The effect of early dental treatment of anhydrotic ectodermal dysplasia

The effect of early dental treatment of anhydrotic ectodermal dysplasia

The effect o f e a rly dental tre atm e n t o f a n h y d ro tic ecto d e rm al d y s p la s ia H u sh a n g B o r jia n * D .D .S ., C orp u s Chris...

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The effect o f e a rly dental tre atm e n t o f a n h y d ro tic ecto d e rm al d y s p la s ia

H u sh a n g B o r jia n * D .D .S ., C orp u s Christi, T e x a s

A ll patients with anhydrotic ectodermal dysplasia have dental defects which may lead to serious consequences. T h e pur­ pose o f this paper is to show the need o f early oral rehabilitation and to de­ scribe the procedures involved in the treatment of these dental defects. A d e ­ quate early treatment is necessary in order to provide more normal physical development through better nutrition, and better social and emotional adjust­ ment by improving the appearance o f the child. REVIEW OF TH E LITERATURE

Koszewski and Hubbard have offered the most concise definition o f this condition when they say, “ Hereditary ectodermal dysplasia presents a syndrome o f incom­ plete development o f the epidermis and its appendages. . . .” It has been pointed out that the disease involves not only skin and structures derived from ecto­ derm, but also organs o f mesodermal and endodermal origin. T h e clinical aspects o f individual cases vary depending on the extent of tissue involvement.1 This condition occurs far more fre­ quently in males than in females.2 Wechselmann3 claims that this syndrome is transmitted as sex-linked-hereditary fac­ tor from clinically normal females to

the male offspring o f a family in the same manner as hemophilia. I f this is true, there must be more than one type o f hereditary transmission since females are affected in 4 to 20 per cent of cases.4,5’ 2 This syndrome seems to occur in all races as has been reported by Everett and others.6,5 Th e patient who is affected by the anhydrotic type o f ectodermal dysplasia can be identified by his prominent fore­ head, wide brows, saddle nose (depressed nasal root and bridge), rather thick and everted lips, satyr-like ears, soft skin and sparse hair.4'6 Th e skin is soft, thin and dry with few or no functioning sweat glands evident. Th e first outstanding characteristic of this disease is anhydrosis or hypohydrosis. For this reason these individuals are in­ tolerant to heat and are incapacitated during hot weather. T h e skin also tends to be hypersensitive to irritants.7 The second outstanding feature is anodontia or oligodontia. There are other anomalies in connec­ tion with anhydrotic ectodermal dyspla­ sia which have been described as sympto­ matic o f this syndrome. T h e hair o f the scalp, eyelashes and eyebrows is sparse. Th e scalp hair usually becomes short, fine and stiff after puberty. T h e beard is more frequently normal, and axillary

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and pubic hair is either scanty or absent. Sebaceous gland secretion is either im ­ paired or absent.8,5 Clarke and M cCance and others report that patients with this condition often have poorly developed or absent mammary glands.9 Th e nails of patients vary: they may be normal, may show slow growth, may be dystrophic with scalelike, longitudinal furrows or they may be completely absent from some of the fingers.10,5 One anhydrotic patient has been reported with six fingers on each hand.10 Metson and Williams11 mention atrophic rhinitis, chronic pharyn­ gitis, laryngitis and the absence of smell and taste. Upshaw found the glands o f the mucous membrane of the nose and throat to be deficient in secretory func­ tion, resulting in dysphagia, hoarseness and intermittent complete aphonia. These patients suffer frequent upper respiratory infections and some have malodorous se­ cretions from the nose.12'14’ 5 It has been mentioned also that the mouth is dry with a somewhat atrophic mucosa. Th e absence o f tears and myopia have been reported by Bowen.4 Adrenal and pitui­ tary dysfunctions have been reported in occasional instances.5 Koszewski and Hubbard claim that chronic anemia ap­ pears to be a significant feature in many instances.1 Th e mentality o f the patients varies from normal to inferior.15,4’ 7 Some report that mentality is average at an early age although the child matures poorly so as to be inferior as he grows older.8 Sexual development is often poor.5,10 Anodontia or oligodontia is extremely grave because o f its effect on the nutri­ tion and emotional stability of the indi­ vidual. Missing teeth (oligodontia) or absence o f teeth (anodontia) occur in both the deciduous and the permanent dentition. In the case o f oligodontia, the teeth are slow to erupt and abnormal in appearance. They are often coneshaped, fragile, milk-white and some­ what opaque, while some are darkly pigmented.16,10,5,6,7 T h e erupted teeth,

particularly the incisors, cuspids and bi­ cuspids, are often widely separated.5,12 Th e pulp and root canal of these teeth seem to be large, and the occurrence o f carious lesions is high.6 T h e alveolar pro­ cesses may not develop because o f the absence o f teeth but the basal bone may develop normally.17 Histopathologic study o f the epidermis o f anhydrotic patients reveals it to be thinner than normal. T h e sweat glands are either completely absent or poorly and irregularly developed.5,8 T h e hair follicles are scanty and rudimentary with a deficiency o f pilosebaceous structures. Th e vascular beds are normal. T h e mu­ cous membranes of the mouth and throat show hypoplastic changes. T h e under­ lying glands as well as the epithelium are affected. There are some other syndromes which must be differentiated from anhydrotic ectodermal dysplasia. These are hydrotic ectodermal dysplasia, W erner’s syndrome and progeria of the Hutchinson-Gilford type.5 REPORT OF CASE

A two and one-half year old male child o f Mexican extraction was referred to the dental department o f Driscoll Foun­ dation Children’s Hospital on January 2, 1959, because o f apparent absence of teeth. Th e primary concern of the parents was that he was not playing well with the other children, because they would bite him and he was not able to bite in return. A history was given o f frequent episodes o f high fever, occasionally ac­ companied by convulsions. These attacks were associated with exposure to hot weather. H e had always had crusted nares, sparse light hair and scanty eye­ brows and eyelashes. His parents reported that he has never perspired or cried with tears. His growth and development had progressed within the limits of the normal and his mental development had seemed normal.

B O R J IA N . . . V O L U M E 61, N O V E M B E R I960 • 39/557

Fig.

I



Full fa c e v ie w o f p a tie n t a t first visit

Th e family history was negative for any o f the characteristic signs o f ecto­ dermal dysplasia. H e has a ten-month old sister who appears norm al; a paternal granduncle was an albino. Th e physical examination revealed the hair to be very sparse and blond, the skin dry, the eyebrows and eyelashes scanty, both nipples rudimentary in ap­ pearance and the nose saddle shaped. T h e stomatological examination showed the oral epithelium to be normal in ap­ pearance, the lips everted and rather thick (Fig. 1) and the jaws small with knife edge alveolar ridges. There were only two protruded milk-white and coneshaped maxillary central incisors. Roentgenographic examination o f the mouth and head indicated the presence o f four maxillary and two mandibular poorly formed anterior teeth (Fig. 2 ). Th e tongue appeared somewhat large whereas other structures o f the face and neck were normal. T h e remainder o f the ex­ amination was essentially negative. T h e findings warranted a dental diag­ nosis o f oligodontia as a symptom o f anhydrotic ectodermal dysplasia. It was decided to construct prosthetic dental appliances to take the place o f the miss­ ing natural dentition. This was necessary to correct the eversion o f the lips, to im­ prove the ability to masticate food, to

allow normal development of the muscles of the face and of mastication and to improve the appearance o f the child. This cosmetic improvement was impor­ tant not only from an esthetic standpoint but from a psychological point o f view as w ell; the child was the butt o f the jokes o f his playmates and a subject o f shame for his parents. During the first attempt to make impressions for construction of the dentures the patient was apprehen­ sive and in spite o f premedication became highly emotional with an attendant eleva­ tion o f temperature and convulsions, so that we were unable to complete the pro­ cedure. A t the next appointment, light nitrous oxide-oxygen supplemented with trichloroethylene was administered. H e tolerated the anesthetic very well and pre­ liminary impressions were taken with the maxillary central incisors present. A p ­ proximately one week later the same anesthetic was again used and the final impressions were taken. It was necessary

Fig. 2 * Profile, o p e n g ra m o f h e ad a n d neck

m ou th

view

ro e n t g e n o ­

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Fig. 3 • Left: Extraoral a p p e a r a n c e o f m outh re g io n d u r in g first w eek a fte r in se rtio n o f b o th u p p e r a n d low e r d en tu res. C e n te r: Extraoral .a p p e a ra n c e o f m ou th r e g io n a fte r tw o m on th s o f w e a rin g th e c o m p le te d en tu res. R ig h t: Extraoral a p p e a r a n c e o f m ou th r e g io n a fte r six m o n th s' use o f the d e n tu re s

to use this anesthetic a third time for a try-in o f the dentures before processing. On the day the dentures were to be de­ livered, the patient refused to keep them in his mouth for more than a few minutes at a time. T h e mother was instructed in the method of placing only the maxillary denture and was told to replace it each time the child removed it. W ithin 24 hours the child was content to leave the upper denture in place. T h e upper den­ ture was worn for one week and at that time the lower denture was inserted and the child tolerated it very well (Fig. 3, le ft). T h e occlusion was excellent and tissue adaptation was rapid. W ithin three weeks, the child completely mastered his

dentures and was able to masticate a diet compatible with his age. H e has become very attached to the dentures and cries when they are taken from him. Th e eversion o f the lips has been cor­ rected and the profile improved (Fig. 1. 3, 4 ). T h e child’s social behavior has improved considerably. His mother was instructed in maintaining good oral hy­ giene to prevent untimely decay o f the erupted teeth. These appliances will be changed periodically with the growth and development o f the jaws and the eruption of the six unerupted teeth. SUM M ARY

A case o f anhydrotic ectodermal dyspla­ sia with oligodontia has been reported A t the time o f this report, nine month: after starting dental treatment, the chilc is improved, cosmetically, physiologically and psychologically.

* D e n t a l d e p a r t m e n t , D r is c o ll H o s p it a l, C o r p u s C h r is t i, Texas.

F o u n d a t io n

C h i ld r e n ’

1. K o sze w sk i, B. J . t a n d H u b b a r d , T. F. C o n q e n it c a n e m ia in h e r e d it a r y e c t o d e r m a l d y s p la s ia . A . M . / A r c h . D e r m a t . 74:159 A u g . 1956. 2. P e a rso n , H . A . , a n d C o n e , T. E., J r. A n h y d r o t i e c t o d e r m a l d y s p l a s ia w ith v a s c u la r p u r p u ra . J. P e d ia ’ 51:306 S e p t . 1957. 3. W e c h s e lm a n n , W . , a n d L o e w y , A . U n t e r s u c h u n g e a n d r e i b lu t s v e r w a n d t e n P e rso n e n m it e k t o d e r m a le H e m m u n g s b il d u n g e n b e s o n d e r s d e s h a u td ru se n s y s te m : Berl. K lin . W c h n s c h r . 21369:1373 J u ly 1911.

Fig. 4 • Full fa c e a fte r six m o n th s' w e a rin g o f the d e n tu re s

4. E ve re tt, F. S . , a n d o th e rs. A n h y d r o t i c e c t o d e rm c d y s p l a s ia w ith a n o d o n t ia : a s t u d y o f t w o fa m ilie s J . A . D . A . 44:173 F e b . 1952.

M c C A L L — S Z M Y D . . . V O L U M E 61, N O V E M B E R I960 • 41/559

5. Upshaw, B. Y., and M o n tg o m e ry , H . H e re d ita ry a n h y d ro tic e c to d e rm a l d ysplasia ; c lin ic a l and p a th o ­ lo g ic study. A rc h . D e rm at. & Syph. 60:1170 Dec. 1949. 6. Bowen, R. H e re d ita ry e c to d e rm a l dysplasia o f the a n h y d ro tic ty p e . South. M . J . 50:1018 A u q . 1957. 7. Stiles, F. C ., and W ie r, J. R. E ctoderm al dysplasia presentin g as fe v e r o f unknown o rig in . J .A .M .A . 158:1432 A u g . 20, 1955. 8. C o le . H . N ., and others. C o n g e n ita l ca ta ra c ts in sisters w ith c o n g e n ita l e cto d e rm a l d ysplasia . J . A .M A . 129:723 N ov. 10, 1945. 9. C la rk e , R. E., and M cC ance , R. A . F a m ilia l sexlinked e c to d e rm a l dysplasia w ith in c o m p le te form s. A rc h . Dis. C h ild h o o d 9:34 Feb. 1934. 10. G a tto , I. Ellis-van C re ve ld P a edia t. A c ta 6:437 N o v. 1951.

syndrom e.

H e lv e t.

11. M etson, B. F .p and W illia m s , B. K. H e re d ita ry e c to d e rm a l dysplasia o f a n h y d ro tic ty p e . J . Pediat. 40:303 M arch 1952. 12. Bruno, F. E., and E n gelh ardt, H . T. H e re d ita ry e c to d e rm a l d ysplasia . A n n . In t. M e d . 20:140 Jan. 1944. 13. Thom a, K. H . O ra l p a th o lo g y . St. Louis, C . V. M osby C o ., 1950, p . 171. 14. G ra n t, R., and Falls, H . F. A n o d o n tia : r e p o rt o f a case associated w ith e c to d e rm a l dysplasia o f the a n h y d ro tic ty p e . A m . J. O rth o d o n t. & O ra l Surq. (o ra l surg. sect.) 30:661 N o v. 1944. 15. I p p o lito , A n th o n y . E ctoderm al d ysplasia . J .A .D .A . 58:92 A p r il 1959. 16. Finn, S. S. C lin ic a l p e d o d o n tic s . P h ila d e lp h ia , W . B. Saunders C o ., 1957, p. 425. 17. C ohen, M ich a e l M . P e d ia tric d e n tis try . St. Louis, C . V. M osby C o ., 1957, p. 530.

C lin ica l e va lu atio n o f u ltra so n ic s c a lin g

C la ren ce M . M c C a ll, J r.,* D .M .D ., M .S ., and L u cia n S zm yd ,* D .M .D ., M .S ., B rooks A ir F o r ce Base, T ex a s

A

clin ical eva lu a tion

o f th e ultrasonic

scaling tec h n ic was carried ou t on 240 m ilitary su b jects. T h e u ltrasonic tech n ic was fo u n d to be as e ffe c tiv e as th e c o n ­ ven tion a l scaling m e th o d in th e rem ova l o f calculus, w hile th e co n v en tio n a l oral prop h yla xis tech n ic was superior to the ultrasonic m e th o d in th e rem o v a l o f stain. M o s t o f th e su b jects p r efe rr e d th e ultra­ son ic m e th o d o f oral p rop h yla xis o v er the co n v en tio n a l scaling tech n ic. N o statis­ tical d iffe r en ce

in

to o th

sensitivity

or

tissue traum a was n o te d b e tw e e n th e tw o scaling tech n ics.

Evaluation o f the ultrasonic scaling tech­ nic as an adjunct in oral prophylaxis is a problem now confronting the dental profession. Several investigators have re­ ported satisfactory results with the ultra­ sonic method. Zinner1 found no adverse

clinical symptoms in patients whose teeth were scaled with the ultrasonic instru­ ment. H e also noted a lower degree of root sensitivity and very little gingival hemorrhage. Johnson and Wilson2 scaled the teeth o f ten clinical patients with heavy calculus. Th ey demonstrated that ultrasonic energy could successfully re­ move stain and calculus. Wilson3 re­ ported that patients expressed a prefer­ ence for the ultrasonic method. H e noted that the ultrasonic technic is not nearly so effective as the rubber polishing cup for removing stain. Mallernee4 studied the effect o f ultrasonic energy on the periodontal membrane, alveolar bone and gingivae o f dogs. H e observed no injury to these tissues. Additional clinical and experimental research is necessary to supplement these preliminary reports before accepting the ultrasonic method for routine oral pro­ phylaxis. T h e purpose o f this investiga­ tion was to compare the clinical effec-