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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 8  |  Issue : 1  |  Page : 56-59

Proboscis lateralis with cleft lip and palate and its surgical management


Department of Cleft And Craniofacial surgery, GSR Institute of Craniofacial Surgery, Hyderabad, Telangana, India

Date of Submission07-Jun-2020
Date of Acceptance18-Sep-2020
Date of Web Publication13-Jan-2021

Correspondence Address:
Dr. Megha Sahu
GSR Institute of Craniofacial and Facial Plastic Surgery, No. 17.1.383/55, Vinay Nagar Colony, I.S Sadan, Saidabad, Hyderabad - 500 059, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jclpca.jclpca_16_20

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  Abstract 


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.

Keywords: Cleft lip and palate, craniofacial anomaly, heminose, lateral proboscis


How to cite this article:
Reddy SG, Sahu M. Proboscis lateralis with cleft lip and palate and its surgical management. J Cleft Lip Palate Craniofac Anomal 2021;8:56-9

How to cite this URL:
Reddy SG, Sahu M. Proboscis lateralis with cleft lip and palate and its surgical management. J Cleft Lip Palate Craniofac Anomal [serial online] 2021 [cited 2021 Apr 11];8:56-9. Available from: https://www.jclpca.org/text.asp?2021/8/1/56/298624




  Introduction Top


Proboscis lateralis (PL) is an primitive nasal appendage, 2–3 cm in its greatest dimension, representing the incomplete formation of external nose framework.[1] It has more female prevalence with a ratio of 2:1. It can exist unilaterally or bilaterally.[2] It was first documented in the year of 1869 by Foster in a monograph entitled “Congenital Malformations of the Human Body.”[3] A 1 in 100,000 incidences makes this aberration a rare one[4].

These can occur as an isolated entity or in association with conditions such as, meningoencephalocele, microphthalmos, Arhinencephaly, heminasal aplasia/hypoplasia, and retardation.[2],[5]

PL is usually confined between the fusion lines of frontonasal process with the maxillary process[6] [Figure 1].
Figure 1: Various localization points of proboscis lateralis[6]

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  Case Report Top


A female patient 6 months of age reported to our institute with cleft lip, alveolus, palate and associated with a consolidated structure, tube shaped with dimensions of 2 cm × 1 cm stemming from the medial canthus, a 5 mm crevice distally. This en routed into a 2 cm long canal along the appendage. Lower eyelid revealed a cleft near the inner canthus of the left eye. Columella was absent, flat nasal bridge, and single nostril was present. On palpation, tubular structure was soft in consistency and bridge of the nose revealed a deficiency in the formation of nasal bone. The clinical picture helped to arrive at the diagnosis of lateral proboscis with cleft of upper lip, alveolus, and palate [Figure 2]a,[Figure 2]b,[Figure 2]c. The patient was worked up for the surgery.
Figure 2: A 6-month-old child with left-sided proboscis lateralis with cleft of upper lip, alveolus, and palate. (a) Front view, (b) worm view, (c) intraoral view

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A staged surgical procedure was planned.

Stage I: At the age of 6th month, cleft lip repair was performed with Afroze incision along with joining the PL to existing left hypoplastic nostril. This is done by incision on the tubular structure medially with further meticulous dissection layer by layer. The hypoplastic nostril is opened up at the lateral wall, creating space for proboscis [Figure 3]a and [Figure 3]b.
Figure 3: (a) Cleft lip repair, and (b) the lateral wall of left hypoplastic nostril was opened and proboscis sutured

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Stage II: Cleft of soft-palate repair was performed at the age of 1 year, in three layers nasal, muscle, and oral mucosa by using Delaire's technique [Figure 4]a.
Figure 4: (a) Cleft of soft palate repair. (b) Cleft of hard palate repair

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Stage III: Cleft of the hard palate was performed at the age of 2 year, by using two flap technique [Figure 4]b.

Stage IV: Lateral proboscis correction was performed at the age of 8 years. Preoperative view of lateral proboscis [Figure 5]a and [Figure 5]b. The right heminose was taken as a template from the proboscis, the reverse of the same was considered to design the left heminose [Figure 6]a. The mid of columella to the alar base dimensions was taken as a guide to mark and create the left alar subunit. After determining its final location, it is secured in place with 5-0 polyglactin sutures. The nasoseptal lining was recreated by de-epithelizing the mid potion of proboscis. This was sutured to the nasal septum (5-0 polyglactin) [Figure 6]b. The future nostril was reformed by horizontally incising the aplastic one. This was followed by suturing the deepithelized skin over the proboscis to the opposite nostril, eliminating furrows. The suture removal was done after 2 weeks and regular follow maintained up to 15 months [Figure 6]c and [Figure 6]d. Definitive rhinoplasty can be planned on the later stage at the age of 16th years.
Figure 5: Preoperative view of proboscis lateralis. (a) Front view, (b) worm view

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Figure 6: Lateral proboscis correction. (a) Foil template made; (b) proboscis was sutured; (c) postoperative follow.up after 1 year 3 months (front view), (d) worm view

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  Discussion Top


PL results in a recognizable deformity involving the facial structures. Conjoined with craniofacial deformities such as cleft lip and palate was a clinical picture of our reported case. PL occurs due to the displacement of the nasal placode during the formation of the nose while cleft lip and palate is usually confined between the fusion lines of frontonasal, maxillary, and mandibular processes. Khoo[2] recommended the formation of a classification defining the four clinical groups [Table 1].
Table 1: Boo-Chai classification system[2] (1985) into four distinct categories based on clinical presentation

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Sakamoto et al.[8] revised Khoo's classification by the inclusion of two groups based on interorbital distance [Table 2].
Table 2: Sakamoto et al.[8]; (expanded Boo-Chai classification system) based on interorbital distance (2012)

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Ultrasound proves to be a promising tool aiding in the diagnosis of the anomaly at the prenatal period. Computed tomography and magnetic resonance imaging help in detecting cerebral changes.[9]

Initially, treatment of PL was recommended simple surgical excision. Lately, reconstruction lays an important step in the surgical management of PL. Patent nasal airway allows the proboscis to be spilt all through the length, sutured to the dorsum of the nose and pyriform aperture. If the affected side is cavity free, the deepithelized proboscis could be transversed and secured under the bipedal flap.[7] Facial symmetry is an arduous goal, multiphase management including reconstruction ladders and revisions are required to achieve it.[10]

Surgical repair at a younger age provides favorable outcomes in terms of esthetic and psychological point of views.[7]

Khoo suggested[2] that for Group I, simple excision (amputation) was as a multifaceted path is Group II–IV's requirement. He also recommended dilation of proboscis before the initiation of reconstruction as the tube opening attachment is facilitated to the normal heminose. This also eliminates a narrow opening and size discrepancies.

Once the last growth spurt is achieved, the final esthetic revisions are done.


  Conclusion Top


We conclude that lateral proboscis coupled with other craniofacial anomalies form a challenging entity due to its functional and esthetic concerns.

A multiphase management in terms of surgery and long-term close follow-up to evaluate the recreated units.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Sakamoto Y, Nakajima T, Miyamoto J. A rare case of proboscis lateralis with median cleft lip. Cleft Palate Craniofac J 2010;47:553-6.  Back to cited text no. 1
    
2.
Khoo BC. The proboscis lateralis – A 14-year follow-up. Plast Reconstr Surg 1985;75:569-77.  Back to cited text no. 2
    
3.
Foster A. Die Missbildung Des Menschen Systematisch Dargestellt. Jena: F Mauke; 1861.  Back to cited text no. 3
    
4.
Acarturk S, Kivanc K, Atilla E, Sekucoglu T. Proboscis lateralis: Evaluation of the anomaly and a review of two cases. Plast Reconstr Surg 2006;117:2119-27.  Back to cited text no. 4
    
5.
Magadum SB, Khairnar P, Hirugade S, Kassa V. Proboscis lateralis of nose-A case report. Indian J Surg 2012;74:181-3.  Back to cited text no. 5
    
6.
Thorne MC, Ruiz RE, Carvalho J, Lesperance MM. Proboscis lateralis: Case report and review. Arch Otolaryngol Head Neck Surg 2007;133:1051-3.  Back to cited text no. 6
    
7.
Eroglu L, Uysal OA. Proboscis lateralis: Report of two cases. Br J Plast Surg 2005;58:124-5.  Back to cited text no. 7
    
8.
Sakamoto Y, Miyamoto J, Nakajima H, Kishi K. New classification scheme of proboscis lateralis based on a review of 50 cases. Cleft Palate Craniofac J 2012;49:201-7.  Back to cited text no. 8
    
9.
Abou-Elhamd KE, Al-Hewaige MT. Proboscis lateralis: Clinical and radiological features. J Laryngol Otol 2005;119:158-60.  Back to cited text no. 9
    
10.
David LR, Sanger C, Fisher D, Argenta LC. Proboscis lateralis: A rare craniofacial anomaly, reconstruction, and long-term evaluation. J Craniofac Surg 2008;19:1107-13.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
 
 
    Tables

  [Table 1], [Table 2]



 

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