As previously described (Pruzansky 1955), in complete unilateral and bilateral clefts of the lip and palate, after the lip is united, the overexpanded palatal segments move together, reducing the cleft width along its entire posterior length. Subtelny (1955), using laminographs, has shown that newborns with complete clefts of the lip and palate have wider than normal pharyngeal widths and the perpendicular plates of the sphenoids are distorted in their relationship. Aduss and Pruzansky (1967) have demonstrated that, in complete unilateral cleft lip and palate, any one of three arch forms can result after the lip is repaired (Fig. 6.1):
(a) CUCLP. Facial and palatal casts. (b) Complete unilateral cleft lip and palate (CUCLP) before (A) and after (B) lip surgery. With the establishment of muscle continuity, the lesser segment moves medially, while the premaxillary portion of the larger segment moves medio-inferiorly, both acting to reduce the cleft width. Any of the following segmental relationships can result. (B) No contact between segments. The inferior turbinate on the cleft side makes premature contact with the bowed nasal septum. (C) The premaxillary portion of the larger segment overlaps the smaller segment. (D) The segments form a butt joint showing good approximation. Pruzansky and Aduss have shown that there is no correlation between the original cleft width and the resultant arch form. Wider clefts seemed to demonstrate less of a tendency toward collapse than did the narrower clefts (Aduss and Pruzansky 1967)
The alveolar segments can move into end-to-end contact, producing a symmetrical arch form.
The alveolar segments can overlap, producing what is erroneously known as a “collapsed” arch form.
The alveolar segments can move closer together but not make contact. This occurs because of an inhibiting factor of the inferior turbinate on the cleft side, making contact with the distorted bulge of the nasal septum.
In a series of 58 patients who had no presurgical orthopedics or primary bone grafting, Aduss and Pruzansky (1967) found that approximately 43 % had overlap of the alveolar processes (mistakenly called collapsed arch). Among these patients, crossbites of the canine and first deciduous molar were the most common finding at 5 years of age. There were no anterior crossbites. Other investigators have reported similar results (Bergland 1973; Bergland and Sidhu 1974). Berkowitz (1985), in a serial study of 36 cases with complete unilateral clefts of the lip and palate in which the lip had been united between the ages of 3 and 5 months and the palatal cleft closed between 18 and 24 months using a von Langenbeck with modified vomer flap without neonatal maxillary orthopedics, showed that 5 of the 36 cases had a complete buccal crossbite which was corrected within 6–10 months with fixed palatal expanders. Cuspid crossbite was the most frequent occurrence and was due to angular palatal rotation as well as to ectopic eruption of the deciduous cuspids. The cleft and noncleft segments were in either a class I or class II occlusal relationship. In no instance were any of the segments in a class III relationship.
This confirms Berkowitz’s belief that the cleft palatal segment is not retropositioned within the skull relative to the mandible and that the maxillary-mandibular relationship is similar to that seen in the noncleft population. Whether the maxilla and mandible are both posteriorly positioned within skull has not been determined, and others (Semb 1991; Ross 1987a, b, c) have found this to be the case. Therefore, the palatal segments do not need to be brought forward by the use of neonatal protraction forces as Latham (1980) and his mentor McNeil (1950) have suggested. There are, however, some cases when, because of an unfavorable facial growth pattern coupled with a retruded maxilla relative to the anterior cranial bases, orthopedic protraction forces will be beneficial in the mixed (transitional) and permanent dentition.
According to Aduss and Pruzansky (1967), four factors govern arch form:
The size and shape of the alveolar process adjacent to the cleft. A bulbous and fully toothed alveolar process acts as an impediment to the collapse of the arch, whereas a thinly formed and dentally impoverished alveolar process leads to the overlapping of segments.
The size and shape of the inferior turbinate on the side of the cleft. A thick, rounded, well-modeled inferior turbinate can block excessive medial movement of the palatal segments.
The size and geometrical inclination of the nasal septum. A highly inclined septum with a contiguous bulbous turbinate will affect the movement of the palate and its final position.
The size and shape of the palatal shelves. Shelves of disproportionate size are more prone to overlap. One can certainly visualize that a long noncleft segment coupled with a short cleft segment will end up with the premaxillary portion overlapping the short cleft palate segment.
6.1 Facial Characteristics
Aduss (1971) in 1971 examined 50 males and 21 females with UCLP; their age range was between 4 and 14 years. He described craniofacial growth in the male cleft group as essentially equivalent to the female cleft group. He found that the gonial angle for the cleft patients was consistently larger than the noncleft group and the mandible appeared to be more retrognathic. He concluded that the craniofacial complex in the cleft sample tended to grow in a similar manner to that reported for the noncleft populations. The results of his study, based on a conservative method of surgery, negate the conclusions reached at the time regarding the deleterious effects of surgery on the growth of midface.
Hayashi et al. (1976) studied craniofacial growth in unilateral complete clefts using lateral cephalograms of 135 males and 120 females with an age range of 4–18 years. Control subjects included 120 noncleft males and 120 noncleft females of similar age to the cleft subjects. They concluded that the cleft group differed from the control group in several major respects: (1) Their overall growth trend showed a more downward or vertical direction; (2) the cranial base angle was more flattened; (3) the maxilla was smaller and was located in a more posterior and upward position; (4) ramal height was shorter, the gonial angle was more obtuse, and mandible was generally retrognathic; (5) upper face height was smaller and lower face height was greater; (6) underdevelopment in both the maxilla and the mandible was more pronounced in cleft females than in cleft males.
Smahel and Mullerova (1986), in 1986, studied 30 boys with UCLP prior to palatoplasty using cephalometry. A comparison with 27 normal individuals matched in age showed that most basic deviations of the craniofacial configuration recorded in adults developed at an early age, often prior to palatoplasty, i.e., reduced height of the upper anterior face, maxillary dentoalveolar retroclination, displacement of the upper jaw backwards, widening of some components of the maxillary complex, and a shortening of the mandibular body and ramus. Only the length of the upper jaw was not reduced. The shortening of maxillary dimension occurred postoperatively at a more mature age.
Later on 1992, Smahel et al. (1992) presented another study of craniofacial morphology in UCLP in 58 adult males. The results showed a shortening of maxillary depth, reduction of the upper face height, increased lower anterior facial height, and mandibular changes resulting from growth deficiency that consisted of shortening of the body and ramus, obtuse gonial angle, steep mandibular plane, and retrognathia.
Again in 1992, Smahel et al. (1992) studied growth and development of the face in UCLP during prepubertal and pubertal periods. He concluded that there were no definitive differences in the growth rate between the pre- and postpubertal periods. Therefore, the worsening of overjet during puberty could be due to the depletion of the compensation and adaptation after the previous orthodontic treatment rather than to the enhanced growth rate. In addition, he found that during the prepubertal period, the lower jaw showed a very slight posterior rotation, while during puberty, an anterior growth rotation was present. A marked retrusion of the maxilla developed already in the prepubertal period. During both periods, there occurred an identical impairment of sagittal jaw relations and of the upper lip prominence, accompanied by a flattening of the facial profile and reduction of the nasolabial angle.
In 1996, Smahel and Mullerova (1996) reported a longitudinal study regarding postpubertal growth and development of the face in UCLP as compared to the pubertal period. The data showed that in boys, facial growth persists after the age of 15 years and maxillary growth attains almost half the values recorded in the period of puberty, while mandibular growth attains almost the same values as during puberty. In girls, the growth is almost terminated except for the lower jaw, where it is still significant though several times slighter than during puberty. Due to the gender differences in the amount of postpubertal growth, developmental changes in facial configuration do not occur in girls during this period, while in boys, there is a further deterioration of maxillary protrusion, sagittal jaw relations, and flattening of the face.
In 1988, Hoswell and Levant (1988) reported another long-term follow-up of skeletal growth of UCLP subjects ranging in age from 8 to 18 years. Serial cephalographs taken every 2 years were utilized for determination of six cephalometric dimensions: anterior cranial base, upper and lower facial heights, posterior nasomaxillary height, maxillary horizontal length, and mandibular length. These were compared to published cephalometric standards of a noncleft group. All dimensions except mandibular length were smaller in the UCLP group. The horizontal maxillary length appeared to be most affected in UCLP. Mandibular length was not affected in the cleft group.
6.1.1 The Oslo Study
Because of the stable and long history of meticulous record keeping and protocols that characterizes the data acquisition of the Oslo team, the following studies on unilateral cleft lip and palate are presented to provide a unique perspective on treatment strategies and facial growth standards based on longitudinal data. The author does not follow the same surgical strategies as those of the Oslo team but recognizes that the differences are not significant enough to interfere with obtaining a successful long-term outcome.
Semb’s (1991) 20-year serial cephalometric study taken from the Oslo Archives gathered during Bergland’s leadership involved 76 males and 81 females (157 individuals) who did not have neonatal maxillary orthopedics. All of the children in the study had lip closure in infancy using a modified Le Mesurier or, after 1969, a Millard procedure. During the same operation, the nasal floor was closed using a one-layer vomer flap. The remaining posterior palatal cleft was closed between 4 and 5 years of age using a von Langenbeck palatoplasty. Secondary alveolar bone grafts from the iliac crest were placed at between 8 and 11 years of age. By 1974, all palate repairs were completed by 18 months of age. Superior-based pharyngeal flap surgery for velopharyngeal insufficiency was performed in about 20 % of the cases.
Compared with normal males and females, the pooled sample with unilateral cleft lip and palate showed (1) skeletal and soft tissue maxillary retrusion, (2) elongation of the anterior face (even though the upper face height was shorter), (3) a retrusive mandible, (4) reduction in posterior face height, and (5) a slight increase in the angle of the cranial base.
The pattern of growth also was different from that of noncleft individuals. Between 5 and 18 years of age, there was almost no increase in the length of the maxilla. There was a marked reduction in maxillary and mandibular prominence. Vertically, the excessive lower face angulations changed slightly.
Ross’s multicenter study involved data from 15 cleft palate centers around the world collected for the purpose of determining the effects of manipulative and surgical treatment on facial growth. A sample of 1,600 cephalometric radiographs of males with complete unilateral cleft lip and palate were traced, digitized, and analyzed in the Craniofacial Center of the Hospital for Sick Children. The seven series of studies considered virtually every aspect of treatment that might influence facial growth.
Ross concluded that the type of surgical repair used does not make an appreciable difference to facial growth. It appears, however, that there are differences that can only be explained on the assumption that some surgeons induce less growth inhibition than others. Variation of the timing of hard and soft palate repair within the first decade does not influence facial growth in the anteroposterior or vertical dimension. Ross admits that very early soft palate repair was not well represented in this study, and there is some suspicion that there might be untoward results.
Berkowitz et al. (2005) clinical findings suggest a different conclusion that, in most cases, early surgery (before 12 months) will have a negative effect on palatal growth in all three dimensions. It all depends on the size of the cleft defect relative to the area of the surrounding mucoperiosteum (see Chap. 7).
Ross’s study did not include palatal surgery from 6 to 12 months. This study also reported that the resulting face is flat in profile and decreased in depth, with a vertical deficiency in the midface and vertical excess in the lower face. The mandibles in these faces characteristically are slightly shorter in total length so that the chin is retruded. The occlusion is more of a molar and incisor mesiocclusion in clefts with less overbite and overjet. The soft palate in this sample is appreciably more posterior. The mandibular plane angle is greater, possible due to the need for more interincisal space.
Ross further stated that the bony pharynx was unaffected by treatment and that the variation in midface development can be attributed to maxillary length rather than to maxillary position. He also noted that the mandible is not directly affected by treatment. Facial growth is intrinsically compromised by an underlying deficit, and surgery acts to further interfere with growth of the midface by inhibiting forward translation.
The best results appear to follow lip repair at 4–5 months with no repair of the alveolus. Early alveolar repair restricts its vertical growth and should be avoided in individuals with poor growth potential. This leads to deficient midfacial height and poor vertical height proportions, with more acute nasolabial angles. There is no evidence that periosteoplasty will cause similar results. Berkowitz et al. (2005) study conclusively shows that periosteoplasty inhibits midfacial development, especially that of the premaxilla (see Chap. 10).
Ross further states that the maxilla in the UCLP is not more posteriorly positioned to any appreciable extent, but it is much shorter in length. The repaired lip affects the basal maxilla more than the alveolar process. Vertical development of the posterior maxilla is more deficient than the anterior part. The mandible is shorter with a steeper mandibular plane angle.
Hard and soft palate surgical repair procedures provide the greatest potential for inhibiting the maxilla in length, forward translation, and posterior height.
Kwon’s (1998) retrospective longitudinal study of the skeleto-facial growth in unilateral cleft lip and palate documented and evaluated the proportional craniofacial growth horizontally and vertically in 14 UCLP patients of the ages 5–18 years by using modified Coben’s basion horizontal analysis. There were three populations included in this study: The Eastman cleft group (sample size, 24) and the Miami cleft group (sample size 23) served as patient group, and the Bolton templates (ages 5–18) served as controls. Samples were divided into four age periods according to the chronological ages, and then the growth pattern of each period were evaluated and compared. A total of 301 images of lateral cephalograms were examined and digitized. These characteristics of the skeletal facial growth of the UCLP are summarized as: (1) There is no difference of posterior cranial base over time; (2) maxilla is positioned posteriorly relative to basion (BA) during the early ages and is getting retrusive due to the deficient growth with time; (3) upper anterior facial height (UAFH) is almost the same as the control; (4) lower posterior facial height (LPFH) is increased but is not as much as lower anterior facial height (LAFH); (5) lower anterior facial height (LAFH) is significantly increased; (6) total facial height (TFH) is significantly increased; (7) mandible is positioned backward and downward due to the posterior position of the maxilla and the elongation of LPFH and LAFH; and (8) skeletal profile is more convex and is getting straight and finally is flatter over time in the clefts than in the controls. Generally, the manifestation of the cleft characteristics of the Miami group is increased when compared to that of the Eastman cleft group. The skeletal growth leads to not only the maxillary retrusion but also to position the mandible down and back. Early orthopedic intervention followed by the fixed edgewise appliance and prolonged retention is recommended to try to correct the skeletal problems, camouflage by dental correction, and maintain to the treatment outcome with reasonable retainer.
In the foreword of the multicenter study, Treatment Variables Affecting Facial Growth in Complete Unilateral Cleft Lip and Palate, Bruce Ross discussed the difficulty of performing this type of study due to the variability in sample size, age, sex, precise cleft type, and ethnic origin. He then mentioned the problems associated with doing cephalometric measurements and suggested using one center to control measurement errors; this was an excellent solution. According to Ross (1987a, b, c