Letterer-Siwe type of

re ticuloendo t heliosis

Case report on orthodontic interrelationship

W. BURNIE BUNCH, I).l).S., hl.S.1).

Jaoksonuille, Fla.

IN *JGI,Y, 1951, when the boy shown in P’igs. I to S was 15 months of age, he was found to have an cosinophilic granuloma. of the left tibia. When hc was hospit,alized, examination revealed many areas of osteolytic bone changes, and the diagnosis of rcticulocndotheliosis was made. The patient received s-rag therapy, nitrogen mustard, Xureomycin. eor.t,isonc, and antibiotics over a long period of time. As the disease progrcsscd, the boy was again hospitalized in 1952, when a diagnosis of J&trrcr-Siwc type of reticuloendothcliosis was made. In 1954 he developed diabetes insipidus, which responded to s-ray treatment. J)uring the course of t,he disease and therapy, the mandible was completclv invaded and most of the deciduous teeth wcw extracted. There was no rcgcn- cration of t,he mandible. When the patient was 12 pears 3 months of age the diagnosis of inactive Tdctterer-Siwc type ot’ rrticuloendotheliosis was confirmed.

This patient was start,cd on s-ray therapy in duly, 1951, at t,he age of 15 mont,hs, and received s-ray treatment IO various parts of the body. The right and left sides of the jaws were given 600 T. In *July, 1952, he was given 950 Y to each side of the neck; the mandible also received some x-ray treatment at that time. JIuring tho autumn of 1952. he again received x-ray t,herapy, but none was directed over the maxilla or mandible. The last s-ray therapy, given in 1954, was to the frontal arca and also to the pituitary fossa. The fossa received 650 r on each side. These treatments were given with a 250 kv. machine and a thorium filter which gave maximum protection to the skin and underlying soft tissue. I.

A recent physical examination reveals a short boy, of less-than-average stature, aged 32 years 3 months. There is a marked droop of the right shoulder, which is lower than the left. Both shoulders are rounded. The cervical area is

Letterer-Siwe type of reticuloendoth~eliosis I) I 7

Fig. 1. Fig. 2

shorter than is usually scow. A dcfittitc ~~X~~~l~tl~illl~lOS is prcsfttt. (‘rat&t1 pt’o- portion is gcnwally good. The chest is clear, and the heart and abdomen are normal. The trstivlcs show matttratiott, and pnhic haiy is ptwcnt. Thn estremi- tic+ arc notwtal at this tinto.

1 lospital clinical discussiott wttwt2titig tlw ohjwt ivtrs 0I’ cbstlic4 ic: and i’tttt(*- 1 ional c*twtiofacial itnptwvcntent to 1~~ gained through possihlv ])iastic+ wpair 01 t Itt> tltatidihlc icld to art ot~thodonti~ consultation ;rncl walua.tion.

‘l’lrc~ l)oy sl~ows cstt*(~tw t~ti~t~o~ttitthiit. Hot II maxillary and t~~at~dihttlat~ itIt- leropostt~tkt~ atttl latt~ral ditncttsiorts are ~rratkcclly dcficicrtl. The chin is deviated to the left. The infrahyoid muscle groups, actin, 0 strongly, deflect the matrdit~lc

9 1 8 Bunch

Fig. Z. Fig. 6.

Fig i. Fig. 8

Figs. 5 to 8. Patient I,. E., ngcd 12 yonrs 0 Inollths.

and hyoid posteriorly and inferiorly. The hyoid hone could barcl~- be I)alpatcti low over the sternum. Perioral musculature is tensed.

The maxilla is diminutive, poorly formed, and relatively fiat. l’oorly formed rudimentary maxillary permanent teeth with short,encd roots and a fully formed mottled carious maxillary right central incisor are present. An anterior man- dibular segment is present which contains :1n incompletely -formed, partially erupted, mandibular right canine and four fully formed mandibular central and lateral incisors. X-ray examination, however, shows the root formations

Fig. 9. Fig. 10.

Figs. 9 and 10. Patient L. E., aged 12 years 3 months. Models showing dental and cranio- facial relationship.

of these teeth to bc deficient (Figs. 9 to 12). Thcsc four teeth, lying in a hori- zontal plane, are contained in a segment that is loose except for a fibrous con- nection and sequestered from the poorly formed mandible. Jlandibular lateral and antcroposterior growth is markedly restrictctl.

At the time of the recent examination poor oral hygiene and halitosis wcrc evidenced, but there were no lesions of the oral I~UCOLIS membrane. The hard palate is narrowed laterally and ;lnteropost,c~~iorl!-. The soft palate appears to rise and fall during function. The oral pharynx at, rest is apparently obliterated by approximation of the tongue, soft IlalaIe, ;~ntl pharl-ngeal pillars.

90 definite glenoid fossac, mandibular hotly, mmi, temporomandibular joints, and maxillary sinuses are seen ~~oentgcno~rapllicallr (Figs. 11 and 12). Marked deformity of the maxilla and teeth, rctrobulhnr wmprcssion, improper

Fig. 11. Roentgenograms of Patient L. E., aged 12 pears 3 months.

vertical devc~lopnient of the mitldlc Cl’illli:Ll fossa, aid a pooi~l\- outlined sella turcka arc drmonstrated ~ocrrt,gerro~i~a~~Ilicall~ ( l?igs. 11 and 12) . The corl- dploid and coronoid pr~~ccuses of thcl vvutigial mandible, the glenoid fossil, and the t~mpo~orrrandibula~ joint cannot 1~ ~~a,lpatcd on cithcr side. IIowrvcr. the d&rite masseter and temporal contrirc~tion ran be felt, particularly on the right side. This woultl indicate a functional (fi hrous!) J)sc~ndotcm~~or~ornandilrrxlar joint on the infratcmporal surfaccl.

Jkfinite rnovrmc~nt is t’clt lIilEltfZl’i~l1~ antrrior to the place whcrc the tcrn- l)or~omandibala~ joint is customarily SPCII. Intraorally, irn oblique vcrtigial ‘ ‘ asccndinp r’amus ’ ’ can 1,~ palpated bilatc~rall,v; t.liis is more prominent on the right side than on the lcf’t. Iking mandibular ~~losu~, intraoral palpation rc- veals that, this “as~iidin~ r’anius’ ’ moves postcrkly and superiorly, apparently pivoting at its superior cud. The mandibular pcrmancnt central and lateral incisors are hinged upon the sequestered vestigial segment, of the alveolar process and follow the gvncral mandibular position durin g swallowing. The tongue is strong and evidently has a thrusting action. IM5ng swallowing the mandibular srgmclnt, incaluding the four incisors and caninr (Figs. 9 arrd IO), moves labi- ally, apparently as a result of tongue-thrusting, into a morv horizontal plant? than dons the rudirnent,ary mandible itself.

The tongue is strong, highly mobile, large, and wide, ant1 it overflows thcl rnandibular~ antvrGor* tret,lr. The floor of the rnotrt 11, l)ar?icular4y t,hc: myoh;r-oitl ry?gion as palpated from above, seems st nmg and functional during nlarrdibular tuovement in swallowing. The boccinat 01’ tuusc~lr Iil~liS ST rmgt.h hit apparcntl! is I’un~tional. Masticatioll is ac~c~orn~~lislr(~~l t)>- St roiig lolrguc~ adion ag&iTlSt, tllcb

rudimentary mandible, the: “floor of t.11~ Irlouth, ” thcl maxilla, t lie anterior ina~i-

tlitnrlar section to which tllc fi1.c inaritlil~lilar anterior tcttrtll itl’f: ill t?iPllPtl, tllcl

~hccks, the lips, and adjacent, nmsculatnrc. I )t4initc mnsclc action of tlic 111ilS-

stlttlr t’illl 1)t: O~SCI*VN~ l)iiaterally ; :Ipparthntly it att,acllt+ to the rlldin~t~nt,ilr>- tltscending ralnils. Ttit: i~i;isSCtt~l~ originates From :I tltlcl’ Htl.Ht’tllllt’llt llpri tlltl xygomwtic arch intxlially wrd postt~rioi~l~~, appartwtly gaining its diagonal in- scrtion Iwal’ t hti I~~tPl’ill anti itlfci*ior bol~tlrr of tht> rntlimt~ntary “ asenntling ramus ” 1 .

Strong orbicularis oris function is dcfinitel~- observed, with the inferior portion being shortened and less functional. Facial musculature generally is flaccid. C+cnion is directed downward and forward markedly in an anteropos- terior vertical plane with the post,crior rim of the orbit.

Swallowing is accomplished with difficulty and is of a risceral t.ypc. The infrahyoid group of muscles contracts strongly with swallowing, the rudimen- tary chin point on the mandihlc moving more backward than downward during t.he swallowing action.

The hyoid is located slightly lx~low the rudimentary mandible in the median sagittal plane. The attachment from the hyoid to the sternum is extremely short because of the depressed position of thtl chin. ‘l’hc chin dcviatcs to the left approximately 1 cm.

Speech, while articulate, is constantly modula,tcd in tone, with projection of sound lacking. Ilowcver, it, is possible for this patient to speak very loudly. The impression is given that the patient feels he lacks control of his voice and t,hat his restraint in speaking is psychological.

The mother reports that the patient had a normal infancy until the age of 15 months, when an cosint~philic granuloma of the left tibia was discovered. She reports that bctwecn the ages of 3 and 5 ?-cars, the left leg was $5 to I/ii inch shorter than the right. The paGent has since outgrown this deformity. Ortho- pedic shoe lifts wcrc used until he was 8 years old, and the difference in leg length was graduall,v compensated for during growth.

Photographs of the boy to the age of 2 years show a round, babyish fact, oval in shape. Facial characteristics were balanced, with a possible slight man- dibular deviation to the left. Ilea\-y mental furrows are noted. Mild dcriation to the left, is evident, in photographs taken when the boy was about 3 years of age. Bctwccn the ages of 3 and 4 pears a marked mandibular retrusion became evident. I3sophthnlmos became evident at approximately 4 to 5 years of age. A shortcninp of the neck was seen at the age of I years. At t,he age of 6 peal3 mantlibnlar deviation to the left increased and was accompanied by increasing t~?co~~hthalmos. The mandibular retrusion became increasingly scvcrc bct,ween lhe ages of 6 and 8 years, with the fullness of the hccal areas following the adolcsrent adipose patt,crn. IDcviation of the chin is to the left, occasionall>- moving towartl the midline. A ~narlretl increase in the depth of the mental furrow and rctr’usion of the mental arca (tlrviatcd strongly to the left) and shortening of the infrahyoitl group becomes evident in photographs of the patient, between t,hc ag’cs of 9 and IO years. These symptoms increasttd toward the ag:c of 11 years, as shown in the photographs.

The mother reports that t,hc patient’s diet, is varied and includes all foods cxccpt those that remain had and fibrous after special preparation.

9 2 2 Bunch

His sixth grade t,eacher reports that now, as in the past (according to irr- formation from his pn~ious tcachcr*s) , the pitti(‘rrt is a. well adjusted 12-K student who is liked by a11 his classrjratps.

RECOMi\IENDATIONS

Plastic repair of the temporomandibular joint in this case would appear impractical for the following reasons :

1. Rudimentary underdevelopment structures are related. 2. Growth potential is not at a high (or even “normal”) level. 3. There is no irregular osseous stabilizing area on the infraternporal

surface of the temporal bone upon which to reconstruct a simulated temporomandibular joint with any reasonable expectation of satis- factory function.

4. At present there is a satisfactory simulated functional temporomarr- dibular joint.

5. The tensed perioral and infrahyoid musculature is displaced pas-

teriorly and J’unctionally shortened, which would interfere with and displace posteriorly any interposed mandibular implant.

6. This patient has acquired practical masticatory functional balance. 7. The postoperative dietary imbalance introduced by surgical inter-

ference could result in unsatisfactory altered masticatory function.

The following procedures appear- constructive :

1. Uental hygiene should be regular and more efYective. 2. Cleaning of the teeth and periodontal care should be done and re-

peated each four months. 3. Carious areas should be eliminated. 4. The carious protruding maxillary right central incisor should be ex-

tracted. 5. Speech therap)- should be given. 6. Surgical reconstruction of Ibe tcmporornandibular joints may prove

of dubious value. 7. At the age of 17 to 18 years, after removal of the mandibular arr-

terior section containing five attached permanent teeth, a surgical prosthesis or bone irnplant fixed fir.rnl)- anteriorly against the mandible might be considered practical. The size and placcrnent of this implant should be in harmony with the maxillary arch and within the adjusting lirnits of the perioral and infrahyoid muscu- lature. Possibly, to remain within tolerance of this musculature, performing this surgical procedure in t,wo stages might be construc- tive. Subsequent rnyofunctional training and speech therapy would be beneficial. Subsequent extraction OF all maxillary teeth and placing of a prosthetic restoration could sornewhat improve dental functional efficiency.

DISCUSSION

In viewing the over-all evaluation,l there may be interrelated a neurogenic deficiency dating from ea.rly rmhryonic de\-elopment when the time ’ ’ organizer hormones”” were operating in the emlqvonic stage.”

The freeing of perioral, masticatory, facial, suprahyoid, and inl’rahyoid muscles to encourage movement and thus aid growth of the organs of mastica- tion was considered but was not recommcndcd because this boy apparently lacked basic grow-t11 potential.

SUMMARY

Delaying plastic surgery repair until the patient reached the age of 17 to 18 years was suggested1 so that the growth potential would bc practically cs- hausted, cvcn though clinically signifirant mandibular improvement with growth is not anticipated or expected.

This appeared to bc primarily a case for plastic surgery rathrr than for orthodontic guidance. The history and physical examination, along with the presence of only rudimentary mandibular and maxillary development,, indicatctl that little maxillary and mandibular growth potential was prcscnt or had been present.

REFERENCES

I. Salzmann, J. A.: Personal Communication. 2. Willis, R. A. : The Borderland of Embryology and Pathology, London, 1938, Rutttsr\\ llrtll

& Co., Ltd., pp. 4-6. 2. TT’ilkins, Lawson: Diagnosis and Treatment of Endocrine Disorders in Chiltlhootl an,1

Adol~~enc~, ed. 2, Springfield, Ill., 1957, Charles C Thomas, p. 4.