formation of a new nostril requires a new nasal placode to form and grow.

Coexistence of proboscis lateralis and multiple craniofacial, neurological, cardiac and spinal deformities: a one-of-a-kind case report

BackgroundProboscis lateralis (PL) is an uncommon congenital facial deformity marked by the protrusion of a primitive tubular structure made up of skin and soft tissue that generally emerges from the eye's medial canthus and is associated with some craniofacial deformities. We report the first case of PL with multiple craniofacial, neurological, cardiac, and spinal anomalies.Case presentationA full-term female baby delivered by cesarean section cried immediately at birth. The mother reported having a normal pregnancy but has a history of x-ray during her first trimester. The baby was born with a rare presentation of proboscis lateralis which was accompanied by multiple anomalies, including but not limited to bilateral colpocephaly, corpus callosum agenesis, complex cyanotic congenital heart disease, and hemivertebra of the T10 body.ConclusionPL is an uncommon congenital condition that causes a variety of craniofacial abnormalities. Multiple additional defects affecting various organ systems should also be evaluated in a person diagnosed with PL.

Proboscis Lateralis With Basal Encephalocele: A Report of Clinical Management and Reconstructive Approach.

Proboscis lateralis is a rare craniofacial anomaly in which a rudimentary nasal appendage arises at the medial canthal area. The severity depends on organ involvement, including eyes, nose, cleft lip/palate, and/or concomitant intracranial anomalies. Here, we present a child with proboscis lateralis and associated trans-ethmoidal encephalocele. We suggest doing the preoperative CT and/or MRI to rule out associated intracranial anomalies and reliably preoperative planning tools. Moreover, we proposed an alternative nasal reconstructive technique using a composite graft from the proboscis mass at the same time as encephalocele repair with promising results.

PROBOSIS LATERALIS

Proboscis lateralis is an embryological inconsistency which is identified with the middle facial cleft. This outcomes in fragmented arrangement of one side of nose. This underdeveloped nose structure is situated close to the nose but off the midline. It seems like a cylinder and is appended at the undeveloped combination line anytime between the foremost maxillary interaction and the frontonasal cycle. This issue ought to be tended to at an early age and with stage shrewd methodology as this proboscis itself is a valuable design for remaking. We report an instance of proboscis lateralis in a 1-year-old female patient.

VP10.17: Lateral proboscis associated with lip and cleft palate: prenatal approach in abnormal placental invasion case

VP10.17: Lateral proboscis associated with lip and cleft palate: prenatal approach in abnormal placental invasion case

Proboscis lateralis with cleft lip and palate and its surgical management

Proboscis lateralis (PL) is an uncommon facial abnormality presented as soft tubular structure with most often originating from the medial canthal region. PL is an ideal choice for heminose reconstruction, provided started at a younger age. This multiphase treatment implements the planning of cartilaginous supports in the late teenage. A 6-year-old female patient diagnosed with PL, conjoined with craniofacial deformities such as cleft lip and palate is discussed in this case report.

Proboscis Lateralis: A Case Report of Nasal Aplasia with Complete Agenesis of Maxilary and Ethmoidal Sinuses

We report a child with Proboscis Lateralis with nasal aplasia and complete agenesis of maxillary and ethmoidal sinuses. This is a rare congenital malformation. Embryological basis has not been proven, and it is often associated with facial malformation. However, no such abnormality was noted in our patient. This condition is diagnosed clinically and confirmed radiologically of which CT scan plays a pivotal role. Reconstructive surgery is planned at the age of fifteen years.

Updates on congenital lacrimal drainage anomalies and their association with syndromes and systemic disorders: A major review

BackgroundTo review and update the syndromic and non-syndromic systemic associations of congenital lacrimal drainage anomalies. MethodsThe authors performed a PubMed search of all articles published in English on congenital lacrimal drainage anomalies (CLDA). The current review provides an update from January 2017 to August 2020 on all CLDA associated with clinical syndromes or non-syndromic systemic disorders. The update intends to appraise the readers on all papers that were published in the interim 3-year period since the publication of the previous major review by authors (1933–2016). Patients with specific syndromes or systemic disorders were then reviewed. Pertinent cross-references from each of the short-listed articles were also included. Data reviewed include syndromic descriptions, systemic details, demographics, lacrimal presentations, management, and outcomes. ResultsThere have been significant new updates. Eleven new syndromes have been added to the list of syndromic associations, of which three were suspects. Among the new syndromic associations, three (PHACE, Williams–Beuren, and Peter’s plus syndromes) described CLDA details in depth. Several new non-syndromic systemic conditions with associated CLDA also came to light, the foremost amongst them being proboscis lateralis, diprosopus dirrhinus, cerebral palsy, and NGLY-1 related disorders. Although familial presentations have been reported, the inheritance patterns are unclear for most anomalies. Surgical challenges in these patients are distinct, and a thorough pre-operative assessment, including detailed imaging when indicated, may facilitate good outcomes. Two updated tables reflecting the summary of syndromic and non-syndromic systemic associations are provided to capture the details at a glance. ConclusionIt is not very uncommon to find CLDA in syndromic or non-syndromic systemic disorders. Diagnosis of a craniofacial syndrome should prompt the physician to look out for CLDA. Similarly, a diagnosis of multiple CLDA should alert the examiner for the possible presence of associated systemic anomalies.

Triple nostrils in a calf

BackgroundNasal abnormalities are rare in bovines. In humans, nasal deformities are mainly classified as proboscis lateralis or supernumerary nostrils. This report discusses the etiology of triple nostrils in a calf, based on computed tomography, magnetic resonance imaging, and endoscopy.Case presentationA female Holstein calf presented with triple nostrils. The following abnormalities were observed: (1) formation of a small and flat blind-ended middle nostril between the right and left nostrils; (2) presence of a hair-bearing surface on the muzzle; (3) abnormal curvature of the nasal septum, resulting in a narrower right nasal cavity due to transformation of the nasal bones; and (4) formation of a bone-like structure within the nasal septum. These findings were similar to those of supernumerary nostrils in humans.ConclusionsTo the best of our knowledge, this represents the first description of a calf with triple nostrils. The use of imaging modalities is necessary for investigating the etiology of triple nostrils.

The arrhinias: Proboscis lateralis literature review and surgical update

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. In this case it is a ‘hemi-arrhinia’ (or heminasal agenesis. The arrhinias represent three groups of anomalies, each with different levels of clinical severity, some involving association with the labio-palatal cleft or agenesia of the premaxilla (1). In PL the nasal cavity on the affected side is replaced by a tubular appendage located off-center from the midline of the face, arising commonly from the medial aspect of the roof of the orbit (2). It is usually associated with heminasal aplasia or hypoplasia, microphthalmia, and — less commonly — with midline clefting. Associated brain and cranial vault anomalies are seen in 19% of these patients. PL is usually unilateral, with very few symmetrical/bilateral cases being reported (3). Morpho-aesthetic and psychological problems are frequent concerns for the patients and their families.In this study, the authors describe a clinical case and the chosen surgical technique, as well as reviewing the alternative techniques present in the literature.

Proboscis lateralis: A case report of a rare giant craniofacial teratoma in an infant

Teratomas can occur in almost any region of the body and are the most common extragonadal germ cell childhood tumors. However, craniofacial teratomas are rare. Craniofacial teratomas can present unique features and cause significant functional and aesthetic concerns. There are complex lesions that can have components intra-cranially and extra-cranially. Therefore, their management requires significant multi-stage multidisciplinary surgical procedures. Herein, we present a case of craniofacial teratoma in a child with the phenotype of proboscis lateralis that highlights some of the pertinent point of the diagnosis and management of congenital neonatal teratomas.

Proboscis Lateralis

A 9-month-old male child born out of nonconsanguineous marriage was brought with the history of abnormally bisected nose since birth (Fig. 1). On examination, the child was able to fix and follow the light in each eye separately, but because of the broad nasal bridge, there was pseudoesotropia. Anterior segment and fundus examination was essentially within normal limits. The external nasal openings were widely displaced on either side with a broad sheet of skin between them; nasal examination revealed patent nasal openings on each side, which were independently opening in the nasopharynx and the nasolacrimal system on each side was patent.Figure 1.: Proboscis Lateralis with deformed nose.There are 4 groups of proboscis lateralis (proboscis is a blind ended tube-like structure located in the midface) according to Khoo. Group I affected individuals will have proboscis lateralis with a normal nose. Group II individuals will have proboscis lateralis with an ipsilateral deformed nose. Similarly, Group III patients will have proboscis lateralis with an ipsilateral deformed nose, eye, and ocular adnexa and Group IV patients have an ipsilateral deformed nose, eye, ocular adnexa, and cleft lip or palate.

Proboscis Lateralis with Rhinosinusitis

Objective: This report aims to describe unique manifestations of proboscis lateralis and highlight the importance of a multidisciplinary approach to address the problems that arise from this rare congenital anomaly.
 Methods: 
 Study Design: Case Report
 Setting: Tertiary Government Hospital
 Patient: One
 Results: A 13 year-old girl diagnosed with proboscis lateralis presented with a trunk-like appendage projecting from the surface of the right supramedial canthal area. She also had clear nasal discharge, nasal congestion, mouth-breathing, and snoring since birth. Paranasal Sinus (PNS) CT scan with 3D reconstruction showed agenesis of the right paranasal sinuses, and expansile aeration of the left paranasal sinuses. Due to her condition, the drainage system of the paranasal sinuses was obstructed, causing chronic rhinosinusitis (CRS). This hindered plans for reconstructive surgery despite medical management, hence the patient underwent Endoscopic Sinus Surgery (ESS).
 Conclusion: Proboscis lateralis is a rare congenital anomaly that results in aesthetic problems as well as airway concerns, such as rhinosinusitis and obstructive sleep apnea syndrome (OSAS). Management entails a multidisciplinary approach to address functional and aesthetic problems of the patient.
 Keywords: proboscis lateralis, chronic rhinosinusitis, obstructive sleep apnea, endoscopic sinus surgery, multidisciplinary approach, plastic surgery, reconstructive surgery

Proboscis Lateralis : Report Of One Congolese Case

Introduction: Proboscis lateralis is known as a rare anomaly related to facial fusion defect. The optimal care of the patient with a multidisplinary approach that may involve an ophthalmologist, plastic surgeon, oro-maxillofacial surgeon or otolaryngologist has necessary.Case-report: It's a case note ofa 1 day-old female infant was consulted into our department because of the complaint of nasal anomaly. The physical examination revealed a tubular structure originated from the medial canthal region, an absent left nasal structure or right heminasal hypoplasia with a normal right nasal structure. According to those signs, the diagnostic of proboscis lateralis was given.Discussion: PL is known as a rare anomaly related to facial fusion defect.The precise embryologic mechanism responsible for the development of PL has not been defined.Nowadays, it does not seem to be any racial predilection in proboscis lateralis.In this case, it is very difficult to prove the association between: the embryologic mechanism of PL, the bad follow up of pregnancy, and the prenatal history of exposure to alcohol, drugs auto medicationConclusion: Ignorance and poor management, and lack of equipment yield impossible the care of proboscis lateralis's patient in our context.

Proboscis Lateralis: A Unique Case with Choanal Atresia and Bilateral Ophthalmopathy

Proboscis Lateralis (PL) is a rudimentary nasal appendage which is located off-center from the vertical midline of the face. It is a very rare craniofacial malformation frequently associated with abnormalities of the eyes and/or ocular adnexa. We report a unique case with bilateral ophthalmopathy and choanal atresia.

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.

Congenital Lateral Nasal Proboscis with Orbital Mass

ABSTRACT Proboscis lateralis is a rare craniofacial malformation frequently associated with congenital ocular abnormalities. These abnormalities included anophthalmia, microphthalmia, microcornea, lenticular opacities, cyclopean eye, and colobomas of the choroid, retina, iris and eyelids. We report a case of unilateral proboscis lateralis in a newborn infant with an associated solid orbital mass. How to cite this article Kaur B, Bansal S, Saikia UN, Gupta P. Congenital Lateral Nasal Proboscis with Orbital Mass. Clin Rhinol An Int J 2014;7(3):139-141.

Proboscis lateralis

Proboscis lateralis is a rare congenitally acquired facial abnormality characterized by a soft-tissue tube- or trunk-like appendage projecting from the surface of the face, most frequently rooted in the medial canthal region. Proboscis lateralis is generally associated with a wide range of concomitant craniofacial anomalies, giving rise to multiple theories describing the embryological pathogenesis and various classification systems to account for the pathological associations. This paper provides a literature review of the rare manifestations of proboscis lateralis and represents a summary of current literature related to embryological pathogenesis, definitive diagnosis, and surgical management approaches.

New Classification Scheme of Proboscis Lateralis Based on a Review of 50 Cases

Among congenital nasal deformities, proboscis lateralis is one of the rarest. Boo-Chai classified proboscis lateralis into four groups. Recently, we encountered a new case of proboscis lateralis with median cleft lip. We noticed that this classification had not been considered according to convalescence and embryologics, and further refinement seemed to be needed. We reviewed all cases of proboscis lateralis reported in English through 2009 and classified them by intercanthal distance. A total of 34 studies involving 50 cases were reviewed. Six cases were identified as having normal intercanthal distance. Three of them presented nose abnormalities and fit Boo-Chai group II category. The other three were consistent with group I. Hypertelorism was observed in 27 cases and was further divided into two groups based on the occurrence of a frontal encephalocele. Seventeen cases without a frontal encephalocele were compatible with Boo-Chai groups III and IV. The other 10 cases associated with a visible encephalocele had encephalopathy; most died at an early age, and long-term survival cases suffered developmental delay and mental retardation. Seventeen cases were defined as hypotelorism, and all cases also presented as holoprosencephaly. The redefined classification contains two new groups: group V as hypertelorism with encephalocele and group VI as hypotelorism. A new classification scheme is proposed as not only convenient for clinical application but also embryologically accurate.