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Proboscis lateralis is a rare congenital anomaly and heminasal aplasia is the most common associated anomaly. The proboscis is an ideal donor structure that has good colour and texture match to reconstruct the heminose and should not be sacrified. We present the staged reconstruction of two patients using a subcutaneous tunnel method. An individualised approach to the patient determines the ideal method of reconstruction.
The proboscis is a tubular structure measuring 2–3 cm long and about 1 cm diameter arising from the medial canthal region. This tubular structure may contain a small lumen along its length. Atypical proboscis lateralis cases arising in the outer canthal region, nose and chin have been reported.
In very few cases, the nose is normal and heminasal aplasia or hypoplasia is generally associated with proboscis.
Heminasal aplasia or associated anomalies should be corrected early because this anomaly may cause psychological problems especially for the patient's family. Some heminasal reconstruction techniques using the tubular structure as a donor have been reported.
A 3 days-old male infant presented with right heminasal aplasia and cleft lip. A soft clublike structure measuring about 3 cm long and 1 cm diameter originated from the medial canthal region. Incomplete cleft lip was associated with ipsilateral proboscis lateralis (Fig. 1) . Epiphora was detected. There was no consanguinity between the parents. The other three female siblings were normal and there was no congenital abnormalities in the family. The pregnancy had been completely normal.
Fig. 1Group IV Proboscis lateralis—preoperative view.
The pedicle of the proboscis was thin and there was not a track in the proboscis. There was no nasal cavity nor ethmoid cells on the affected side on radiological evaluation. The nasolacrimal duct was closed on dacryocystography. This proboscis lateralis was categorised as group IV proboscis lateralis according to the associated deformities.
The patient was operated upon first at five months of age. A bipedicled skin flap was elevated on the cheek-nose region. The circumferentially deepithelialised proboscis was passed under the skin flap (Fig. 2) . In the second stage the pedicle was excised and adapted, and the incomplete cleft lip was corrected using the rotation-advancement technique. After several months the new nostril base was excised and two z-plasties were performed at the alar base and dome region to achieve symmetry. During three years follow-up there was no further problem and the patient's family is satisfied with the result (Fig. 3, Fig. 4, Fig. 5) .
Fig. 2Passed desepithelialised proboscis under the tunnel.
This one day-old male infant was brought into our clinic because of the complaint of nasal anomaly and a tubular mass originating from the medial orbital region (Fig. 6) .
Fig. 6Group III Proboscis lateralis—preoperative view.
The other two siblings were normal and there was no congenital anomaly in the family. Pregnancy had been completely normal although the mother had been treated for primary amenorrhea. On physical examination heminasal aplasia and proboscis was detected on the right side. The proboscis measuring 2.5 cm long and 1 cm diameter was originating from the medial canthal region and mucous excretion was observed from the dimple of the proboscis. Hypertelorism was detected and there was a coloboma on the right lower eyelid.
On radiological examination the nasal cavity and the ethmoid cells were absent on the affected side. There was a canal into the proboscis that was not opened to the nasopharynx but extended to the frontal region. The nasolacrimal canal was absent.
The operation plan was the same as in the first case. In the first stage the deepithelialised proboscis was passed under the bipedicled transverse skin flap. In the second stage, the pedicle was excised and the sinus was followed to the frontal region and resected. Clear fluid was seen at the deepest point and it was thought that it could be cerebrospinal fluid. A small fascial graft was used to occlude the fistula and antibiotic prophylaxy was started. There was no problem after the operation. Z-plasties were performed after several months at the nostril area. The patient has been following for three years and is waiting for the final nasal correction (Fig. 7, Fig. 8)
In group I, the nose is normal and this group is the least common type of proboscis lateralis. The most common is group II in which nasal abnormalities are associated with proboscis. In group III, eye abnormalities are seen with proboscis and nasal abnormalities. Cleft lip and/or palate is associated with all these abnormalities in group IV.
Heminasal hypoplasia or aplasia is the most common abnormality in proboscis lateralis. In nasal reconstruction regional flaps such as forehead flap or distant flaps can be used but it should not be forgotten that the proboscis is an ideal donor tissue. It is a natural tube that has a liberal blood supply. In typical cases, the proboscis is close to the nasal area and colour and texture match are features that should not be sacrified.
There are several nasal reconstruction methods using the proboscis as a donor.
We can divide reconstruction methods into two. In one method, the posteromedial surface of the proboscis is deepithelialised or the proboscis is split along its entire length and then a raw surface facing the proboscis is prepared in the aplastic nose area and sutured to it.
In the second stage, the base of the proboscis is excised and adapted. Consequently, only the distal part of the proboscis is used for nasal reconstruction and the most obvious defect in heminasal aplasia is at the distal part. There is no bulky apperance in the medial canthal region because of the excision of base of the proboscis. Another advantage of the subcutaneous tunnel method is that there is no long vertical scar. Horizontal scar contractures can be avoided by multiple w-incisions and burying the proboscis. This method needs one more stage than the other method.
To achieve nostril symmetry and get a better contour, minor surgical touch-ups such as excisions, z-plasty and dermofat graft can be performed in other stages.
In suitable cases, attempts can be made to reconstruct the nasal passage but in the absence of the nasal cavity the chances of a patent airway have been generally poor.
In our cases we did not attempt to create a nasal passage because there was no nasal cavity on the affected side.
In conclusion, the proboscis is an ideal donor tissue to reconstruct the nose in proboscis lateralis associated with heminasal aplasia. The texture and the localization of the proboscis, existing nasal cavity and the associated abnormalities such as complete cleft lip are the important factors in determining the reconstruction method. In our opinion, an individualised approach to the patient determines the ideal route for reconstruction.
References
English G.M.
Congenital anomalies of the nose, nasopharynx and paranasal sinuses.
in: English G.M. Otolaryngology. vol. 2. Lippincott,
Philadelphia1988: 1-39
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