ORIGINAL ARTICLES
C hildren with cystic fibrosis have skeletal m aturation and epiphyseal anom alies sim ilar to those in children with nutritional problem s. H owever, these patients have been found to have norm al som atic developm ent if treatm en t is begun early. In a g roup of children w ith cystic fibrosis undergoing m edical trea tm en t, cep h alom etric m easurem ents com pared favorably w ith those established by norm al standards.
A cephalometric study of patients with cystic fibrosis Albert A. Reitman, DDS Richard D. Faber, DDS, MS, New Hyde Park, N Y *
Early detection and diagnosis of children with cystic fibrosis treated with modern therapeutic regimens have increased the survival rate in these patients well beyond childhood. Haung and others1 reported in 1970 that the survival rates have increased from 35% (1952 to 1957) to 76.6% (1962 to 1967). Shwachman and Kulcz2 reported that, with antibiotic therapy to treat the gastroin testinal and respiratory problems, life expectancy has been increased for most patients. This in crease is attributed to early diagnosis and current therapeutic regimens. Cystic fibrosis3 is consid ered to be a hereditary disorder of children and young adults, which has both primary and secon dary effects on the digestive and respiratory tracts from dysfunction of exocrine glands. These dis orders can have dramatic effects on the physical development of children.
Changes in physical development in patients with cystic fibrosis have been reported. The premise usually proposed for this development parallels that for children with genetic and pre natal influences, nutritional disturbances, and pulmonary disease. In 1964 Sproul and Huang4 reported on a study of 50 patients with cystic fibrosis in which height, weight, and bone age were evaluated during a two-year period; a signif icant physical somatic retardation was noted at all ages. The deficiency in height and weight was noticeable in preadolescent age groups with an absence of the growth spurt. Height changes and gain and loss of weight showed a direct corre spondence with respiratory conditions, especially bouts of pneumonia. Skeletal retardation was noted in 38% of the population studied; the most pronounced abnormality appeared in the preado JADA, Vol. 96, January 1978 ■ 83
le s c e n t g ro u p . In n on e o f th e age groups did th e m ed ian o f the m easu red d a ta in th e c y s tic fib ro s is group e x c e e d th e te n th p e rc e n tile o f th e n orm al group d ata. T h is stud y a lso sh ow ed th a t w ith the in stitu tio n o f th e ra p y th e re w as a c a tc h -u p p h e n o m en o n c o n c u rre n t w ith th e age and sta g e o f d ev elo p m en t at th e tim e th e ra p y w as in itia te d . It ca n b e co n clu d e d fro m th is stud y th a t in a n un tre a te d p o p u latio n it m ight b e e x p e c te d th a t so m a tic g row th w ould be n o tic e a b ly a ffe c te d . In a tre a te d p o p u latio n th e c h a n g e s m ay n ot b e as d ra m a tic. In a 1972 study by K re ib l and o t h e r s ,5 p h y sica l d ev elo p m en t o f 53 ch ild ren w ith c y s tic fib ro sis w as e v alu a te d fo r h eigh t and w eig h t, lin e a r and v e lo c ity ch a n g e s , and sk e le ta l a g e s. W eig h t and w eight v e lo c ity w ere fo u n d to b e d ire ctly c o r r e lated w ith th e se v e rity o f d is e a s e . N o sig n ifica n t re la tio n sh ip w a s found b etw ee n h eig h t and s k e le ta l age a s d eterm in ed from fig u res fro m an a tla s . T h e c o r re la tio n b e tw e e n s k ele ta l and c h ro n o lo g ic age sh ow ed a sig n ifica n t n u m b e r o f v alu es b elo w th e norm al 50th p e rc e n tile — m ainly in th e p re sch o o l age g ro u p . T h e m ed ian v a lu es fo r h eight in b oth m a les and fe m a le s fo llo w ed th e 25th and 50th p e rc e n tile s . T h e m a jo rity o f c o rre la tio n s in th is stud y show ed d ata b elo w norm al fig u res. C a tch -u p gro w th w as d o cu m en ted a s d iet in ta k e in cre a se d and th e ra p y w as in stitu te d . In stu d ies by G r e e n and o th e r s 6 in 1967 and M ilu n sk y and o th e r s 7 in 1971, grow th h o rm o n e le v e ls w ere e le vated in p a tie n ts w ith c y s tic fib ro s is . A stud y b y D e n n is and P a n o s 8 in 1965 in w h ich a n a b o lic s te ro id s w ere used to tre a t 31 p a tie n ts w ith c y s tic fib ro s is in th e p rep u b erta l age group sh ow ed re sp o n se in h eig h t g a in , w eight g ain , m u scle to n e , and b o n e ag e. T h e a n a b o lic stero id tre a tm e n t had a d efin ite in flu e n c e on s k e le ta l m atu ratio n as m easu red fro m rad io g rap h s o f th e w rist. T h e a u th o rs sta te d th a t, alth ou gh the tre a tm e n t is n o n s p e c ific , th e re sp o n se has b ee n p o sitiv e . A stu d y by S n o d g ra ss and o th e r s 9 in 1955 sh ow ed th a t ch ild re n w ith c y s tic fib r o s is had a sy m m e tric s k e le ta l m atu ratio n and e p ip h y sea l a n o m a lies sim ila r to th o se in ch ild ren w ith n u tri tion al p ro b lem s. T h e stud y w as m ad e on a c r o s s se c tio n a l group o f 255 c h ild re n , age 5 to 14. A g re a te r lag b e tw e e n sk e le ta l age and c h ro n o lo g ic age w as n o ted in th e ch ild re n w ith c y s tic fib ro s is and nu tritio n al p ro b lem s than in th e norm al g ro u p , as noted fro m th e rad io g rap h s o f the w rists. In 1973 T a u s s ig and o th e r s 10 p re se n te d a c a s e 84 ■ JADA, Vol. 96, January 1978
stu d y o f c y s tic fib r o s is and S ilv e r-R u s s e ll d w arf ism in a tw in co m p a re d w ith h is norm al sib lin g . T h e cra n ia l b a se m e a su re m e n ts in th e tw in w ith c y s tic fib ro s is w ere sig n ifica n tly le ss than th o se o f the norm al tw in and th e sta n d a rd s used fo r c o m p a riso n . T h e a re a a ffe c te d m o st s e v e re ly w as th e p o ste rio r cra n ia l b a s e . F a c ia l h eight m e a s u rem en ts sh ow ed d e c r e a s e s in b oth u p p er and lo w e r h eigh t m e a su re m e n ts. T h e r e w as a d e c r e a s e in m an d ibu lar size w ith an in cre a se d gonial an g le. T h e m a x illa w as sm a lle r th an n o rm a l. T h e m e a su re m e n ts o f th e norm al tw in co m p are d fa v o ra b ly to the stan d ard u sed . U n fo rtu n a te ly , o n ly o n e su ch c a s e has b e e n re p o rted w ith d e tailed re c o rd e d ce p h a lo m e tric m e a su re m e n ts. T h is stud y e x a m in e s in d etail th e sk ele ta l c e p h a lo m e tric m e a su re m e n ts o f a g ro u p o f c h il d ren w ith c y s tic fib ro s is and th e ir sib lin g s.
M e th o d s a n d m ate ria ls T h e d ata sam p le c o n s is te d o f 61 la tera l c e p h a lo m e tric rad iog rap h s o f p a tie n ts w ith c y s tic fib r o s is and 15 la tera l c e p h a lo m e tric rad iog rap h s o f th e n orm al sib lin g s o f so m e o f th e s e p a tie n ts. T h e d ata w e re c o lle c te d fro m th e c y s tic fib ro s is c lin ic p o p u latio n at th e L o n g Isla n d Je w is h H ill sid e M e d ic a l C e n te r. E a c h y e a r th is c lin ic tre a ts an a v e ra g e o f 150 p a tie n ts w ith c y s tic fib r o s is . In
THE AUTHORS
REITMAN
FABER
Drs. Reitman and Faber are on the staff, de partment of dentistry, Long Island Jewish Hill side Medical Center, New Hyde Park, NY, and are assistant professors of clinical children’s den tistry, State University of New York at Stony Brook. Address requests for reprints to Dr. Reit man, Garden City Medical Center, 520 Franklin Ave, Garden City, NY 11530.
CYSTIC FIBROSIS PATIENTS 27 34 61 -2
MALES FEMALES TOTAL NOT USEABLE
59
USED IN STUDY
NORMAL SIBLINGS 7 8 15 -4
MALES FEMALES TOTAL NOT USEABLE
11 USED IN STUDY
K 7 Ie Uj G 5
6 5 4
S
2
! j i Ui . .
2 3 4 5 6 7 8 9 10 II 12 13 14 15 16 17 18 19 20 21 22 23 AGE(YEARS)
5 6 7 8 9 10 I M 2 13 14 15 AGE (YEARS)
Fig 1 ■ Histogram of age distribution of patients and siblings in cephalometric study.
the group with cystic fibrosis, two of the radio graphs were not usable for analysis, thus reduc ing the sample size to 59 patients. In the sibling group, because of missing data and poor films, four could not be used, thus reducing the number to 11 patients. The patients ranged in age from 2 to 23. The distribution of the data sample is shown in Figure 1. The males and females were grouped together since no sexual dimorphic characteris tics were evident. However, the male patients were compared to male standards and the female patients to female standards on an individual basis initially. As the histogram of the combined sample in Figure 1 shows, there are natural breakpoints in the data at ages 9 and 14. It was therefore decided to use three groups for analysis, resulting in pre pubertal, pubertal, and postpubertal groups. The sibling group was similarly divided. The data groups for both the cystic fibrosis sample and the sibling sample were compared to a third standard, since the normal sibling group was too small to give a statistically meaningful refer ence group. The standard used was a Caucasian longitudinal growth sample obtained from the Denver Growth Center by the University of
Connecticut Health Center department of or thodontics. The sample was composed of about 200 patients who had been observed from birth to maturity. The data analysis was performed using the computerized cephalometric analysis system developed at the University of Connecticut Health Center department of orthodontics. The system consists of a digitizer that electronically enters the X -Y coordinates of cephalometric landmarks, marked on a tracing, into a computer program to make the measurements and compare them with the standards that are already stored in the computer program. The system error is about 0.25 mm. The standardized data are stored in the computer program longitudinally by age from 3 to 23. A tracing was made of each radiograph before information was entered into the computer. Each patient was compared with a standard for the cor responding age and sex. The cephalometric analysis performed relates size, position, and angular relationship of the skeletal parts (cranial base, maxilla, mandible) to themselves and each other. The measurements in the table are those examined in this study. The Reitm an— Faber: CEPHALOMETRIC STUDY OF CF PATIENTS ■ 85
Fig 2 ■ Cephalometric measurements used in study.
FH refers to the effective distance in millimeters along a constructed Frankfort Horizontal refer ence line (FH is -7 ° to S-N ). The pFH refers to those measurements made perpendicular to the FH reference line. The actual measurements made are illustrated in Figure 2. The standards chosen for comparison in the table in each group were the mean age standard for each of the three groups analyzed. This was extrapolated from the age distribution in each group. The analysis was done individually for each radiograph. Those data are not discussed in this paper.
were exceptions. The exceptions seemed to be in the A -Pg measurement in patients in groups 1 and 3 who had cystic fibrosis and normal patients with increased measurements. Comparison of meas urements of siblings with standard measurements must be done with care, especially in group 1 where the sample size was limited to one sib ling. In groups 2 and 3, the trend appeared to be that the sibling measurements for cranial base and maxilla length were larger than the standard. In comparison, the measurements for patients with cystic fibrosis were closer to the standard and smaller than the sibling group. In group 2, the pubertal group, the measurements compared favorably with the standards.
Results The data for this study are summarized in the Table; measurements are compared for each age group which includes patients with cystic fibrosis and normal sibling data to the mean standards for that group. In each of the groups the data for patients with cystic fibrosis compared favorably with the mean standard used. This also was gen erally true on an individual basis although there 86 ■ JADA, Vol. 96, January 1978
Discussion The data presented in this study seem to indicate that the skeletal cephalometric measurements of patients with cystic fibrosis in the prepubertal, pubertal, and postpubertal periods compare favorably with a longitudinal Caucasian sample of normal data. The sibling data do not present a
Table ■ Com parison of cephalom etric data between groups 1, 2, and 3: cystic fibrosis and sibling groups.* N-Ar(FH) GROUP 1 C ystic fibrosis 1 Age: 15-25 yrs. n = 16 Avg age: 17.6±2.1 S iblings Age: 15-25 yrs. n= 1 Avg age: 15.5 S tandard 17.0 yrs. GROUP 2 C ystic fib ro sis 2 Age: 10-14 yrs. n=23 Avg age: 11.9±1.3 S iblings Age: 10-14 yrs. n=5 Avg age: 11.0±0.8 Standard 12.0 yrs. GROUP 3 Cystic fibrosis 3 Age: 3-9 yrs. n=20 Avg age: 6.7±1.4 S iblings Age 3-9 yrs. n=5 Avg age: 7.8±2.1 S tandard 7.0 yrs.
PSN-A(FH) Ar-Pg(FH)
N-ANS(pFH)
ANS-Me(pFH)
N-A-PG
N-A(FH)
N-Pg(FH)
A-Pg(FH)
89.7 ±5.6
51.0 ±5.1
80.9 ±11.3
55.9 ±3.7
64.4 ±4.0
4.4 ±5.2
-1 .9 ±5.1
-7 .8 ±9.4
- 6 .4 ±5.8
98.1
53.0
79.8
57.6
74.8
-0 .1
-9 .0
-1 8 .3
- 9 .3
86.3 ±4.4
52.5 ±2.5
80.9 ±6.3
52.4 ±2.8
65.0 ±3.6
3.3 ±5.6
-1 .0 ±3.7
- 5 .4 ±6.8
0.0 ±3.4.
83.7 ±4.5
47.7 ±4.0
73.1 ±6.3
51.4 ±3.4
58.7 ±4.8
7.1 ±5.3
- 2 .4 ±3.5
-9 .8 ±5.5
-7 .7 ±4.1
91.6 ±4.0
51.0 ±3.4
82.0 ±6.5
53.2 ±0.9
67.9 ±3.4
4.4 ±2.9
- 2 .8 ±1.8
- 9 .8 ±7.3
- 7 .0 ±3.5
82.5 ±4.0
47.3 ±2.3
73.8 ±5.0
49.2 ±2.7
59.3 ±3.3
5.3 ±4.7
-2 .0 ±3.4
-8 .6 ±5.5
- 7 .2 ±3.3
78.3 ±4.6
43.9 ±2.6
64.3 ±6.4
46.0 ±4.2
53.6 ±2.9
12.6 ±6.2
-1 .9 ±2.5
-14.1 ±5.9
-1 2.1 ±5.1
82.9 ±5.0
45.1 ±3.4
68.5 ±6.5
49.8 ±2.3
60.0 ±5.1
11.0 ±5.1
-2 .3 ±1.6
-1 4 .4 ±9.9
-1 2 .2 ±6.9
76.6 ±3.8
43.8 ±2.0
66.0 ±4.3
43.6 ±2.7
54.1 ±2.7
7.6 ±4.4
-1 .9 ±3.3
-1 0 .7 ±5.1
-0 .5 ±2.6
'G ro u p s 1 and 3 in the A-Pg (FH) measurement showed som e tendency fo r Class II m alocclusion, but the results of the N-A-Pg measurement, w hich is sim ilar to an ANB measurement, do not bear this out. Since there was a con flict, no com m ent can be made at this time. In addition, the CF population looked at in th is study was screened from the dental clin ic patients the m edical c lin ic called. Therefore, it may be a som ew hat biased sample.
clear pattern, probably because of the statistically small sample size. Thus, no firm conclusions can be drawn from the sibling data. The population with cystic fibrosis examined in this study was undergoing medical treatment so the favorable correspondence with the standards used was ex pected. The literature shows that the treated pa tient with cystic fibrosis has normal somatic development if treated early.
S u m m a ry
A study of 61 patients with cystic fibrosis and their siblings was performed with cephalometric analyses of their facial skeletons. The data showed that in the sample of treated patients their cephalometric measurements compared favora bly with normal Caucasian standards.
S upported by research fund no. 3-224, Long Island Jewish H ill side Medical Center, New Hyde Park, NY. The authors tha nk Dr. Jack Gorvoy, d ire c to r of the Cystic Fibro sis Center at Long Island Jewish H illside Medical Center, fo r pro
viding the patient population fo r study; Dr. Leon Eisenbud, d ire c to r of de ntistry at the Long Island Jewish H illside Medical Center fo r encouragem ent and facilities in w hich to exam ine patients and record find in gs; and Dr. Charles B urstone at th e University of C onnecticut Health Center Departm ent o f O rthodo ntics fo r use of the com puter fa c ility and program s fo r data re duction and analy sis. 1. Haung, N., and others. S urvival of patients w ith cystic fibrosis. Am J Dis Child 20:289 Oct 1970. 2. Shwachm an, H.,and Kulcz, L. Long term study of one hundred five patients w ith cystic fibrosis. Am J Dis Child 96:6 Jan 1958. 3. G orlin, R.J., and Goldman, H.M., eds. Thom a's oral pathology. St. Louis, C. V. Mosby, 1970, p 995. 4. S proul, A., and Huang, N. G row th patterns in children w ith cystic fibrosis. J P ediatr 65:664 May 1964. 5. Kreibl, T., and others. The physical developm ent of children w ith cystic fibrosis. Z K inderheilkd 113:93 Jan 1972. 6. Green, O.C., and others. Plasma grow th ho rm one levels in children w ith cystic fibrosis and short stature: unresponsiveness to hypoglycem ia. E ndocrinol Metab J Clin 27:1059 June 1967. 7. M ilunsky, A., and others. Insulin, glucose, grow th horm one and free fatty acids. Am J Dis Child 121:15 Jan 1971. 8. Dennis, J.L., and Panos.T.C. G row th and bone age retardation in cystic fibrosis: a response to an anabolic steroid. JADA 94:855 Nov 1965. 9. Snodgrass, R.M., and others. The association between anom alous ossification centers in the hand skeleton, nu tritiona l status and the rate o f skeletal matu rati o n in c h ild re n fiv e to fo u rteen years o f age. Am J Roentgenol Radium Ther Nucl Med 74:6 Dec 1955. 10. Taussig, L., and others. Silver-Russell dw arfism and cystic fibrosis in a tw in. Am J Dis Child 125:495 A pril 1973.
Reitman— Faber: CEPHALOMETRIC STUDY OF CF PATIENTS ■ 87
C hildren with cystic fibrosis have skeletal m aturation and epiphyseal anom alies sim ilar to those in children with nutritional problem s. H owever, these patients have been found to have norm al som atic developm ent if treatm en t is begun early. In a g roup of children w ith cystic fibrosis undergoing m edical trea tm en t, cep h alom etric m easurem ents com pared favorably w ith those established by norm al standards.
A cephalometric study of patients with cystic fibrosis Albert A. Reitman, DDS Richard D. Faber, DDS, MS, New Hyde Park, N Y *
Early detection and diagnosis of children with cystic fibrosis treated with modern therapeutic regimens have increased the survival rate in these patients well beyond childhood. Haung and others1 reported in 1970 that the survival rates have increased from 35% (1952 to 1957) to 76.6% (1962 to 1967). Shwachman and Kulcz2 reported that, with antibiotic therapy to treat the gastroin testinal and respiratory problems, life expectancy has been increased for most patients. This in crease is attributed to early diagnosis and current therapeutic regimens. Cystic fibrosis3 is consid ered to be a hereditary disorder of children and young adults, which has both primary and secon dary effects on the digestive and respiratory tracts from dysfunction of exocrine glands. These dis orders can have dramatic effects on the physical development of children.
Changes in physical development in patients with cystic fibrosis have been reported. The premise usually proposed for this development parallels that for children with genetic and pre natal influences, nutritional disturbances, and pulmonary disease. In 1964 Sproul and Huang4 reported on a study of 50 patients with cystic fibrosis in which height, weight, and bone age were evaluated during a two-year period; a signif icant physical somatic retardation was noted at all ages. The deficiency in height and weight was noticeable in preadolescent age groups with an absence of the growth spurt. Height changes and gain and loss of weight showed a direct corre spondence with respiratory conditions, especially bouts of pneumonia. Skeletal retardation was noted in 38% of the population studied; the most pronounced abnormality appeared in the preado JADA, Vol. 96, January 1978 ■ 83
le s c e n t g ro u p . In n on e o f th e age groups did th e m ed ian o f the m easu red d a ta in th e c y s tic fib ro s is group e x c e e d th e te n th p e rc e n tile o f th e n orm al group d ata. T h is stud y a lso sh ow ed th a t w ith the in stitu tio n o f th e ra p y th e re w as a c a tc h -u p p h e n o m en o n c o n c u rre n t w ith th e age and sta g e o f d ev elo p m en t at th e tim e th e ra p y w as in itia te d . It ca n b e co n clu d e d fro m th is stud y th a t in a n un tre a te d p o p u latio n it m ight b e e x p e c te d th a t so m a tic g row th w ould be n o tic e a b ly a ffe c te d . In a tre a te d p o p u latio n th e c h a n g e s m ay n ot b e as d ra m a tic. In a 1972 study by K re ib l and o t h e r s ,5 p h y sica l d ev elo p m en t o f 53 ch ild ren w ith c y s tic fib ro sis w as e v alu a te d fo r h eigh t and w eig h t, lin e a r and v e lo c ity ch a n g e s , and sk e le ta l a g e s. W eig h t and w eight v e lo c ity w ere fo u n d to b e d ire ctly c o r r e lated w ith th e se v e rity o f d is e a s e . N o sig n ifica n t re la tio n sh ip w a s found b etw ee n h eig h t and s k e le ta l age a s d eterm in ed from fig u res fro m an a tla s . T h e c o r re la tio n b e tw e e n s k ele ta l and c h ro n o lo g ic age sh ow ed a sig n ifica n t n u m b e r o f v alu es b elo w th e norm al 50th p e rc e n tile — m ainly in th e p re sch o o l age g ro u p . T h e m ed ian v a lu es fo r h eight in b oth m a les and fe m a le s fo llo w ed th e 25th and 50th p e rc e n tile s . T h e m a jo rity o f c o rre la tio n s in th is stud y show ed d ata b elo w norm al fig u res. C a tch -u p gro w th w as d o cu m en ted a s d iet in ta k e in cre a se d and th e ra p y w as in stitu te d . In stu d ies by G r e e n and o th e r s 6 in 1967 and M ilu n sk y and o th e r s 7 in 1971, grow th h o rm o n e le v e ls w ere e le vated in p a tie n ts w ith c y s tic fib ro s is . A stud y b y D e n n is and P a n o s 8 in 1965 in w h ich a n a b o lic s te ro id s w ere used to tre a t 31 p a tie n ts w ith c y s tic fib ro s is in th e p rep u b erta l age group sh ow ed re sp o n se in h eig h t g a in , w eight g ain , m u scle to n e , and b o n e ag e. T h e a n a b o lic stero id tre a tm e n t had a d efin ite in flu e n c e on s k e le ta l m atu ratio n as m easu red fro m rad io g rap h s o f th e w rist. T h e a u th o rs sta te d th a t, alth ou gh the tre a tm e n t is n o n s p e c ific , th e re sp o n se has b ee n p o sitiv e . A stu d y by S n o d g ra ss and o th e r s 9 in 1955 sh ow ed th a t ch ild re n w ith c y s tic fib r o s is had a sy m m e tric s k e le ta l m atu ratio n and e p ip h y sea l a n o m a lies sim ila r to th o se in ch ild ren w ith n u tri tion al p ro b lem s. T h e stud y w as m ad e on a c r o s s se c tio n a l group o f 255 c h ild re n , age 5 to 14. A g re a te r lag b e tw e e n sk e le ta l age and c h ro n o lo g ic age w as n o ted in th e ch ild re n w ith c y s tic fib ro s is and nu tritio n al p ro b lem s than in th e norm al g ro u p , as noted fro m th e rad io g rap h s o f the w rists. In 1973 T a u s s ig and o th e r s 10 p re se n te d a c a s e 84 ■ JADA, Vol. 96, January 1978
stu d y o f c y s tic fib r o s is and S ilv e r-R u s s e ll d w arf ism in a tw in co m p a re d w ith h is norm al sib lin g . T h e cra n ia l b a se m e a su re m e n ts in th e tw in w ith c y s tic fib ro s is w ere sig n ifica n tly le ss than th o se o f the norm al tw in and th e sta n d a rd s used fo r c o m p a riso n . T h e a re a a ffe c te d m o st s e v e re ly w as th e p o ste rio r cra n ia l b a s e . F a c ia l h eight m e a s u rem en ts sh ow ed d e c r e a s e s in b oth u p p er and lo w e r h eigh t m e a su re m e n ts. T h e r e w as a d e c r e a s e in m an d ibu lar size w ith an in cre a se d gonial an g le. T h e m a x illa w as sm a lle r th an n o rm a l. T h e m e a su re m e n ts o f th e norm al tw in co m p are d fa v o ra b ly to the stan d ard u sed . U n fo rtu n a te ly , o n ly o n e su ch c a s e has b e e n re p o rted w ith d e tailed re c o rd e d ce p h a lo m e tric m e a su re m e n ts. T h is stud y e x a m in e s in d etail th e sk ele ta l c e p h a lo m e tric m e a su re m e n ts o f a g ro u p o f c h il d ren w ith c y s tic fib ro s is and th e ir sib lin g s.
M e th o d s a n d m ate ria ls T h e d ata sam p le c o n s is te d o f 61 la tera l c e p h a lo m e tric rad iog rap h s o f p a tie n ts w ith c y s tic fib r o s is and 15 la tera l c e p h a lo m e tric rad iog rap h s o f th e n orm al sib lin g s o f so m e o f th e s e p a tie n ts. T h e d ata w e re c o lle c te d fro m th e c y s tic fib ro s is c lin ic p o p u latio n at th e L o n g Isla n d Je w is h H ill sid e M e d ic a l C e n te r. E a c h y e a r th is c lin ic tre a ts an a v e ra g e o f 150 p a tie n ts w ith c y s tic fib r o s is . In
THE AUTHORS
REITMAN
FABER
Drs. Reitman and Faber are on the staff, de partment of dentistry, Long Island Jewish Hill side Medical Center, New Hyde Park, NY, and are assistant professors of clinical children’s den tistry, State University of New York at Stony Brook. Address requests for reprints to Dr. Reit man, Garden City Medical Center, 520 Franklin Ave, Garden City, NY 11530.
CYSTIC FIBROSIS PATIENTS 27 34 61 -2
MALES FEMALES TOTAL NOT USEABLE
59
USED IN STUDY
NORMAL SIBLINGS 7 8 15 -4
MALES FEMALES TOTAL NOT USEABLE
11 USED IN STUDY
K 7 Ie Uj G 5
6 5 4
S
2
! j i Ui . .
2 3 4 5 6 7 8 9 10 II 12 13 14 15 16 17 18 19 20 21 22 23 AGE(YEARS)
5 6 7 8 9 10 I M 2 13 14 15 AGE (YEARS)
Fig 1 ■ Histogram of age distribution of patients and siblings in cephalometric study.
the group with cystic fibrosis, two of the radio graphs were not usable for analysis, thus reduc ing the sample size to 59 patients. In the sibling group, because of missing data and poor films, four could not be used, thus reducing the number to 11 patients. The patients ranged in age from 2 to 23. The distribution of the data sample is shown in Figure 1. The males and females were grouped together since no sexual dimorphic characteris tics were evident. However, the male patients were compared to male standards and the female patients to female standards on an individual basis initially. As the histogram of the combined sample in Figure 1 shows, there are natural breakpoints in the data at ages 9 and 14. It was therefore decided to use three groups for analysis, resulting in pre pubertal, pubertal, and postpubertal groups. The sibling group was similarly divided. The data groups for both the cystic fibrosis sample and the sibling sample were compared to a third standard, since the normal sibling group was too small to give a statistically meaningful refer ence group. The standard used was a Caucasian longitudinal growth sample obtained from the Denver Growth Center by the University of
Connecticut Health Center department of or thodontics. The sample was composed of about 200 patients who had been observed from birth to maturity. The data analysis was performed using the computerized cephalometric analysis system developed at the University of Connecticut Health Center department of orthodontics. The system consists of a digitizer that electronically enters the X -Y coordinates of cephalometric landmarks, marked on a tracing, into a computer program to make the measurements and compare them with the standards that are already stored in the computer program. The system error is about 0.25 mm. The standardized data are stored in the computer program longitudinally by age from 3 to 23. A tracing was made of each radiograph before information was entered into the computer. Each patient was compared with a standard for the cor responding age and sex. The cephalometric analysis performed relates size, position, and angular relationship of the skeletal parts (cranial base, maxilla, mandible) to themselves and each other. The measurements in the table are those examined in this study. The Reitm an— Faber: CEPHALOMETRIC STUDY OF CF PATIENTS ■ 85
Fig 2 ■ Cephalometric measurements used in study.
FH refers to the effective distance in millimeters along a constructed Frankfort Horizontal refer ence line (FH is -7 ° to S-N ). The pFH refers to those measurements made perpendicular to the FH reference line. The actual measurements made are illustrated in Figure 2. The standards chosen for comparison in the table in each group were the mean age standard for each of the three groups analyzed. This was extrapolated from the age distribution in each group. The analysis was done individually for each radiograph. Those data are not discussed in this paper.
were exceptions. The exceptions seemed to be in the A -Pg measurement in patients in groups 1 and 3 who had cystic fibrosis and normal patients with increased measurements. Comparison of meas urements of siblings with standard measurements must be done with care, especially in group 1 where the sample size was limited to one sib ling. In groups 2 and 3, the trend appeared to be that the sibling measurements for cranial base and maxilla length were larger than the standard. In comparison, the measurements for patients with cystic fibrosis were closer to the standard and smaller than the sibling group. In group 2, the pubertal group, the measurements compared favorably with the standards.
Results The data for this study are summarized in the Table; measurements are compared for each age group which includes patients with cystic fibrosis and normal sibling data to the mean standards for that group. In each of the groups the data for patients with cystic fibrosis compared favorably with the mean standard used. This also was gen erally true on an individual basis although there 86 ■ JADA, Vol. 96, January 1978
Discussion The data presented in this study seem to indicate that the skeletal cephalometric measurements of patients with cystic fibrosis in the prepubertal, pubertal, and postpubertal periods compare favorably with a longitudinal Caucasian sample of normal data. The sibling data do not present a
Table ■ Com parison of cephalom etric data between groups 1, 2, and 3: cystic fibrosis and sibling groups.* N-Ar(FH) GROUP 1 C ystic fibrosis 1 Age: 15-25 yrs. n = 16 Avg age: 17.6±2.1 S iblings Age: 15-25 yrs. n= 1 Avg age: 15.5 S tandard 17.0 yrs. GROUP 2 C ystic fib ro sis 2 Age: 10-14 yrs. n=23 Avg age: 11.9±1.3 S iblings Age: 10-14 yrs. n=5 Avg age: 11.0±0.8 Standard 12.0 yrs. GROUP 3 Cystic fibrosis 3 Age: 3-9 yrs. n=20 Avg age: 6.7±1.4 S iblings Age 3-9 yrs. n=5 Avg age: 7.8±2.1 S tandard 7.0 yrs.
PSN-A(FH) Ar-Pg(FH)
N-ANS(pFH)
ANS-Me(pFH)
N-A-PG
N-A(FH)
N-Pg(FH)
A-Pg(FH)
89.7 ±5.6
51.0 ±5.1
80.9 ±11.3
55.9 ±3.7
64.4 ±4.0
4.4 ±5.2
-1 .9 ±5.1
-7 .8 ±9.4
- 6 .4 ±5.8
98.1
53.0
79.8
57.6
74.8
-0 .1
-9 .0
-1 8 .3
- 9 .3
86.3 ±4.4
52.5 ±2.5
80.9 ±6.3
52.4 ±2.8
65.0 ±3.6
3.3 ±5.6
-1 .0 ±3.7
- 5 .4 ±6.8
0.0 ±3.4.
83.7 ±4.5
47.7 ±4.0
73.1 ±6.3
51.4 ±3.4
58.7 ±4.8
7.1 ±5.3
- 2 .4 ±3.5
-9 .8 ±5.5
-7 .7 ±4.1
91.6 ±4.0
51.0 ±3.4
82.0 ±6.5
53.2 ±0.9
67.9 ±3.4
4.4 ±2.9
- 2 .8 ±1.8
- 9 .8 ±7.3
- 7 .0 ±3.5
82.5 ±4.0
47.3 ±2.3
73.8 ±5.0
49.2 ±2.7
59.3 ±3.3
5.3 ±4.7
-2 .0 ±3.4
-8 .6 ±5.5
- 7 .2 ±3.3
78.3 ±4.6
43.9 ±2.6
64.3 ±6.4
46.0 ±4.2
53.6 ±2.9
12.6 ±6.2
-1 .9 ±2.5
-14.1 ±5.9
-1 2.1 ±5.1
82.9 ±5.0
45.1 ±3.4
68.5 ±6.5
49.8 ±2.3
60.0 ±5.1
11.0 ±5.1
-2 .3 ±1.6
-1 4 .4 ±9.9
-1 2 .2 ±6.9
76.6 ±3.8
43.8 ±2.0
66.0 ±4.3
43.6 ±2.7
54.1 ±2.7
7.6 ±4.4
-1 .9 ±3.3
-1 0 .7 ±5.1
-0 .5 ±2.6
'G ro u p s 1 and 3 in the A-Pg (FH) measurement showed som e tendency fo r Class II m alocclusion, but the results of the N-A-Pg measurement, w hich is sim ilar to an ANB measurement, do not bear this out. Since there was a con flict, no com m ent can be made at this time. In addition, the CF population looked at in th is study was screened from the dental clin ic patients the m edical c lin ic called. Therefore, it may be a som ew hat biased sample.
clear pattern, probably because of the statistically small sample size. Thus, no firm conclusions can be drawn from the sibling data. The population with cystic fibrosis examined in this study was undergoing medical treatment so the favorable correspondence with the standards used was ex pected. The literature shows that the treated pa tient with cystic fibrosis has normal somatic development if treated early.
S u m m a ry
A study of 61 patients with cystic fibrosis and their siblings was performed with cephalometric analyses of their facial skeletons. The data showed that in the sample of treated patients their cephalometric measurements compared favora bly with normal Caucasian standards.
S upported by research fund no. 3-224, Long Island Jewish H ill side Medical Center, New Hyde Park, NY. The authors tha nk Dr. Jack Gorvoy, d ire c to r of the Cystic Fibro sis Center at Long Island Jewish H illside Medical Center, fo r pro
viding the patient population fo r study; Dr. Leon Eisenbud, d ire c to r of de ntistry at the Long Island Jewish H illside Medical Center fo r encouragem ent and facilities in w hich to exam ine patients and record find in gs; and Dr. Charles B urstone at th e University of C onnecticut Health Center Departm ent o f O rthodo ntics fo r use of the com puter fa c ility and program s fo r data re duction and analy sis. 1. Haung, N., and others. S urvival of patients w ith cystic fibrosis. Am J Dis Child 20:289 Oct 1970. 2. Shwachm an, H.,and Kulcz, L. Long term study of one hundred five patients w ith cystic fibrosis. Am J Dis Child 96:6 Jan 1958. 3. G orlin, R.J., and Goldman, H.M., eds. Thom a's oral pathology. St. Louis, C. V. Mosby, 1970, p 995. 4. S proul, A., and Huang, N. G row th patterns in children w ith cystic fibrosis. J P ediatr 65:664 May 1964. 5. Kreibl, T., and others. The physical developm ent of children w ith cystic fibrosis. Z K inderheilkd 113:93 Jan 1972. 6. Green, O.C., and others. Plasma grow th ho rm one levels in children w ith cystic fibrosis and short stature: unresponsiveness to hypoglycem ia. E ndocrinol Metab J Clin 27:1059 June 1967. 7. M ilunsky, A., and others. Insulin, glucose, grow th horm one and free fatty acids. Am J Dis Child 121:15 Jan 1971. 8. Dennis, J.L., and Panos.T.C. G row th and bone age retardation in cystic fibrosis: a response to an anabolic steroid. JADA 94:855 Nov 1965. 9. Snodgrass, R.M., and others. The association between anom alous ossification centers in the hand skeleton, nu tritiona l status and the rate o f skeletal matu rati o n in c h ild re n fiv e to fo u rteen years o f age. Am J Roentgenol Radium Ther Nucl Med 74:6 Dec 1955. 10. Taussig, L., and others. Silver-Russell dw arfism and cystic fibrosis in a tw in. Am J Dis Child 125:495 A pril 1973.
Reitman— Faber: CEPHALOMETRIC STUDY OF CF PATIENTS ■ 87
