growth of the cranial vault, the maxilla, and the mid- dle face and the increase in length and height of the mandible (Fig. 8.1b). Gradual improvement in the fa- cial profile was recorded by the changes in the facial angle and in the angle of convexity.The facial angle is a measure of the degree of protrusion or recession of the chin. In this instance, the facial angle increased from 64°, at 3 months of age, to 70°, at the age of 3 1/2 years,indicating a reduction by 6° in the recessive- ness of the chin. While the mandible is still in a retru- sive relation to the rest of the face, the potential for further improvement with continued growth still ex- ists. The changes in the angle of convexity were more interesting. This measurement relates the maxilla to the total facial profile.At 3 months of age,the angle of convexity was 140°, and at 31/2years it measured at 154°. The integrated growth of the several areas of the face was such as to improve the overall configuration of the facial profile. Serial photographs at 2 months,at 13 months, and at 3 years of age further testify to the changes in this child’s face. In the last photograph,the patient is posed beside her older sibling. The changes in the position of the hyoid bone are of particular interest, insofar as they reflect a change in the relative position of the tongue. The tongue is composed of several individual muscles originating from the base of the skull, the mandible, the hyoid bone,and the walls of the pharynx.Changes in the po- sition of any of its bony or fibrous attachments would Chapter 8 Pierre Robin Sequence 217 Fig. 8.2. Pierre Robin sequence. A series of tracings of the lat- eral film from 3months,1 day of age (0-3-1) to 3 years,5months, 12 days (3-5-12). The pharyngeal airway is filled in (black), and the stippled area denotes the border of the tongue.A tracheoto- my tube is visible in the first two films. Soon after birth (3 and 4 months of age) the dorsum of the tongue is visible at a level above the palatal plane within the palatal cleft space. Posterior- ly, the tongue just above the epiglottis impinged on the airway. At this level, the airway was almost completely occluded. Com- parison with the remaining three figures, ages 0-8-10, 1-1-0 and 3-5-12, reveals the configuration of these structures under nor- mal circumstances after closure of the cleft. The airway in its posteroanterior dimensions is fairly wide, and the tongue occu- pies a more protrusive relationship to the mandible.(Reprinted with permission from [1]) 3-5-12 3-5-12 3-5-12 3-5-12 3-5-12 tend to reflect on the position of the tongue. Con- versely,changes in the posture of the tongue would re- flect on the spatial relations of the mandible and hy- oid bone.Therefore,to study the position of the hyoid bone is, in a sense,to study the position of the tongue. With growth there occurs a forward and downward migration of the hyoid bone from the base of the skull. The pattern of changes in the posture of the hyoid bone observed in this patient sheds further light on the favorable adjustments consequent to growth. Dur- ing the first 5months of our studies, the hyoid bone migrated downward and forward. This resulted in an increase in the angle S-N-H. But, from 8months on- ward, this angle became fairly stable and the hyoid bone began to descend principally in a downward di- rection. Comment: This case was selected to typify the find- ings in several similar cases, one of which has been followed to the age of 7 years. Not all cases of Pierre Robin syndrome present such acute histories. When clinical evaluation suggests that there will be no im- provement or that possibly death may ensue, tra- cheotomy should be undertaken without hesitation to prevent further aggravation of the symptoms.Once an adequate respiratory exchange was made possible, improvement in oxygenation and feeding followed.In such instances, we have recorded rapid growth and favorable changes in the facial appearance. 8.1.2 Case 2 J.G,a white girl,was referred to the outpatient clinic of the Cleft Palate Center at the age of 2 months with a diagnosis of cleft palate and mandibular micro- gnathia. Following an uneventful pregnancy, the de- livery was normal and at full term. The birth weight was 6 lb. 11oz. (3,030gm). The infant had some diffi- culty in breathing, but this was relieved by placing her in a prone position. Tube feeding was employed for the first few days after which she was given bottle feedings. At 6days of age, the infant was discharged from the hospital. There was no family history of cleft palate. The mother suffered no illness during her pregnancy. Oral examination revealed an unusually small tongue closely attached to the floor of the mouth. In the course of our first examination under sedation, the infant became cyanotic and failed to initiate mandibular movements sufficient to permit the pas- sage of air. This was relieved immediately by main- taining forward traction on the tongue and mandible. After about 5min, the infant recovered control of mandibular movements, and respiration normally. Aside from this isolated episode, which occurred un- der sedation, the parents did not report any similar difficulties. The child has continued to grow and de- velop at a satisfactory rate. Growth Studies: The casts disclose symmetrical cleft of the hard and soft palate,extending distally from the region of the nasopalatine foramen. Additional casts obtained at regular intervals revealed that the cleft had narrowed, so that it now presents a narrow V-shaped defect (Figs. 8.3, 8.4). The earliest lateral head palate, at 2months 10 days of age, displayed a small mandible and small tongue. The latter was positioned high and above the floor of the nose, but relatively remote from the posterior wall of the pharynx. The airway appeared sufficient to sus- tain respiration without any undue effort on the part of the infant. Progressive growth changes recorded up to the age of 3 years, 4months, 13days disclosed mandibular growth and generalized growth in all areas of the face and cranial vault.Mandibular growth was continuous and progressively downward and for- ward. During the period studied, from 2 to 40 months of age, the facial angle increased from 61.5, becoming more obtuse. The angle of convexity increased from 147° to 155°. Altogether, the changes were in a direc- tion tending to minimize the recessiveness of the chin in relation to the rest of the face. Comment: Micrognathia by itself is not sufficient to produce glossoptosis and respiratory embarrassment. If the tongue is large or even normal in size, the small recessive mandible will tend to displace the tongue distally and superiorly. It is this displacement that produces the respiratory obstruction both into the hypopharynx and into the posterior choanae. On the other hand, if the tongue is small, there will be no obstruction of the airway even in the presence of a micrognathic mandible. In this instance, the simulta- neous occurrence of micrognathia and microglossia averted the respiratory difficulties commonly experi- enced in such instances. The tendency to lose reflex control of the muscles of respiration and deglutition under anesthesia or se- dation renders such procedures unusually hazardous in these patients because of the limited reserve. It is, therefore, important that such procedures be under- taken with full knowledge and anticipation of possi- ble respiratory obstruction, in order that adequate emergency provisions for the establishment of an air- way be available. 218 S. Pruzansky · J. B. Richmond 8.1.3 Case 3 E.C.,a white boy aged 5weeks,was referred to the out- patient clinic of the Cleft Palate Center for longitudi- nal growth studies. The delivery had been normal and at full term. His birth weight was 7lb., 8oz. (3,400 gm.). There was no history of cleft on either side of the family. No difficulty in breathing was en- countered, and the infant was discharged from the hospital on the sixth day.After a brief adjustment pe- riod, the infant was readily fed by a combination of a hard nipple and by means of a premature baby bottle nipple. Some snoring sounds were heard, especially as the infant was placed on its back and the head elevated with slight ventroflexion on the chest. The infant pre- ferred to sleep on either side, and in these positions the snoring sounds were at a minimum. This baby Chapter 8 Pierre Robin Sequence 219 Fig. 8.3 a, b. Palatal growth changes in a child with a Pierre Robin sequence.This sequence is characterized by glossoptosis, micrognathia, and isolated cleft palate. In many cases the cleft plate which is initially wide at birth can spontaneously narrow with palatal growth. a Computer-generated tracings of the iso- lated cleft of the hard palate from 2 months and 10days (0-2-10) to 6years, 1month, and 1day (6-1-1). The palate was closed at 4years,2months. b Superimposed tracings of each cast [on the baseline created by connecting postgingivale points (the poste- rior limits of the hard palate) and registered at the bisector of the line] show that the length of the cleft increases with palatal growth and narrows due to spontaneous growth at the medial border of the palatal processes.Obturators which interfere with tongue posturing within a relatively small intraoral space are contraindicated [16] a b showed progressive improvement, and at the age of 5 months he weighed 15 lb., 8 oz. (7,030gm.) Growth Studies: Two sets of records are available in this case. The first was obtained at the age of 1 month 7 days, and the second at 3 months 25days of age. The first cast of maxilla revealed a wide parabolic cleft extending distally from the nasopalatine fora- men. The widest portion of the cleft, at the level of the maxillary tuberosities, measured 16mm. Although the second cast exhibited an increase in the length and width of the palate, there was a decrease of 1.5 mm in the width of the cleft at its widest portion. During the first examination, the tongue was observed to occupy at rest the opening into the nasal chambers provided by the cleft in the palate. This was further confirmed by examining the frontal and lateral views of the head plates. The second series of films indicated that the tongue was now postured in a more inferior position and no longer occupied the nasal cavity to the same extent previously noted. This new position of the tongue could be explained by the downward and for- ward growth of the mandible that had occurred in the interim. In the first lateral film,the recessive chin,the distal- ly and superiorly malposed tongue, and relatively restricted airway were clearly observed. Two and one-half months later, considerable growth in the mandible had occurred to improve the facial profile, alter the posture of the tongue, and increase the an- teroposterior diameter of the airway. The tongue was no longer in close apposition to the posterior pharyn- geal wall, and its superior margin did not extend into the nasal cavity to the degree previously observed.Co- incidentally, the mother reported a diminution of the stertorous breathing that had been present. Superimposition of the tracings of the bony struc- tures revealed the rapid growth characteristic of this early period in life. In 2 1/2 months, that cranial vault and all parts of the face exhibited proportionate in- creases.Particularly encouraging was the amount and direction of growth displayed by the lower jaw. Mandibular growth was responsible not only for re- ducing the glossoptosis and increasing the airway, but for the improvement in the appearance of this baby’s face. Comment: The problem presented by this baby was unique and different from the two previous cases of the partial obstruction of the airway. Diligent nursing care to determine the most comfortable position for breathing and feeding may be sufficient to tide such cases through their critical period. In some instances the prone positioning and orthostatic feeding sug- gested in the literature are most successful.Again,one is impressed by the remarkable potential for prolific growth during this period of life; a potential that is shared by the small mandible. It follows then, that every effort must be made to permit the realization of the baby’s potential for growth by providing an ade- quate airway, which, in turn, facilitates feeding. The clinical course to be followed is varied and depends on 220 S. Pruzansky · J. B. Richmond Fig. 8.4. A series of cast of the maxillary arch from 2months 10days of age to 6 years 1 month. Note the progressive narrowing in the lateral dimension of the cleft. (Reprinted with permission from [1]) the severity of the symptoms and principally upon the degree of obstruction of the airway. 8.2 Comment The representative sampling of cases presented pro- vides an answer to the questions which the study was designed to solve.It is observed from the data present- ed that the mandible possesses remarkable potential- ities for growth in patients with the Pierre Robin syn- drome. Thus, all efforts should be directed toward sustaining life in a metabolically favorable climate in order that a more physiologic airway may be estab- lished as growth proceeds. With growth, the glossop- tosis is minimized and spontaneous resolution of the respiratory and feeding problems occurs. It is our opinion that tracheotomy should be resorted to promptly if respiratory embarrassment is significant, in order to achieve a sufficient airway to provide ade- quate oxygenation. This is undoubtedly a life-saving procedure in some patients. On the basis of our longitudinal growth studies, certain prognostications concerning the future growth of the micrognathic mandible are permissible. In most instances, the increment in mandibular growth, as related to total facial growth,is sufficient to overcome the extreme recessiveness of the chin that is observed at birth. Since mandibular growth continues until late adolescence, it is possible to hope for an es- thetically pleasing profile in adulthood The manage- ment of the cleft palate has been in keeping with the established criteria for the treatment of palatal de- fects. The lateral cephalometric film served as a valuable diagnostic tool in estimating the degree of obstruc- tion of the airway as a result of the glossoptosis.In our experience, there was a high positive correlation be- tween the degree of obstruction revealed in the x-ray film and the incidence and severity of the respiratory difficulties. When obstruction of the air passage was complete and the tongue was practically in contact with the posterior wall of the pharynx, tracheotomy was recommended as a life saving procedure. If the obstruction was incomplete,more conservative meas- ures were employed. Care was taken to ascertain the most comfortable postures for breathing and feeding for the individual case, and the nurse or parent was carefully instructed in the care of the infant. Appro- priate nipples were selected to minimize the energy expended by the infant in the feeding process. In the course of these studies, we were aware of an obvious objection to placing so much reliance on these roentgenograms. Since many of these films were obtained under mild sedation, was it not possible that the posture of the mandible or of the tongue might have been altered by the sedative? Secondly, the film depicted a static view of the airway and represented only two dimensions. Did this view properly reflect the kinetic ability of the infant to manipulate the tongue and jaw hence the consistent correlation be- tween the findings in our films and the clinical state? Moreover, when the films were repeated in the same infant without sedation,similar postures were record- ed for the structures under analysis. It was important that the postures of the head in relation to the neck be kept constant. Dorsiflexion or ventroflexion of the head varied to posture of the mandible and tongue and produced changes in the configuration of the air- way. To indicate alterations in the posture of the head to the neck, our tracings purposely included at least the first two cervical vertebrae. We recognize that few institutions possess cephalo- metric roentgenographic equipment. Therefore, we should like to point out that an ordinary lateral film obtained by carefully positioning the infant can pro- vide useful diagnostic data.To minimize enlargement, a target-object distance of at least 3ft. (90 cm) is rec- ommended. For the sake of definition and to further decrease enlargement, the object film distance should be kept at a minimum.Sjölin [15] has published inter- esting films to describe his experiences with a case of micrognathia. Although his films did not permit quantification of the growth changes, they were ade- quate for diagnostic purposes. A number of papers in the literature claim to “stim- ulate” the growth of the mandible by a variety of me- chanical devices or surgical procedures. For example, a special nursing bottle was designed to force the in- fant to protrude his jaw in order to obtain nourish- ment and,by this protrusion, to stimulate mandibular growth [4]. From our data,we would conclude that the nursing care enabled the infant to survive until mandibular growth was sufficient to provide a more adequate airway. In another report, continuous traction on the mandible was maintained by circumferential wiring around the symphysis. The authors claimed growth- stimulating properties for this procedure [10]. From the findings in our series, it would seem that mandi- bular growth probably occurred spontaneously and not because of the stimulus provided by surgical trac- tion. The important and prime objective in the care of these children is to provide an airway. If possible, this should be accomplished with a minimum of trauma. Secondly,the infant’s total needs should be assessed to provide optimal conditions for somatic growth. As the potential for growth is permitted to express itself, the chin grows downward and forward away from the base of the skull. With this pattern of growth, ade- quate space for the tongue is provided, the airway Chapter 8 Pierre Robin Sequence 221 enlarges, and there follows a spontaneous resolution of the symptoms. Also,there are progressive improve- ments in the facial appearance. There is another dimension to the abnormal pos- ture of the tongue, as observed in these patients, that merits discussion. Not only does the tongue block the pharyngeal processes and hence prevent their fusion. The high incidence of micrognathia in the population of clefts involving only the hard and soft palate lends support to this theory. Mandibular micrognathia is a physiological finding in early intrauterine life. If for some reason the micrognathia persists and fails to carry the tongue down and out of the nasal cavity, a cleft in the palate might result. In early postnatal life, the tongue acts to keep the cleft palatal processes apart. As the tongue descends with mandibular growth and no longer forcefully in- trudes itself into the nasal cavity,the palatal processes tend to approximate in the midline. Fusion of the palatal processes cannot occur, but the narrowing in the clefts is recorded fact. 8.3 Summary and Conclusions The development of the accurate techniques for cephalometric roentgenography of infants has made possible a longitudinal study of the growth of the micrognathic mandible. As a result of these studies, useful diagnostic and prognostic information has been obtained to provide a rationale for the manage- ment of individual cases. The lateral cephalometric roentgenogram is a valu- able diagnostic aid in assessing the severity of the glossoptosis and its obstruction of the airway. A defi- nite correlation exists between the degree of constric- tion of the airway and the severity of the clinical state. On the basis of these findings, it is possible to recom- mend either conservative management or tracheoto- my in extreme situations, or distraction osteogenesis. Three cases,out of a larger series of similar cases,were presented to indicate the spectrum of variations to be encountered. In all instances, it was found that where an ade- quate metabolic situation was provided and the infant gained weight,mandibular growth during the first few months was sufficient to provide for a natural resolu- tion of the symptoms attending the glossoptosis. Longitudinal records have indicated that mandibu- lar growth is proportionally adequate to reduce the retrognathic profile and provide an esthetically har- monious facial appearance. Based on investigations performed during the tenure of Special Research Fellowship from the Na- tional Institute of Dental Research Institutes of Health (Dr. Pruzansky, Senior Assistant Dental Surgeon [R], United States Public Health Service,National Institute of Dental Research, Department of Health, Education and Welfare). References 1. Pruzansky S, Richmond JB. Growth of mandible in infants with micrognathia.Am J Dis Child 1954; 88:29–42. 2. Robin, P. La glossoptose: un grave danger pour nos enfants. Paris: Gaston Doin; 1929. 3. Robin, P. Glossoptosis due to atresia and hypoplasia of the mandible.Am J Dis Child 1934; 48:541–547. 4. Eley RC, Farber S. Hypoplasia of the mandible (microg- nathy) as a cause of cyanotic attack in newly born infant: Report of 4 cases.Am J Dis Child 1930; 39:1167–1175. 5. Davis AD, Dunn R. Micrognathis: a surgical treatment for correction in early infancy. Am J Dis Child 1933; 45:799– 806. 6. Callister AC. Hypoplasia of the mandible (micrognathy) with cleft palate: treatment in early infancy with skeletal traction.Am J Dis Child 1937; 53:1057–1064. 7. Lleweyllyn JS,Biggs AD.Hypoplasia of the mandible: report of case, with resume of literature and suggestions for mod- ified form treatment.Am J Dis Child 1943; 65:440. 8. Douglas B. The treatment of micrognathia associated with obstruction by plastic procedure. Plast Reconstruct Surg 1946; 1:300. 9. Nisenson A.Receding chin and glossoptosis: cause of respi- ratory difficulty in infant. J Pediat 1948; 32:397–401. 10. Longmire WP Jr, Sandford MC. Stimulation of mandibular growth in congenital micrognathia by traction. Am J Dis Child 1949; 78:750–755. 11. May H, Chun LT. Congenial ankyloglossia (tongue-tie) as- sociated with glossoptosis ( retruded mandible) and pala- tum fissum (clift palate). Pediatrics 1948; 2:685–687. 12. Brodie AG. On the growth pattern of the human head from the 3rd month to the 8th year of life. Am J Anat 1941; 68: 209–262. 13. Brodie AG.Behavior of normal and abnormal facial growth patterns.Am J Orthod 1941; 27:633–655. 14. Pruzansky S. Description, classification and analysis of un- operated clefts of the lip and palate. 1953; Am J Orthod 39:590. 15. Sjolin S.Hypoplasia of the mandible as a cause of respirato- ry difficulties in the infant. Acta Paediat 1950; 39:255– 261. 16. Berkowitz S. Cleft lip and palate-perspectives in manage- ment. 1st ed. Little, Brown; 1996. 222 S. Pruzansky · J. B. Richmond 9.1 Introduction Congenital clefts of the lip and/or palate can arise in isolation or together with other malformations (syn- dromes) [28]. This chapter deals solely with “nonsyn- dromic” clefts. Both individuals with unoperated and operated clefts have a face which differs from those of unaffect- ed individuals. Since the introduction of roentgen- cephalometry more than 70years ago [8] hundreds of cephalometric studies, including both unoperated and operated cleft individuals have suggested that some deviations are directly caused by the primary anomaly,while others are caused by the surgical inter- ventions and the following dysplastic and compensa- tory growth of the facial bones [e.g., 1, 3, 4–10, 12, 15, 18–27,29, 30,48,50,51, 53–55,57,59–61].However, the relative importance of the intrinsic factors, the iatro- genic factors, and the functional or adaptive factors for the facial development is still unclear. There are probably several reasons for this. Firstly, comprehen- sive knowledge of craniofacial morphogenesis in cleft newborns or infants before surgery, based on large, consecutive, well-controlled samples, is very scarce. This situation is not surprising since, in developed countries, the cleft of the lip is surgically treated with- in the first couple of months after birth. Thus,the pos- sible period of examining the unoperated state is short and several methodological problems are in- volved.Secondly, the cephalometric analyses are most often limited to the lateral projection using simplistic cephalometric analyses, typically based on 15–20 reference points, and almost invariably measuring maxillary prognathism as the S-N-A angle or similar measurements to the premaxilla, and the use of infant cephalometry has been very limited. These authors are of the opinion that incomplete knowledge about the intrinsic factors related to the cleft anomaly has automatically lead to excessive emphasis on the im- portance of iatrogenic and adaptive factors in facial development of cleft children. 9.2 The Danish Experience In the middle of the 1970s we decided to take advan- tage of the very favorable sampling conditions in Den- mark in an effort to contribute to the question of the characteristics of facial growth and development in children born with clefts [42]. In Denmark, for more than 65 years,all newborns with facial clefts have been recorded at the Institutes for Speech Disorders in Copenhagen and Århus.Repeated follow-up examina- tions have shown that the registration of clefts in Denmark is highly reliable and nearly complete. The population is homogeneous and stable, and only very few children are lost to follow-up. Furthermore, all primary cleft surgery is performed in one hospital by one surgeon. Inspired by [52] we constructed a three-projection infant cephalometer, which can obtain truly orthogo- nal lateral, frontal, and axial cephalograms [43]. A comprehensive cephalometric analysis system was developed including all craniofacial regions (calvaria, cranial base,orbits,maxilla,mandible,airway,cervical spine, and soft-tissue profile) [44, 32, 36], and the method was validated [36]. Furthermore, new meth- ods of visualization of differences in craniofacial mor- phology and growth between different groups were developed using mean plots [36, 44], color-coded vectorgrams [36], and color-coded surfaces on a 3D CT-model [16]. During the 6 years from 1976 to 1981, there were 359,027 live births in Denmark. A total of 678 new- borns of Northern European ancestry with cleft lip, cleft palate, or both were registered in the period. Twenty-four infants died before 22 months of age,and for practical reasons material uptake had to be omit- ted in some patients with isolated cleft palate. Only nonsyndromic clefts were included in the study, but 602 of the 678 children (about 90%) were examined by us [42] and nearly all at both 2 months of age (before any surgical or orthopedic treatment) and at 22months of age (before closure of the posterior Characteristics of Facial Morphology and Growth in Infants with Clefts Sven Kreiborg, Nuno V. Hermann, Tron A. Darvann 9 palate in the children with clefts of the secondary palate).All children were treated by the same surgeon (Dr. Poul Fogh-Andersen), and in the children with cleft of the primary palate the cleft lip was, in all cas- es, closed using a Tennison procedure. One third of the children had isolated cleft lip (CL), about 40% had combined cleft lip and palate (CLP), and about 27% had isolated cleft palate (CP).The clefts were subclas- sified according to the method of Jensen et al. [42]. 226 S. Kreiborg · N.V. Hermann · T.A. Darvann Table 9.1. Summary and comparison of the most important findings in the primary anomaly in children with RS, ICP, BCCLP, and UCCLP* Anomaly RS ICP BCCLP UCCLP Maxilla Decreased length measured to premaxilla (sp-pm) a + 1 +– 2 – Retrognathia measured to premaxilla (s-n-ss) b ++ – 3 – Decreased posterior length (ci-pm) c ++ ++ Retrognathia measured to base of jaw (s-n-ci) d ++ ++ Decreased posterior height + + + + Increased width + + + + + + Nasal cavity Increased width + + + + + + Mandible Decreased length + + + + + + + + Retrognathia + + + + + + + + Pharyngeal airway Reduced size + + + + + + + * RS, Robin Sequence; ICP, Isolated Cleft Palate; BCCLP, Bilateral Complete Cleft Lip and Palate; UCCLP; Unilateral Complete Cleft Lip and Palate. 1 The deviation from the norm is shown as + or –, meaning, e.g., that decreased total length of the maxilla was observed in the ICP and RS groups but not in the UCCLP group, and that the length of the mandible is decreased in the UCCLP group, very decreased in the ICP and BCCLP groups, and severely decreased in the RS group. 2 The total length was significantly increased. 3 The prognathism was increased measured to the premaxilla. a sp-pm: Anterior nasal spine to point pterygomaxillare. b s-n-ss: S-N-A. c ci-pm: Point crista infrazygomatica to point pterygomaxillare. d s-n-ci: Maxillary prognathism measured to the infrazygomatic crest. Fig. 9.1. a The facial morphology in a 2-month-old unoperated infant with unilateral complete cleft lip and palate (UCCLP). b The facial morphology in a 2-month-old unoperated infant with bilateral complete cleft lip and palate (BCCLP) a b In the 602 children included in the study, cephalo- grams were obtained in the lateral, frontal, and axial projections by three experienced orthodontists (Dr. Birgit Leth Jensen, Dr. Erik Dahl, and Dr. Sven Krei- borg). In addition, impressions were made of the maxilla, and anthropometric registrations (body height, body length, and head circumference) were carried out. The results of the cephalometric analyses have been presented in a number of publications [13, 14, 17, 33–41, 45–47]. So far, we have analyzed infant craniofacial morphology and early craniofacial growth in detail in three dimensions in the following groups: incomplete unilateral cleft lip (UICL),isolated cleft palate (ICP), Robin sequence (RS), unilateral complete cleft lip and palate (UCCLP) (Fig. 9.1a), and bilateral complete cleft lip and palate (BCCLP) (Fig. 9.1b). In the following, we shall summarize our findings, with emphasis on the unoperated infant to shed light on the intrinsic factors related to the cleft condition (see Fig. 9.2 and Table 9.1), and compare them to data in the literature on unoperated adoles- cents and adults with clefts. 9.2.1 Cleft Lip (CL) Isolated CL involves only structures of the embryonic primary palate. The craniofacial morphology in CL subjects has been shown to be fairly normal except for the small region of the cleft including the premaxilla and the incisors. In unoperated bilateral complete CL the premaxilla may, however, protrude markedly. In unilateral, complete CL the protrusion is less pro- nounced but asymmetric. In subjects with unoperat- ed,unilateral,incomplete cleft lip UICL the protrusion of the premaxilla is negligible [33]. The interorbital distance in CL subjects seems to be slightly increased compared to the norm (11). The basal part of the maxilla has a normal prognathism in relation to the anterior cranial base, and the mandible is of normal size, shape, and inclination [12; 33]. Following lip sur- gery,the premaxilla is molded into a normal position, and maxillary prognathism measured to the point A or ss (subspinale) is normal [12,31, 33–35]. In conclu- sion, subjects with UICL have a very close to normal craniofacial morphology from infancy to adult age, and consequently, we have used our group of infants with UICL as a control group in the study of devia- tions in craniofacial morphology and growth of infants and young children with ICP, RS, UCCLP, and BCCLP,since no actual normative cephalometric data for Danish infants and young children are available. 9.2.2 Cleft Palate (CP) Isolated cleft palate (ICP) involves only structures of the embryonic secondary palate. In Fig. 9.2a, the mean facial diagrams of the ICP group is superim- posed on the mean facial diagram of a group of age- matched infants with UICL (control group). The ma- jor deviations in the ICP group were: reduced length and posterior height of the maxilla; maxillary retro- gnathia; increased width of the maxilla and the nasal cavity; and reduced length of the mandible with mandibular retrognathia. Thus, the ICP group re- vealed bimaxillary retrognathia. The sagittal jaw rela- tionship was, however, normal. In addition, in the ICP group the upper airway dimensions were reduced. Bimaxillary retrognathia and a short mandible were previously documented in unoperated older children [60] and adults with ICP [12, 2]. 9.2.3 Robin Sequence (RS) Robin sequence (RS) is defined as a triad of symp- toms: isolated cleft palate, micrognathia,and glossop- tosis [28]. RS may be part of several syndromes, e.g., Treacher-Collins syndrome [11, 46]. In this chapter, only nonsyndromic cases of RS will be discussed.We consider this group as a subgroup of the ICP group [39]. In Fig.9.2b, the mean facial diagram of the RS group at 2months of age is superimposed on the mean facial diagram of the control group. The major devia- tions in the RS group were: decreased length and pos- terior height of the maxilla; maxillary retrognathia; increased width of the maxilla and nasal cavity; very short mandible with marked mandibular retro- gnathia. Thus, the RS group revealed bimaxillary retrognathia; the retrognathia was, however, most marked for the mandible and the sagittal jaw relation was increased. In addition, the RS group had a signi- ficantly smaller cranial base angle (n-s-ba) resulting in a smaller depth of the bony nasopharynx than the controls, and the upper airway dimensions were markedly reduced. The degree of maxillary retro- gnathia was similar in the RS and the ICP group.How- ever, the mandibular retrognathia in the RS group was even more marked than in the ICP subjects. It would seem that RS subjects probably represent the extreme part of the ICP population in terms of mandibular retrognathia and upper airway constriction. As men- tioned above, we consider the RS group as a special subgroup of the ICP group. Accordingly, we believe the bimaxillary retrognathia to be intrinsically associ- ated with the cleft of the secondary palate. Chapter 9 Characteristics of Facial Morphology 227 228 S. Kreiborg · N.V. Hermann · T.A. Darvann d c b a [...]... morphology and growth in children with bilateral complete cleft lip and palate Cleft Palate Craniofac J 20 04; 41 :42 4 43 8 42 Jensen BL, Kreiborg S, Dahl E, Fogh-Andersen P Cleft lip and palate in Denmark 1976–1981 Epidemiology, variability, and early somatic development Cleft Palate J 1988; 25:1–12 43 Kreiborg S, Dahl E, Prydsø U A unit for infant roentgencephalometry Dentomaxillofac Radiol 1977; 6:29–33 44 ... 61.6±6 61.3±6 50.3±6 49 .2±5 SNB Mandible protrusion 82 4 83 .4 3 80±2 81±3 81.6±5 81.7±6 81.3±5 81.3 4 AR-TGO Ramus height 57.5 50.0 51.3 48 .9 48 .5 4 47.8 41 48 .6 47 .1 S_N_ANS Upper face height N-S-Ptm Upper post face height 54. 5 44 .0 51.5 40 .3 52.6 38.3 47 .5 37.0 46 .6±3 33.5 4 45.5±2 31.2±3 49 .2 39.7 45 .6 33 .4 Temperatures were always around 30 °C, and the humidity was often near 100%, but this did not... for the 2-monthold a ICP and UICL groups, b RS and UICL groups, c UCCLP and UICL groups, and d BCCLP and UICL groups 9.2 .4 Cleft Lip and Palate (CLP) Combined clefts of the lip, alveolus, and palate involve structures of both the embryonic primary palate and secondary palate In Fig 9.2c the mean craniofacial morphology in 2-month-old unoperated infants with unilateral complete cleft lip and palate (UCCLP)... vomero-premaxillary suture in children with bilateral cleft lip and palate Scand J Plast Reconstr Surg 1976; 10 :45 –55 23 Friede H Studies on facial morphology and growth in bilateral cleft lip and palate University of Göteborg, Göteborg; 1977 Thesis 24 Friede H The vomero-premaxillary suture – a neglected growth site in mid-facial development of unilateral cleft lip and palate patients Cleft Palate. .. unilateral cleft lip and palate Cleft Palate J 1990; 27: 146 –1 54 16 McCance A, Roberts-Harry D, Sherriff M, Mars M, Houston WJ Sri Lankan cleft lip and palate study model analysis: clefts of the secondary palate Cleft Palate Craniofac J 1993; 30:227–230 Chapter 10 17 Sell DA, Grunwell P Speech results following late palatal surgery in previously unoperated Sri Lankan adolescents with cleft palate Cleft Palate. .. patients with clefts of the lip and palate: Part 1 Principles and study design Cleft Palate J 1992; 29:393–397 36 Mars M, Asher-McDade C, Brattstrom V, Dahl E, McWilliam J, Molsted K, Plint DA, Prahl-Andersen B, Semb G, Shaw W, The R.P.S A six-centre international study of treatment outcome in patients with clefts of the lip and palate: Part 3 dental arch relationships Cleft Palate J 1992; 29 :40 5 40 8 37 Worrell... JC, Sosa-Martinez R Cephalometric comparisons of individuals from India and Mexico with unoperated cleft lip and palate Cleft Palate J 1986; 23:116–125 5 Boo-Chai K The unoperated adult bilateral cleft of the lip and palate Br J Plast Surg 1971; 24: 250–257 6 Dahl E Craniofacial morphology in congenital clefts of the lip and palate An x-ray cephalometric study of young adult males Acta Odontol Scand 1970;... significance of cleft palate in a Sri Lankan population Cleft Palate J 1990; 27:155–161 13 Lamabadusuriya SP, Mars M,Ward CM Sri Lankan Cleft Lip and Palate Project: a preliminary report J Royal Soc Med 1988; 81:705–709 14 Mars M, James DR, Lamabadusuriya SP The Sri Lankan Cleft Lip and Palate Project: the unoperated cleft lip and palate Cleft Palate J 1990; 27:3–6 15 McCance AM, Roberts-Harry D, Sherrif... in patients with unilateral complete cleft lip and palate Acta Chir Plast 19 94; 36:38 41 235 10 Facial Growth and Morphology in the Unoperated Cleft Lip and Palate Subject: The Sri Lanka Study Michael Mars Studies of subjects with unoperated clefts of the lip and palate have been undertaken for the past 80 years [1–29] Adults with unoperated clefts of the lip and palate provide the ideal control group... craniofacial morphology and growth in children with nonsyndromic Robin Sequence Cleft Palate Craniofac J 2003a; 40 :131– 143 40 Hermann NV, Kreiborg S, Darvann TA, Jensen BL, Dahl E, Bolund S Craniofacial morphology and growth comparisons in children with Robin Sequence, isolated cleft palate, and unilateral complete cleft lip and palate Cleft Palate Craniofac J 2003b; 40 :373–396 41 Hermann NV, Darvann . morphology and growth in children with bilateral complete cleft lip and palate. Cleft Palate Craniofac J 20 04; 41 :42 4 43 8. 42 . Jensen BL, Kreiborg S, Dahl E, Fogh-Andersen P. Cleft lip and palate. 49 .2±5 SNB Mandible protrusion 82 4 83 .4 3 80±2 81±3 81.6±5 81.7±6 81.3±5 81.3 4 AR-TGO Ramus height 57.5 50.0 51.3 48 .9 48 .5 4 47.8 41 48 .6 47 .1 S_N_ANS Upper face height 54. 5 51.5 52.6 47 .5 46 .6±3 45 .5±2. bilateral cleft lip and palate. Tand- laegebladet 1971; 75:1157–1169. 54. Sandham A, Foong K.The effect of cleft deformity, surgical repair and altered function in unilateral cleft lip and palate. Transactions