The Nose, The Alveolar Arch and the Anterior Bony Palate in the Unilateral Cleft (incomplete and complete)
1. Anatomy and nomenclature: The height of the nose or its projection in profile is contributed mainly by the nasal bones in the upper one third and the anterior border of the nasal septum in its lower two thirds. The septum rises from the Vomer bone and in its upper half sends out two processes on either side like awnings called the lateral processes of the septal cartilage (LPSC) which in common usage are called upper lateral cartilages and therefore somewhat mistakenly assumed to be independent, which they are not. The two cartilages (LPSC) on either side are joined in the mid line by tough fibrous tissue. One is therefore left wondering if the septum itself is a bilamellar structure which later fuses imperceptibly to which we give the name septal cartilage. The lateral processes of septal cartilages at their superior margins are in close approximation to the nasal bones and joined by fibrous tissue. In the lower half of the nose there are two cartilages called major alar cartilages each with a medial and a lateral crus with a convexity in between. The medial crus is vertical, lies on the side of the septum and beyond its highest point courses laterally and posteriorly. The two alar cartilages on each side are joined by fibrous tissue in the form of a ligament in the midline and enclose the anterio-superior border of the septum and form a dome. The lateral crus which extends posteriorly in its lateral two-thirds is attached to the lateral process of the septal cartilage (LPSC). It is pertinent to note that the medial crus of the major alar cartilage does not rest on the pre-maxilla, over which the septum rests and the posterior end of the lateral crus does not rest on the alveolar bone. The link is therefore from above downwards, nasal bones to the LPSC, then to the major alar cartilages which join each other at the dome. The septum itself is firmly anchored to the nasal bones. There are two minor cartilages in the ala, also called the soft ala which rests on the alveolar bone (Fig. 1).
2. Pathological anatomy: In an unilateral complete cleft of the lip alveolus and the palate (or in the lesser variations of this deformity), the arrangement of the cartilages as shown in para 1 fig. 1 gets distorted. The location of the soft ala is lateral and also posterior if the cleft alveolus is collapsed. That in turn has a pulling effect on the medial crus of the major alar cartilage which is stretched and moves laterally. Because of all this the lateral crus of the major alar cartilages is unable to maintain its normal configuration and takes on a variety of shapes depending on the severity of the cleft. Generally speaking it becomes either straight or convex downwards instead of upwards and also lies more anteriorly rather than its normal posterior-superior direction. This lateral distortion has an effect on the nasal septum as well as the dome both of which get pulled to the abnormal side. As the anterio-superior part of the septum tilts, its lower half shifts into the zone of the normal nostril. If the distortion is severe, it may be dislocated from its pre-maxillary attachment and may actually enter the normal nostril. In the middle half of the nose this tilt of the septum causes narrowing of the nasal vestibule on the cleft side. However because the septal attachment to the nasal bones is very firm, they also tilt to the cleft side as growth progresses. The pathological anatomy described above is inferential and we do not know if the alar cartilage is merely deformed or also deficient and if it is deficient by how much and where. In fact it has also been inferred that though the nostril is stretched laterally there is in fact present a shortage of mucosa in the affected vestibule. On the other hand the alar rim not normally occupied by the great alar cartilage as it copes with the descent of this cartilage inferiorly expands the skin which hangs lower like a small apron in this area. Even a cursory glance at Millard’s cleft craft volume 1 and his operated cases shows that Millard has taken recourse to trimming this rim of skin after the cartilages have been repositioned (Fig. 2-4).
It is on this background that the seminal work by Harold McComb (Australia) needs to be looked at. He performed a rhinoplasty together with a cleft lip repair in early infancy in which he separated and fixed the deformed major alar cartilage to both the lateral process of the septal cartilage on the same side and also to a point on the contra-lateral side near the lower border of the opposite nasal bone. His is one of the rare papers where a ten year follow up of operated children showed that no further surgery was required for any residual deformity when anatomical restoration is done early (Fig. 5-6).
3. Surgical procedures in children: The template described by McComb has undergone some variations. Most agree that a rhinoplasty should be done early, certainly before the pre-school years.
a. Many surgeons only operate on the cleft lip and equalize the nostrils.
b. Some surgeons take a limited incision either through the columella or inside the nostril to hitch the affected cartilage at the appropriate location.
c. A few surgeons do a formal rhinoplasty with a proper wide exposure through a flying bird incision to dissect the lower alar cartilages, create the dome and hitch it across the nose at the time of cleft surgery.
d. There are others who undertake to centralize the septum at the same time.
In the case illustrated below a child with a complete cleft of the primary palate with a nasal deformity has had surgery both for the lip as well as the nose including work on the septum at a slightly older age of two and a half years (Fig. 7-15).
Contributed by Bharati Khandekar (Plastic Surgeon) and Nisheet Agni (Maxillofacial Surgeon) from Mumbai, India.
4. The basis of surgical treatment in adults:
In addition to the steps described above, the following also require to be performed in adults.
a. Sub-mucous resection of the entire somewhat crooked septum might require to be done leaving only its anterior part which is replaced in its normal groove in the pre-maxilla.
b. Occasionally osteotomies of the nasal bones to correct the nasal bony pyramid tilted to the cleft side might be needed.
c. Any residual deformation can be masked by a bone graft or a costal cartilage graft right angled in a single piece or in two separate parts, one as a columellar strut and another placed over the bridge of the nose which can also be shaped as a single right angled graft (Fig. 16-29).
Contributed by Bharati Khandekar (Plastic Surgeon) and Nisheet Agni (Maxillofacial Surgeon) from Mumbai, India.
THE ALVEOLAR ARCH
5. The alveolar arch on the cleft side in complete clefts of the primary and secondary palate in one word is (also called the lesser segment) ‘adrift’. It might be at a variable distance from the pre-maxilla, might lie posterior to it and when that happens it is commonly referred to as the collapsed arch. In lesser clefts only up to the incisor foramen, both in complete and incomplete clefts of the primary palate the alveolar arch is less deformed, might only show a notch, might be partially abutting the pre-maxilla but yet have a cleft within it and not infrequently abuts against the pre-maxilla but lies posteriorly. The variations in clefts of lesser magnitude are never treated actively, certainly not during infancy and are expected to or actually do mould with the rest of the arch (the greater segment) over the years with growth (Fig. 30).
Attempts to mould the severe alveolar deformities in clefts of the primary and secondary palate to facilitate surgery by reducing the width of the cleft has history stretching back to the 18th century. In recent times it has been generally referred to as pre-surgical orthopedics. An excellent, comprehensive but sharply edited review of the subject can be found in the book Management of Cleft Lip and Palate (Whurr publications, ed. Watson, Sell and Grunwell, written by Hathorn and McNeil). The beginning of surgical orthopedics was made by McNeil in Scotland, later popularized by Burston (Liverpool, England) in which a single intra-oral plate was made to hug over the two alveolar arches and the central part of the plate covered the cleft. The plate helped feeding and therefore is also called a feeding plate. The plate prevented the tongue from entering the cleft and instead pushed on the plate and therefore on the palate to facilitate growth of the alveolar arches and the bony palate as a single unit. The lateral growth that occurred on the cleft side overcomes the collapse by a centrifugal force. The anterior movement and the growth on the medial side of the lesser segment helps to reduce the cleft. The appliances were made as soon as possible after birth and needed to be changed as maxillary arches expanded / normalized with growth. The term pre-surgical orthopedics now seems to be replaced with a more persuasive and softer term called naso-alveolar moulding (NAM) and includes taping of the cheeks beginning on the non-cleft side as a fixed point and then on to the cleft side. The plate also incorporates a wire by which a nasal prosthesis is mounted to raise the height of the nostril (Fig. 31-44).
Contributed by Himanshu Dave from Mumbai, India.
The latter part of the chapter by Hathorn and McNeil mentioned above on pre-surgical orthopedics seems after analyzing the literature to discount any beneficial effects on facial growth and secondary dentition and concludes that this effort is time consuming, expensive and difficult even for the most diligent parents. The difficulties in the less developed countries are even more formidable. Naso-alveolar moulding without doubt makes for easier surgery but even here, long term results do not conclude that wide mobilisation for repairing wide clefts produced any adverse results as compared to cases where the alveolar arches have been moulded pre-operatively. All in all we need to remind ourselves that clefts result in distortion but the clefts could be both due to failure of fusion as well as inherent deficiency which may manifest over the years.
THE ANTERIOR PALATE
6. The term anterior palate is somewhat vague. It is neither anatomical nor embryological and is only used when the palate is cleft and the surgeon attempts to close the palate partially or completely in the post-alveolar region as far back as possible towards the beginning of the soft palate. Some of the illustrations which show such closure include the opposing ends of the alveolar arches from which periosteal flaps are raised in continuity with the bony cleft. Two flaps are raised on either side of the cleft, one of them from the Vomer on the non-cleft side and the other from the nasal lining from behind the palate itself on the cleft side (Fig. 45).
When sutured the raw areas face the oral cavity, the flaps are somewhat fragile (certainly the nasal one on the cleft side) and they often survive only in patches but almost always somehow narrow the bony cleft when the wounds heal with secondary intention. This perhaps act as an adjunct to naso-alveolar moulding (see previous para) from inside by helping to narrow the cleft. Traditionally this repair is performed when the cleft palate is operated prior to the lip repair because the surgeon has better access to the area and parts of the vermillion which might be in excess can be used to bolster the muco-periosteal flaps in the region of the alveolar repair. The successful narrowing of the anterior-bony cleft also reduces the amount of mobilization of the oral muco-periosteal flaps in the final repair (please see the ensuing chapter). The reasoning being the lesser the mobilization, the lesser is the raw area adjacent to the alveolar arches (medially) and therefore lesser scarring and fewer deformities of the maxilla in the long term.
In a recent modification of the Vomer flap the flap raised from the Vomer extends beyond the Vomer to about two or three millimeters on the oral mucoperiosteum on the non-cleft side. The flap is then swung over, the oral mucoperiosteal flap on the cleft side is raised, the vomer flap is placed below it and held with a forceps and a hole is drilled through and through the mucoperiosteum on the cleft side, also through the bone and the nasal layer beneath it with a hypodermic needle and a thread passed through it and then retrieved from behind the palatal shelf on the cleft side and tied. The repair of the cleft lip then follows. The Vomer granulates rapidly and the cavity is soon epithelialized. In the last twelve consecutive cases done at the Godrej Hospital in Mumbai in the Smile Train Project all flaps have survived, remained stuck to the new position and the one layer closure has had no fistulae. In this method the opposing surfaces of the alveolar arches are not denuded and are left untouched for them to close in and abut naturally (Fig. 46-52).
7. Early surgery of the septum (in infants): A major flaw of this chapter is the lack of photographs of any satisfactory long-term results of rhinoplasties done in childhood on patients with clefts. Unfortunately there have been very few papers showing long-term results with a single unchanged protocol of rhinoplasties in children with clefts after Harold McComb published his paper (his method is shown in fig. 6). Some papers do show excellent results in the frontal view but the worms’ eye view is usually disappointing because it shows inequality of the nostrils. It is interesting to note what Sir Harold Gillies wrote together with Kilner in 1932. As quoted by Millard they say, “hitherto this has proved a stumbling block to all surgeons. Optimism in this connection is however justified, for the structural defects underlying the deformity are gradually being made clear and an accurate diagnosis is therefore becoming possible. Cure seldom anticipates diagnosis and is usually quick to follow it. Except from a viewpoint directly below the nostril one to which patients are seldom subjected to except for the preparation of a surgical textbook illustrations in which the nostrils can be made to appear symmetrical.” The expectations in the above quote are on the way to be achieved certainly on the frontal view and the introduction of hollow silicon splints in the nostrils very early are making a difference on the worm’s eye view as shown in many a scientific meetings (see para 8). In this connection the question of what to do with the nasal septum is not yet resolved. There is a long standing dictum that the nasal septum is a “no-go zone” in children for fear of deformities later in adulthood due to arrested growth if the septum is surgically treated. That has led to a rather extreme viewpoint that the septum’s deviation to the normal side in cases of clefts is best left alone and surgically treated at a later date. In fact the correction of this septal deviation and fixing it in its normal groove in the pre-maxilla without any interference with its mucochondrium together with the rest of the rhinoplasty is likely to allow the septum to grow normally when the alar cartilages are repositioned. Such an early correction is seen to be undertaken by many a surgeon but as mentioned earlier those cases have not been published in the long-term. In reality in injuries involving fractures of the nose in children when the septal cartilage is broken or avulsed, an active surgical intervention is always undertaken to prevent deformities in the future. A more radical approach for the septum together with alar cartilage repositioning has now been practiced by Doctors Adenwala, Narayanan, Vasant and Pashupati at the Charles Pinto Plastic Surgery / Jubilee Hospital, Kerala, India and is presented below (Fig. 53-57). This idea was first mooted by Dr. Charles Pinto of Mumbai while on a visit to the hospital. Unfortunately as it happens, long term results are not available. However the practice has been continued and some five year results are reproduced in figures 58-61. Here is the conundrum. Alar deformation leads to septal deviation. Only alar correction continues to allow the septum to grow abnormally because it is already deviated. Can a septal correction together with alar repositioning solve this problem? We can only speculate.
8. Use of silastic splints. There is no denying that splints are a valuable adjunct, both pre and post-operatively in many a deformity. For example, calipes equino varus, congenital torticollis or secondary contractures of deeper tissues following dermal burns because myofibroblasts are resisted only by splints. The rationale of intra-nasal splints is therefore sound but in a long-term follow up of these patients done by Philip Chen as quoted by Dr. Narayanan for this blog, the splints appeared to work the best when the nasal deformity is over-corrected after naso-alveolar moulding. In the paper by Philip Chen, the patients were divided in four categories (see figures below). The last of which was a combination of pre-operative NAM followed by a rhinoplasty in which the alar cartilaginous deformity was over-corrected. How much the over-correction should be depends on the experience of the operating surgeon (Fig. 62).
9. The last word on the subject has obviously not been written. Sir Harold Gilles has already been quoted in paragraph 7. The late Charles Pinto, head of plastic surgery department at KEM hospital in Mumbai used to say that it is in the treatment of the ‘cleft lip nose’ that the boys get separated from the men. As things stand, the men are few in number and even they have not been able to publish convincing results in the long term. Till then let us remember what Hamlet said in Shakespeare’s Hamlet, “Time is out of Joint, and it is my misfortune (o cursed spite) that ever I was born to set it right”.
As this chapter was being uploaded Dr. Adenwala’s unit from Trichur has sent photographs of some long-term follow-ups which are reproduced below.
Renu Parmar, Consultant Orthodontist, Bhagvan Mahavir Jain Hospital from Bangalore (Smile Train Project) adds the following:
The complete bilateral cleft lip and palate (CBCLP) are a real challenge in the field of cleft care. In bilateral cleft lip and palate (BCLP) presurgical nasoalveolar molding (PNAM) has a greater advantage. It not only repositions the protruded and often rotated premaxilla in a more anatomic position giving the surgeon a good platform to perform a tension free closure of the lip but at the same time gives a more esthetic over all outcome. Use of naso-alveolar molding (NAM) technique has eliminated surgical columella reconstruction and the resultant scar tissue in bilateral cleft lip and palate. NAM has been shown to significantly improve the surgical outcome of the primary repair in cleft lip and palate patients.
The cleft deformity is significantly reduced in size with the NAM therapy before surgery, making primary repair of the lip, alveolus and the nose an effortless procedure.
Long-term studies of NAM therapy indicate that the change in the nasal shape is stable with less scar tissue and better lip and nasal form. (Maull DJ, Grayson BH, Cutting CB, Brecht LL, Bookstein FL, Khorrambadi D, et al. Long-term effects of nasoalveolar moulding on three-dimensional nasal shape in unilateral clefts. Cleft Palate-Craniofac J 1999;36:391-7)
This improvement reduces the number of surgical revisions for excessive scar tissue, oronasal fistulas, and nasal and labial deformities. (Sato Y, Grayson BH, Cutting CB, Unilateral cleft lip and palate patients following gingivoperiosteoplasty. San Diego: American Association of Orthodontist; 1999)
Some CBCLP patients treated by PNAM therapy at Bhagwan Mahaveer Jain Hospital, Smile train Unit, Bangalore.