13.1 Correction of Unilateral Cleft Lip Nasal Deformity

The nasal floor and the nostril sill are usually reconstructed during primary lip repair, which should be focused specifically on the repair of the divided oral ring muscle. The ala on the cleft side is repositioned symmetrically with the healthy side and sometimes the lower lateral cartilages (LLCs) also are fixed in a more symmetric way. Only a few surgeons try to position the dislocated anterior septum in the midline, which always follows the dislocated anterior nasal spine (ANS) to the noncleft side. New concepts attempt to reach early symmetry of the nostrils and the tip through subtle techniques without interfering with cartilage growth and avoiding the typical severe nose deformities that stigmatize these patients. ¹ – ³

13.2 Surgical Anatomy

According to Huffmann and Lierle, ⁴ the classic cleft nose is characterized by 22 features, although not all of them are typically present in every patient.

The external examination reveals a deviated nose to the healthy side due to the characteristic septal deformity. The anterior septum follows the dislocated ANS to the noncleft side. Therefore, the caudal border is always subluxated to the noncleft side. Very often, there is also an asymmetry of the nasal bones, so that not only the cartilaginous nose is deviated.

Because of the malposition of the ANS, and thereby induced deformity of the caudal septum, there is always an oblique columella with asymmetric nostrils. In addition, the columella on the cleft side is shortened and the LLC (alar cartilage) on the cleft side presents an S-shaped distortion and is flattened and displaced laterally and/or cranially. Therefore, the nostril on the cleft side is horizontally orientated and presents as an oval shape in contrast to the physiological oblique orientation of the nostril on the noncleft side.

The endonasal examination reveals a bowed septal deformity toward the cleft side, and the septum is deviated in all three planes—a so-called difficult septum being the result. Compensating for this typical deformity, the inferior turbinate of the noncleft side quite often shows a hypertrophy. The most lateral part of the lateral crus on the cleft side slants into the vestibule and forms the vestibular plica. The sill is often missing, depending on the effect of the alveolar bone grafting that is mostly performed at the age of 10 years. There might be a bony deficiency with a depression.

13.3 Indication

In many patients with a unilateral CLP deformity, the correction of the nasal deformity has a high priority because it produces characteristic stigmata in the middle of the face. Upon meeting another person, usually you look into their eyes, but with a conspicuous nose like a cleft nose, your gaze will be drawn to it. This is realized and noticed by these patients, and it affects them.

However, what worries them is not only the typical distortion of the nose, but also the functional impairment. Typically, there is a blockage in the cleft side and quite often breathing is blocked on both sides.

The nose correction is, therefore, an essential part in the rehabilitation of CLP patients.

13.4 Surgical Principles

The goal of surgery is straightening the deviated internal and external nose, creating symmetry of the nostrils, and giving a good contour to the tip. By correcting the anatomical deformities, good function can be achieved.

In our concept, most of these severely deformed septums need an extracorporeal septal reconstruction, which means a temporary explantation of the whole septum. ⁵ Because this drastically affects the growing zones, we only perform this kind of surgery 1 year after menarche in girls and 1 year after a change of voice in boys.

13.5 Operative Technique

Because of the complexity of surgery, we perform all cleft nose corrections under general anesthesia.

There are five complex problems that need to be addressed:

1. 
   

   The deformed septum. To straighten the nose, we need a straight septum. Because the septum presents mostly a three-dimensional deformity, in most cases we perform an extracorporeal septal reconstruction.

   
   
2. 
   

   The displaced anterior nasal spine. Not only must the septum stay in the midline, but the malpositioned ANS also has to be fixed in its normal anatomical position. Therefore, in all major ANS displacements, we osteotomize the ANS, transfer it into the midline, and fix it there by microscrews and microplates.

   
   
3. 
   

   The deviated nose. Because in most cases an asymmetric nasal pyramid is seen, we straighten the bony pyramid via parasagittal medial osteotomies as well as percutaneous lateral and transverse osteotomies. ⁶

   
   
4. 
   

   The distorted ala and deformed nasal tip. To reach symmetry of the nostrils, we generally perform a lateral crural steal technique on the cleft side and then replace the missing lateral crus with a cartilage graft.

   
   
5. 
   

   The malposition of the alar base. Correction of the ala base is optional because this deformity depends a great deal on the previous surgery. There is a wide variety of typical ala base asymmetries, but in most cases the ala on the cleft side is too far lateral and too caudal.

13.6 Septal Correction, Part 1

Using an open approach, we dissect the anterior septal angle, expose the caudal border, perform an extramucous dissection, and cut the upper lateral cartilage (ULC) from the septum. Then the upper and the lower tunnel of both sides are dissected. To remove the whole septum—if possible, take it out in one piece—a parasagittal medial osteotomy is performed. The author prefers a Lindemann fraise (Medicon Company, Tuttlingen) for that maneuver because an exact, straight bone cut can be achieved with this tool, while simultaneously removing some bone that otherwise might block the bone fragments during repositioning. ⁶ To avoid any bad fractures, which might extend into the cribriform area, we make a bone cut downward from the bony dorsum with an angle of about 60 degrees. After dissecting out the base of the septum from the maxillary crest, we fracture the bony septum by pressing with a 5-mm chisel. It is important that the whole mucosal wall be freed from the septum before this step so that the mucosa will not tear during its removal.

To achieve a straight neoseptum, or at least a straight L-shaped frame with the adequate dimensions, the length of the original dorsum and of the original caudal border have to be measured. Very often the explanted septum can be rotated 90 degrees so that the bony–cartilaginous junction becomes the new dorsum and the original dorsum gets the caudal septal border. Bent or weak parts are best splinted with thinned ethmoid bone. For reconstruction of the internal nasal valves and for keeping the neoseptum straight, we always apply spreader grafts. The use of spreader flaps is possible, but in these cases it is technically difficult. Thickened parts of the septum are always flattened with a cylindrical drill.

13.7 Septal Correction, Part 2

After bringing the fragments in the right position, replantation of the neoseptum starts. Safe fixation of the replant is essential for success.

After smoothing the dorsal line by trimming the nasal bones with a drill and the ULC with a scissor, the neoseptum is put back. To avoid the need of a columella strut, the neoseptum is put in a more caudal position to allow a tongue-and-groove technique for tip correction.

First, the cartilaginous dorsum is reconstructed by fixing the neoseptum to the ULC with horizontal mattress sutures, followed by reconstruction of the keystone area. This is a most important step. The best and easiest way to fix the neoseptum to the nasal bones is a crisscross technique. After drilling a hole into the cranial–caudal part of the right nasal bone, a 4–0 PDS suture is placed downward and penetrates the upper edge of the LLC of the opposite left side. Then the nasal bone on the left side is perforated and the same thread is placed analogous through the right ULC—so that, in the end, the thread can get knotted on the outer side at the junction from the nasal bone to the ULC on the right side.

In the end, the neoseptum is fixed to the ANS, respectively, to the microplate (see above). The length of the caudal border can be adapted by trimming the septum at its base. Then the anterior septum is fixed with three passes of a nonresorbable suture either through a drill hole in the ANS or directly to the microplate in case a transposition and osteosynthesis of the ANS is necessary.

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