THE TREATMENT SOME SKELETAL DEFORMITIES · taneous hand deformities, due to bilateral radius defect. Patients with a congenital defect of the femur may be very well helped with a

POSTGRAD. MED. J. (I963), 39, 67

THE TREATMENT OF SOMESKELETAL DEFORMITIES

Alex Simpson Smith Memorial Lecture delivered at The Hospitalfor Sick Children, London, W.C.i,September 6, 1962

M. SULAMAA, M.D.Department of Pediatric Surgery, Children's Clinic, University of Helsinki, Finland

IN infants and children there are some rather rareskeletal deformities which are not usually treatedby pediatric surgeons and therefore often not treatedearly enough. As pediatric surgeons have the bestopportunities to undertake the care of crippledinfants, all diseases and malformations requiringsurgical treatment in early infancy should be in-cluded within the scope of pediatric surgery.The very old Chinese custom of binding female

feet, and the ritual habits of certain native tribesinducing artificial towerheads, are examples of thefact that treatment in early infancy is capable ofmoulding the shape of bones during growth. Thisfact can be made use of in the treatment of certaincongenital skeletal deformities. The causes of

wrong growth or hindrance of normal growthshould also be eliminated as early as possible.

In this lecture I will deal with the treatment ofthree groups of skeletal deformities: craniosyno-stosis, thoracic deformities and congenital bonedefects of the limbs. Table i shows the materialtreated at our hospital.

CraniosynostosisThe flat bones of the skull are joined by sutures

capable of growing like the epiphyseal cartilages inthe long bones (Laitinen, 1956). Precociousbony union ofthe sutures is the cause of craniosyno-stosis. It results in various deformities of theskull observable soon after birth or detected later

CRANIOSYNOSTOSISScaphocephaly 49Brachycephaly 20Trigonocephaly 7Crouzon syndrome 4Apert syndrome 2

TOTAL 82'ERRORS OF SEGMENTATION'

THORACIC DEFORMITIES OF EXTREMITIESFunnel Chest 222 CONGENITAL DEFECT OF:Pigeon Chest 15C. High Scapula 21HUE S 3Costal Defects 13 HUMERUS 3Sternal Fissure 3

ULNA 3TOTAL 274

RADIUS 30

FEMUR 3

TIBIA 3

-FIBULA 4

TOTAL 46

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68 POSTGRADUATE MEDICAL JOURNAL February I963

on account of symptoms caused by increased in-tracranial pressure.The long and narrow scaphocephalic skull

(Fig. i) is due to synostosis of the sagittal suture.

IX

FIG. i.-Scaphocephaly.

.

FIG. 2.-Brachycephaly.

The short and broad, high brachycephalic ' towerhead ' (Fig. :9 is caused by coronal synostosis.As the result of the premature synostosis a dis-proportion develops between the healthy, rapidly-growing brain and the unyielding constricted skull.This will result in elevation of the intracranialpressure, commonest in combination with thebrachycephalic type of craniosynostosis. Per-sistent pressure may result in retardation of mentaland even physical development, The destruction

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Age in yearsFIG. 3.-Increase in weight of brain

from birth to fifteenth year of life.Height of step indicates half-year'sgrowth. (According to Scammonand Dunn.)

of the optic nerve, leading to atrophy and blind-ness, is one of the severest complications.As the growth of both the brain and the skull is

maximal in the first year of life and almost completeat the age of four (Fig. 3) treatment at an early agegives the best cosmetic results and affords the bestguarantee against any possible brain damage, too.In cases which have been diagnosed later onaccount of the symptoms caused by increasedintracranial pressure the results are also satis-factory (Fig. 4).

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FIG. 4.-A case of coronal synostosis.The coronal suture was openedbecause of increased intracranialpressure. Note the marked widen-ing of the artificial suture.

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SULAMAA: The Treatment of Some Skeletal Deformities

Trigonocephaly due to antenatal synostosis of thefrontal suture is usually not accompanied by brainsymptoms. As the deformity tends to diminishduring growth, there is no indication for operativetreatment.

Hereditary craniofacial sysnostosis, the Crouzonsyndrome (Fig. 5), usually leads to pressuresymptoms sooner or later. Early treatment isindicated.

In Apert's syndrome there is syndactylism (Fig.6) combined with coronal craniosynostosis (Fig. 7).Opening of the coronal sutures and plastic opera-tions for syndactylism have given satisfactoryresults in our two patients.We have treated 82 cases of craniosynostosis

by the method recommended by Ingraham andMatson (I954). The results have been satis-factory except for one death due to postoperativeshock. One patient, a boy of 24, had been totallyblind for two weeks. The fused coronal andsagittal sutures were opened. After the operationthe boy's sight little-by-little returned to normal.The post-operative improvement itself is very goodin early cases, but later less good. Fig. 8 shows achild with typical scaphocephaly at the age of Imonth and Fig. 9 the same case after one and a halfyears. Thus, during the period of maximalgrowth, i.e. before the age of 4, we consider surgeryindicated in every case of craniosynostosis excepttrigonocephaly. At a later age an operation isindicated only if there are pressure symptoms,because cosmetically it has little effect on the

FIG. 5.-Crouzon syndrome (hereditarycraniofacial sysnostosis).

deformity and because attempts to ameliorate theretarded mental status by operation at school agehave given disappointing results.

Thoracic DeformitiesDeformities of the thoracic wall are rather

common in infants and children. Small de-formities are unimportant and the indications for

FIGS. 6 and 7.-Apert's syndrome (syndactylism with coronal craniosynostosis).

February I963 69

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POSTGRADUATE MEDICAL JOURNAL

FIG. 8.-Scaphocephaly at age one month.

FIG. 9.-Scaphocephaly at age iI4 years.

therapy are only cosmetic or psychological. De-formities involving a great part of the chest walland sometimes also anomalies of separate bonesmay to a greater or lesser extent disturb thefunction of the chest organs. Our experienceclearly shows that severe symptoms are rare.Therefore we think that operative correction isindicated only if the treatment is relatively simpleand free of risk.

Congenital high scapula (Sprengel's deformity),is often combined with vertebral and costalanomalies. A limited abduction of the extremityis common and the deformity is cosmetically dis-tressing. Operation by the method of Schrock(1926) is easy and gives satisfactory results(Fig. Io).

In antero-lateral defect of the upper ribs thepectoralis muscles are also missing or rudimentary

(Fig. ii). The substitution of bone transplantsfor the missing ribs is recommnended, but seldomnecessary, because the cosmetic effect is usuallydue to missing muscles only.

In sternal fissure the upper thoracic apertureis wide, allowing the heart to bulge up to the neckwhen the child is crying (Fig. I2). Because ofcardio-respiratory distress urgent treatment isneeded. By freeing the sternal attachments ofboth sternocleidomastoid muscles and suturingthem together in the midline (Fig. 13) displace-ment of the heart is effectively prevented.A protruding sternum, or pigeon breast, does not

necessitate operation. Marked deformity hassometimes been considered an indication if theoperation is free of risk. In cases where the chestwall is depressed on both sides of the protrudingsternum cardio-respiratory symptoms tend to be

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February I963 SULAMAA: The Treatment of Some Skeletal Deformities 71

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FIG. Io.-Result of Schrock's operationfor congenital high scapula.

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FIG. i I.-Antero-lateral defect of the upper ribs.

more marked (Fig. 14). In such cases we havemade a subperichondral resection of the depressedcostal cartilages. The perichondria are thenelevated and fixed to steel struts, which perforatethe sternum medially and are fixed laterally to theribs at the normal level (Fig. I5).The indications for the repair offunnel chest, the

commonest thoracic deformity, are nearly alwaysrelative. The operation must therefore be simpleand not attended by too much risk. Our operativemethod earlier consisted of subperichondral re-section of the deformed cartilages, retrosternaldissection of the diaphragmatic attachments andmaintenance of the elevated position of the

FIG. I 2.-Sternal fissure,

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FIG. 13.-Correction of sternal fissure.

sternum with a steel strut passed through thesternum from side to side (Figs. i6 and I7). Aswe were practically never able to find a realretrosternal ligament and as there is always acertain risk of pleural damage, we further modifiedour method by abandoning retrosternal dissection.Our last 70 cases have been treated by this sim-plified method without complications. At followup the result has been considered good in 8o%and satisfactory in 20% of cases.

Congenital Bone Defects of the LimbsThe congenital deformities of the limbs which

are caused by primary defects of the bones are

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FIG. 14.-Pigeon breast.

FIG. I 5.-Correction of pigeon breastdeformity by resection of costalcartilages and insertion of steelstruts.

F. ..........6........

FIGS. I6 and 17.-Funnel chest and its correction. 9

supposed to be due to ' errors of segmentation ' asa result of accidents during early embryonicdevelopment. Until now they have been ratherrare but in recent years their incidence hassuddenly risen in some countries. Thalidomide-containing drugs ingested by the mothers duringthe early stages of gestation are supposed to be thecause of this increase.The skeletal deformities due to bone defects

mainly follow longitudinal divisions of the ftetallimb. It is often the case that correspondingparts of the intermediate and terminal segmentsare lacking.The therapeutic problems in such cases are

difficult. They will be discussed in the followingin the light of our clinical experiences and ofanimal experimentation.

In congenital defect of the fibula (Fig. i8) the

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FIG. i8.-Congenital defect of thefibula.

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FIG. I9.-Correction of defect of the fibula.

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I-;FIG. 20.-Correction of defect of the fibula.

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FIG. 2I.-Correction of defect of the tibia.

lateral part of the foot is lacking. The ankle isdislocated to the extreme valgus-equinus position.The tibia is both short and angulated. Our fourpatients have been treated with early tenotomiesand osteotomies with satisfactory results (Figs. I9and 20). During the first post-operative yearsthere has not been any progressive tendency toshortening of the affected legs.A congenital defect of the tibia may be total or

partial. One of our three patients had a uni-lateral total, one a unilateral partial and one abilateral partial defect. The varus position wascorrected and the ankle stabilized by an operationin which the distal end of the fibula was attachedto a drill hole made in the tarsal cartilages (Fig.2I).

Subsequent attempts to join the upper end ofthe fibula to the epiphysis of the femur in the caseof total absence of the tibia resulted in only partialsuccess (Figs. 22 and 23). In partial defect,osteosynthesis between the rudiment of the tibia

FiGs. 22 and 23.-Total absence of the tibia.

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FIG. 24.-Partial bilateral defect of the tibia.

FIGS. 25 and 26.-The final situation in the same case as in Fig. 24.

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FIG. 27.-Congenital defect of the femur

and the osteotomized fibula succeeded well (Figs.24 and 25). The girl shown in the figures hassince been capable of walking well (Fig. 26).She can also dress herself in spite of the simul-taneous hand deformities, due to bilateral radiusdefect.

Patients with a congenital defect of the femurmay be very well helped with a prosthesis only(Grob, 1957). In one of our three cases, with analmost total defect (Fig. 27), we tried to replace thelacking bone with a fibular autograft. Thegraft survived but showed no evidence of con-tinuous longitudinal growth. In spite of markedshortening of the affected limb, however, the boyis walking and running well with his prosthesis(Fig. 28).

Aplasia and hypoplasia of the radius are thecommonest skeletal defects of the limbs. Ourmaterial consists of 30 cases. Attempts to replacethe missing bone with growing epiphyseal auto-grafts have met with only moderate success (Starr,1945; Riordan, 1955; Heikel, 1959). Our firstmethod of operation, using epiphyseal transplantsof the metacarpal bones (Fig. 29), did not giveenough stability to the wrist (Fig. 30). Later, weadopted a new method, which consists of transposi-tion of the ulnar head to a more radial position

FIG. 28.-Result of correction of defect of femur with autograft.



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FIG. 29.-Aplasia and hypo-plasia of the radius.Operation using epi-physeal transplants ofmetacarpal bone.

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FIG. 3 i.-Aplasia and hypoplasia of the radius. Trans-position of ulnar head.

FIG. 32.-Result of operation inFig. 31.

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FIG. 33.-Defect of ulnacombined with cubitalsynostosis.

against the carpal cartilages (Fig. 3 I). It has givenmore satisfactory results with regard to both thecosmetic appearance and the function (Fig. 32).Our three cases of defects of the ulna were all

bilateral and combined with cubital synostosis(Fig. 33). The corresponding fingers weremissing. No treatment has been considerednecessary.

Subtotal defect of the humerus with correspond-ing peripheral defects is a type of phocomelia.One of our three cases had a unilateral, the others

bilateral defects. One of these also had a simul-taneous bilateral radius defect. The rudimentaryupper extremity floats unstably in the axillaryregion. In order to save as much as possible of theupper extremity function we have tried to producea more stable union between the rudimentary limband the trunk. We freed the sternal end and themiddle part of the clavicle and turned it laterallyto the shaft of the extremity. An osteotomy be-hind the acromion was necessary to achieve thisin the oldest child. The clavicle was fixed to therudiments of the humerus and to the bone of theforearm with wire cerclage (Fig. 34). Thecapacity of the patients to use their hands hasclearly improved post-operatively and the childrencould now get their hands together in the midline(Fig. 35).A patient with pure segmental malformation of

the forearms and legs of the type described onlyby Nivergelt, has been seen at our hospital (Figs.36 and 37). To correct the extreme valgusposition, a wedge-shaped resection was performedon both the tibia and the fibula. The epiphyseallines were turned to a more normal position (Fig.38). The X-ray four years later shows that therestill exists a valgus position of 40 degrees, andtherefore a further operation of the same type isplanned.

The Growth of Epiphyseal and Sutural Graftsin Animal ExperimentsThe question of whether it is possible to increase

the growth of a hypoplastic bone or to inducesignificant growth in a transplanted bone is un-solved. In the cases presented above we haveobtained some positive results in clinical material.These attempts are based partly on previouspublications on this subject, partly on experi-mental studies on animals. Part of this experi-

FIG. 34.-Subtotal defect of the humerus before and after operation.



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February 1963 SULAMAA: The Treatment of Some Skeletal Deformities 79

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FIG. 35.-Result of operation inFig. 34.

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FIG. 36.-Pure segmental malforna-tion of the forearms and legs.

FIG. 37.-Pure segmental malformation of the fore-armns and legs.

44;

FIG. 38.-Operation on case in Figs. 36 and 37.

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i h l;. ar-U--- p] ant a tI )n f*t ro x i(1j1 1gfib)l1:L< ('RiTr1 Eh St'S O f i t

mental work, comprising over ioo experiments ongrowing dogs, rabbits, guinea pigs and rats, wasperformed by my coworker Dr. Ryoppy.* In thefollowing some examples of our experiments arepresented.The proximal fibular epiphyses of a young dog

with a piece of adjoining metaphysis are trans-planted from one side to another, thus changingthe site only (Fig. 39). In the medullary cavitythere is a needle marking the distal point of thegraft. In three months the longitudinal growth ofthe graft is 30 mm. on the right and I7 mm. on theleft. The original length of the graft being 8 mm.,the growth has been, respectively, about fourtimes and twice the original length. The form ofthe right fibula is quite normal and on both sidesthe appositional growth has been normal.

* This part of the experimental material, from whichthe pictures shown here are also chosen, will bepublished later by Dr. Ry6ppy.

Amputation of the humerus has been performedon a newborn rat (Fig. 40). The distal end of theantebrachium, comprising the epiphyses of theradius and the ulna, has been fixed to the stumpof the humerus with a needle. The growth of thegraft is evident. In the histological picture thestructures of the graft and the host area are seen.There is formation of new bone. The medullarycavities of the graft and the humerus areseparated.

Only autogenous transplants have given positiveresults. Theref6re there is no ready source ofgrafting material in the human subject. In asearch for new material for transplantationdifferent animal experiments were performed inwhich cranial sutures were used as grafts. Themembranous bones of the skull grow by prolifera-tion of the sutural connective tissue and secondaryapposition of bone at the suture. This process isparallel to the endochondral growth of the longbones.

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FIG. 40.-Left: the humerus and the antebrachium of a new-born rat. In themiddle: the distal end of the antebrachium, which was amputated inmetaphysis and fixed to the stump of the amputated humerus is seen at thelower end of the humerus. Right: the histological preparation of the graft.

The type of coronal sutural graft, which hasbeen used in the experiments on the rabbit, is seenin Fig. 41. Such a graft, when reimplanted in itsoriginal site, grows in favourable cases in the sameproportion as the other parts of the suture.Growth is not very marked, however, becausethe growth of the skull stops relatively early andbecause it is very difficult to experiment withnewborn animals.But such a sutural graft, when transplanted to

the site- of a removed proximal fibular epiphysis,continues its growth in the same proportion as thecorresponding fibular epiphysis.

FIG. 41.-Coronal sutural graft.

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82 POSTGRADUATE MEDICAL JOURNAL February I963

4 11tIfi-tit

.FIG. 42 (Coi(nil sutural gr-aft on a d gn

Fig. 42 shows an experiment on a dog. Thesutural graft has been fixed in the place of aproximal fibular end removed extraperiosteally.The growth of the graft in three months is two anda half times the original length, corresponding toto 25% of the growth of the leg. In the lowvoltage X-ray of the specimen at the right, thesutural line is visible. At the extreme right of thepicture the proximal end of the fibula of the con-trol side is seen.

Fig. 43 shows an experinient on a rabbit. Onthe right side there is the same type of graft as inthe previous case. The removed right fibularproximal epiphysis is transplanted to the cor-responding place on the left side. Both the

sutural and the epiphyseal graft have grown tothree times their original length, correspondingto 50% of the growth of the leg during the sametime.

It seems evident that the capacity for growthdepends on local factors induced by the host area.

Transplantation frequently fails, however.Many questions concerning the factors contri-buting to the successful results are still un-answered. No definite suggestions for clinicalapplication of these results can yet be given.The treatment of congenital bone defects withgrafts capable of growth, however, seems to bewithin the bounds of possibility.

REFERENCESAsp, K., and SULAMAA, M. (1959): On Rare Congenital Deformities of the Thoracic Wall, Acta chir. scand., II8, 392.GROB, M. (1957): Lehrbuch d. Kinderchirurgie. Stuttgart: Thieme.HEIKEL, H. (1959): Aplasia of the Radius, Acta orthop. scand., SuppI. 39.INGRAHAM, F. D., and MATSON, D. D. (1954): Neurosurgery of Infancy and Childhood. Springfield, Illinois: Charles

C Thomas.LAITINEN, L. (1956): Craniosynostosis, Ann. Padiat. Fenn., Suppl. 6.LENz, W., PFEIFFER, R. A., KosENow, W., and HAYMAN, D. J. (I962): Thalidomide and Congenital Abnormalities

(correspondence), Lancet, i, 45.MCBRIDE, W. G. (I96I): Thalidomide and Congenital Abnormalities (correspondence), Ibid., ii, 1358.PALTIA, V., PARKKULAINEN, K. V., SULAMAA, M., and WALLGREN, G. R. (1959): Operative Technique in Funnel Chest,Acta. chir. scand., II6, 90.RIORDAN, D. C. (I955): Treatment of Radial Club Hand, Y. Bone Jt. Surg., 37, 515.SCHROCK, R. D. (1926): Congenital Elevation of the Scapula, Ibid., 8, 207.STARR, D. E. (1945): Congenital Absence of the Radius: A Method of Surgical Correction, Ibid., 27, 572.

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THE TREATMENT SOME SKELETAL DEFORMITIES · taneous hand deformities, due to bilateral radius defect. Patients with a congenital defect of the femur may be very well helped with a