Quintuplets with Clefts: Follow-up at 5 Years. Y erucham ZirsermaAn, D.M.D. Icana Brin, D.M.D. Dan Mancer, M.D.

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1 Quintuplets with Clefts: Follow-up at 5 Years Y erucham ZirsermaAn, D.M.D. Icana Brin, D.M.D. Dan Mancer, M.D. Follow-up observations are presented of a set of quintuplets at 5 years of age. Three of the children were born with different degrees of oral clefting. Height and weight comparisons between siblings with clefts and those without reveal that the most severely affected children lagged in their height measurements at the age of 5 years. This may indicate need for special awareness when caring for such children. A relative constriction of the anterior segment of the maxillary dental arch in two of the affected siblings may reflect a possible effect from previous surgical intervention. In a previous report, a set of quintuplets, three of whom (one boy and two girls) presented various degrees of clefting, was described (Zilberman et al, 1980). Boy C (Fig. 1) had a bilateral cleft lip, alveolus, and palate. Girl D had a bilateral cleft of the lip and alveolar ridge with an intact palate. Girl E had a left-sided cleft of the lip and alveolar process, with an intact palate. The possible etiologic factors that could account for observed phenomena were discussed. The purpose of this report is to present a follow-up of the quintuplets at the age of 5 years. This age is a milestone in facial and dental growth and development and in the planning of treatment goals (Handelman and Pruzansky, 1968). At this stage, maxillary dental arch form and occlusion may show effects either from earlier surgery or environmental influences but are less subjected to multiple factors that contribute to the development of malocclusions at later ages (Narula and Ross, 1970; Drs. Zilberman and Brin are associated with the Department of Orthodontics at the Hebrew University-Hadassah School of Dental Medicine, founded by the Alpha Omega Fraternity in Jerusalem, Israel. Dr. Zilberman is Senior Lecturer and Dr. Brin is a Lecturer. Dr. Mahler is Head of the Department of Plastic Surgery at Soroka University Hospital and Professor for Plastic Surgery at the Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer- Sheva, Israel. 205 Harding and Mazaheri, 1972). The follow-up of the quintuplets may serve as a biological model for comparison of siblings with and without clefts. PROGRESS REPORT The quintuplets were examined at a 5- year recall appointment at which the following records were obtained: (a) details pertaining to individual development (obtained from the pediatrician), (b) records of height and weight, (c) intraoral examinations, (d) facial and intraoral photographs, (e) study models, ( ) panoramic and selected periapical x-ray films. Cephalometric roentgenographs were not obtained. INDIVIDUAL DEVELOPMENT The children with clefts underwent lip surgery at the age of 3 months. Palatal closure at the age of 18 months was also necessary for boy C (Figs. 1 and 2). All of the quintuplets exhibit physical development within the normal range and attend a regular preschool kindergarten. There are no other congenital malformations except for the clefting. Girl D (Fig. 1) has a mild palsy of the right hand and leg and receives physiotherapy. Her previous motor development was somewhat delayed: sitting at 16 months, standing at

2 206 Cleft Palate Journal, July 1985, Vol. 22 No. 3 FIGURE 1. Full-face and profile views of the quintuplets

3 Zilberman, Brin and Mahler, QUINTUPLETS with CLEFTS 207 ul &. tn... 10k Hs..... FIGURE 2. Frontal view of the nasal area and the maxillary arches of the siblings with clefts 20 months, and walking at 24 months. Boy C and girl D are receiving speech therapy, but this has not been necessary for the other child with a cleft. Height and weight measures and percentiles (Jackson and Kelly, 1945) of the quintuplets at the ages of 10 weeks, 1, 2, and 5 years are presented in Table 1. From these data it is evident that all were born below the tenth percentile. As expected, after birth they caught up in their growth, and by the age of 2 years only boy C (for height) and girl D (both in height and weight) remained under or at the 10th percentile; the others reached at least the 50th percentile. By the age of 5 years, again, only these two siblings exhibited delayed growth and remained under the 50th percentile. FACIAL APPRAISAL The en-face views ofall quintuplets are normal with the exception of the nasolabial area in the affected siblings, which show the typical nasal deformity (Figs. 1 and 2, upper row). Boy A has a dolichocephalic tendency, while boy C and girl D have a brachyfacial appearance when compared to their siblings. In the profile, boy B shows a slightly protruding maxilla which is attributed to his thumb-sucking habit. In boy C and girl D, the nasolabial area is distorted because TABLE 1. Height and Weight (Actual Measurements and Percentiles*) of the Quintuplets, at Different Ages A B C D E Age Ht wt Ht wt Ht wt Ht wt Ht ( 10 weeks $ $900 <10 <10 <10 <10 <10 <10 <10 <30 <10 <10 1 year years < years <90 <10 25 <90 <90 *Height is given in centimeters, weight in grams; the percentiles are from Jackson and Kelly's (1945) growth charts.

4 208 Cleft Palate Journal, July 1985, Vol. 22 No. 3 of the lip cleft condition. This will be corrected later with further lip and nose cosmetic surgery. INTRAORAL EXAMINATION All of the quintuplets exhibit a deciduous dentition (Fig. 3, upper row), and present a low incidence of caries. All deciduous teeth show normal morphology, except for girl D who has an "X-type" (Bohn, 1963) maxillary deciduous incisor (Fig. 2, D, lower row). In the cleft region of siblings C and D, some deciduous incisors are absent. In each of the children with clefts, the width of the palate seems adequate (Fig. 2, lower row), but mild crossbites of deciduous canines appear in two instances (Fig. 3, C and D). Girl D, who was born with a protrusive premaxilla, still exhibits a gap between her premaxilla and palate (Fig. 2, D, bottom row) with minimal overet. The interarch relations at age 5 years are of Angle's class II type, except for girl E who shows normal anteroposterior relations. In addition, four of the children show a tendency toward deep anterior overbite. In girl D, this was less noticeable because of heavy wear on the upper incisors. Boy B has a persistant thumb-sucking habit with an accompanying anterior open bite, increased overjet, and spacing of maxillary anterior teeth (Fig. 3). ROENTGENOGRAPHIC EVALUATION The radiographic records obtained at age 5 years included panoramic x-rays (not shown) and maxillary occlusal views (Fig. 3, lower row). Boy A. The development of the dentition is within the normal range with all deciduous teeth and permanent tooth buds present. The root of the maxillary right deciduous central incisor shows resorption which possibly indicates previous trauma to that area. Boy B. This boy has a full complement of deciduous and permanent teeth but shows a delayed stage of development as compared with boy A. The maxillary per- manent right central incisor tooth bud is severely rotated. Boy C. A delayed stage of dental development is exhibited by boy C when compared to boy A. He has a full complement of deciduous and permanent teeth, except at the premaxillary area where one deciduous incisor is present and one permanent incisor is absent. Girl D. This sibling demonstrates a more delayed stage of dental development and is the only one in whom there are no radiographic signs of any development of the mandibular second premolar. She is missing one deciduous and one permanent incisor in the maxillary arch. Girl E. The most delayed dental development is presented in this sibling. She is the only one in whom there are congenitally missing and supernumerary deciduous and permanent teeth in the cleft area. STtUpy MoDELS Table 2 presents arch measurements, taken from study models obtained at 5 years of age, using the technique described by Moorrees (1959), with slight modification for clefted arches. The following were measured: 1. Arch length. For the maxilla, arch length was measured from the most anterior point on the midline (determined by the continuation of the median raphe) to the distogingival aspect of the second deciduous molar. For the mandible, the midpoint of the central incisors served as the anterior measurement. 2. Arch width. Arch width was measured between the deciduous canines and second deciduous molars in both arches. Intercanine width was measured between the middle of the linguogingival aspects. The intermolar width was measured between the gingival aspects of the mesiopalatal and mesiolingual cusps. The arch measurements of the quintuplets are somewhat similar and generally within the normal range, with some exceptions: Boy A's mandible is probably affected by his deep anterior overbite; thus,

5 Zilberman, Brin and Mahler, QUINTUPLETS WITH CLEFTS 209 FIGURE 3. Anterior dental and the occlusal radiographic views of the premaxillary segments of the quintuplets TABLE 2. Dental Arch Measurements of Left and Right Arches of the Quintuplets (in mm) Arch A B C D E Norms + S.D.* Dimension Ro- L Ro L Ro L Ro L Ro- L Boys Girls Maxillary Arch Arch length Arch widtht Arch width$ Mandibular Arch Arch length *1.75 Arch widtht Arch width$ *Adapted from the diagrams of Moorrees (1959) tarch width at 2nd deciduous molars #Arch width at deciduous canines Combined norms for boys and girls

6 210 Cleft Palate Journal, July 1985, Vol. 22 No. 3 the mandibular intercanine width is decreased." He is the only sibling with a high palatal vault, which is in accordance with his dolichocephalic tendency. Boy B has the largest maxillary arch length, probably because of his thumb sucking habit. Boy C's right and left maxillary arch lengths are unequal because of rotation of the clefted premaxillary segment. The actual measurements for this boy are similar to siblings A, B, and D. Girl D's maxillary intercanine width is decreased and a bilateral crossbite is evident. Girl E's arch width measurements are somewhat wide, which is in accordance with her brachycephalic facial appearance. DIscUssION Evaluation of the growth data of the quintuplets suggests a persistant lag in the development of the more severely affected siblings. This lag may be attributed to their physical disability. In the literature there are opposing views on the possible relationship of clefting to birth weight and height. Although Jensen et al (1983) found that Danish boys with cleft palate were shorter in height than a control group, Ranalli and Mazaheri (1975) found that the severity of clefting did not influence height and weight in these subjects. Hunter and Dijkman (1977) maintain that the effects of clefting on growth are astonishingly few and that individuals with cleft eventually catch up with normal growth. It is interesting to note that the children with clefts in this report were heavier at time of birth compared to their healthy siblings. The birth weights of the infants with clefts were: boy C, 2020 g; girl D, 1850 g; and girl E, 1820 g; the weights of the two normal brothers were: boy A, 1670 g; and boy B, 1580 g (Zilberman et al 1980). This contrasts with the generally expected findings (reported by Spriestersbach et al, 1973) that babies with clefts tend to have a lower birth weight. The fact that the affected siblings in this report were heavier at birth, but later lagged in height and weight development, requires clarification. It could be hypothesized that the more severe the clefting, the greater the childcare requirements for normal physical development. In the present case, the children with severe clefts might not have received the typical amount of attention and stimulation because they were part of quintuplets. This might have had a psychological impact which influenced growth and development. The physical development of the quintuplets at the age of 5 years shows that the more affected siblings still lag in development. It may be assumed that they will continue to catch up toward normal development as was found by Schenker et al (1981). It would be interesting to follow boy C and girl D, especially in their physical growth, in view of the expected improvements in oral function which will be rendered by subsequent therapy. The appraisal of the facial pattern is based on clinical and photographic evaluation, since the children were considered to be too young to undergo cephalometric radiography using the available cephalostat. Subjective evaluations indicated that the boys presented a mild anteroposterior discrepancy between maxilla and mandible, probably because of mandibular undergrowth (mild skeletal class II). Girl D showed a similar, but less pronounced, skeletal pattern; only girl E showed normal anteroposterior jaw relations. However, because of their present age, it 1s also possible that the early mandibular growth spurt has not yet occurred (Nanda, 1955) and that only girl E, who seems to be advanced in her physmal development (height and weight), might have experienced this stage. It is worthwhile noting, however, that girl E is actually the most delayed in dental development. Such a negative correlation between skeletal and dental development sometimes exists, as was reported by Krogman (1968). The measurements of the dental casts of these children reveal some possible effects on the dimensions of the maxillary and mandibular dental arches resulting from either tissue deficiency in the cleft area or early plastic surgery involvement. The most reveahng measurement in this regard is the width of the upper dental arch. The intersibling comparison of this -

7 Zilberman, Brin and Mahler, QUINTUPLETS WITH CLEFTS 211 dimension (Table 2) indicates reduced anterior maxillary width in girl D. Boy C presents anterior maxillary width similar to his brothers. However, taking into consideration his brachycephalic tendency and lack of thumb-sucking habit, this dimension was expected to be wider. This relative constriction in girl D and possibly in boy C, who were born with clefts extending beyond the lip and alveolar process, suggests a plausible constricting effect resulting from the plastic surgery performed. Similar findings of maxillary constriction with crossbite limited to deciduous canines were also reported by Handelman and Pruzansky (1968). The crossbite of the primary canines in these siblings is probably the outcome of this constriction in the intermaxillary relationship. Mandibular arch width measurements (Table 2) are similar to the normative data adapted from Moorrees (1959) and the relatively normal lower arch findings in bilateral clefts reported by Handelman and Pruzansky (1968). Lip correction may affect the anteroposterior position of the anterior maxillary dental segment, as was suggested by Ponitz and Spyropoulos (1979). This is evident in siblings C and D, each of whom was born with a severely protruding premaxillary segment, but at 5 years of age show reduced overjet because of tilting of this segment following lip surgery. Acknowledgement. The authors wish to acknowledge Professor Robert Brent and thank him for his constructive criticism of the manuscript. REFERENCES BoOHN A. Dental anomalies in harelip and cleft palate. Acta Odontolog Scand 1963; 21, Supp 38:1. HANDELMAN CS, PRUZANsky S. Occlusion and dental profile with complete bilateral cleft lip and palate. Angle Orthodont 1968; 38:185. HARDING RL, MAzAHERI M. Growth and spatial changes in the arch form in bilateral cleft lip and palate patients. Plast Reconstr Surg 1972; 50:591. HUNTER WS, DIjKmaNDJ. The timing of height and weight deficits in twins discordant for cleft of the lip and/or palate. Cleft Palate J 1977; 14:158. JACKSON RL, KELLY HG. Growth charts for use in pediatric practice. J Pediat 1945; 27:215. JENsEN BL, DAHL E, KREIBORG S. Longitudinal study of body height, radius length and skeletal maturity in Danish boys with cleft lip and palate. Scand J Dent Res 1983; 91:473. KroGmMAN WM. Biological timing and the dento-facial complex. J Dent Child 1968; 35:328. MoOORREES CFA. The Dentition of the Growing Child. Cambridge: Harvard University Press, 1959:87. NaAnDaA RS. Rates of growth of several facial components measured from serial cephalometric roentgenograms. Am J Orthodont 1955; 41:658. NARULA JK, Ross RB. Facial growth in children with complete bilateral cleft lip and palate. Cleft Palate J 1970; 7:239. PoNITZ PV, SPyROPOULOS MN. The dental occlusion in treated and nontreated cleft lip and palate. Eur J Orthodont 1979; 1:181. RANALLI DN, MAZzAHERI M. Height-weight growth of cleft children, birth to six years. Cleft Palate J 1975; 12:400. SCHENKER JG, YARKONI S, GRANAT M. Multiple pregnancies following induction of ovulation. Fertil Steril 1981; 35:105. SPRIESTERSBACH DC, DIcCKson DR, FRASER FC, ET AL. Clinical research in cleft lip and cleft palate: The state ofthe art. Cleft Palate J 1973; 10:113. ZILBERMAN Y, RoBInsoNn M, WEINSTEIN D. Clefts in quintuplets-a case report. Cleft Palate J 1980; 17:58.