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THE TREATMENT of SOME SKELETAL DEFORMITIES Alex Simpson Smith Memorial Lecture Delivered at the Hospitalfor Sick Children, London, W.C.I, September 6, 1962 M

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THE TREATMENT of SOME SKELETAL DEFORMITIES Alex Simpson Smith Memorial Lecture Delivered at the Hospitalfor Sick Children, London, W.C.I, September 6, 1962 M Postgrad Med J: first published as 10.1136/pgmj.39.448.67 on 1 February 1963. Downloaded from POSTGRAD. MED. J. (I963), 39, 67 THE TREATMENT OF SOME SKELETAL 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 rare wrong growth or hindrance of normal growth skeletal deformities which are not usually treated should also be eliminated as early as possible. bypediatric surgeons and therefore often nottreated In this lecture I will deal with the treatment of early enough. As pediatric surgeons have the best three groups of skeletal deformities: craniosyno- opportunities to undertake the care of crippled stosis, thoracic deformities and congenital bone infants, all diseases and malformations requiring defects of the limbs. Table i shows the material surgical treatment in early infancy should be in- treated at our hospital. cluded within the scope of pediatric surgery. The very old Chinese custom of binding female Craniosynostosis feet, and the ritual habits of certain native tribes The flat bones of the skull are joined by sutures inducing artificial towerheads, are examples of the capable of growing like the epiphyseal cartilages in fact that treatment in early infancy is capable of the long bones (Laitinen, 1956). Precocious by copyright. moulding the shape of bones during growth. This bony union ofthe sutures isthe cause ofcraniosyno- fact can be made use of in the treatment of certain stosis. It results in various deformities of the congenital skeletal deformities. The causes of skull observable soon after birth or detected later CRANIOSYNOSTOSIS Scaphocephaly 49 Brachycephaly 20 Trigonocephaly 7 Crouzon syndrome 4 Apert syndrome 2 http://pmj.bmj.com/ TOTAL 82 'ERRORS OF SEGMENTATION' THORACIC DEFORMITIES OF EXTREMITIES Funnel Chest 222 CONGENITAL DEFECT OF: Pigeon Chest 15 C. High Scapula S 3 Costal Defects 21HUE13 HUMERUS 3 Sternal Fissure 3 ULNA 3 TOTAL 274 on October 1, 2021 by guest. Protected RADIUS 30 FEMUR 3 TIBIA 3 -FIBULA 4 TOTAL 46 Postgrad Med J: first published as 10.1136/pgmj.39.448.67 on 1 February 1963. Downloaded from 68 POSTGRADUATE MEDICAL JOURNAL February I963 on account of symptoms caused by increased in- Off tracranial pressure. The long and narrow scaphocephalic skull (Fig. i) is due to synostosis of the sagittal suture. 'DIanfllate 1AW lieD~~~~~~~~~~~~~~~~~~l SIA{.00n00 4" IX Age in years FIG. 3.-Increase in weight of brain from birth to fifteenth year of life. Height of step indicates half-year's growth. (According to Scammon FIG. i.-Scaphocephaly. and Dunn.) of the optic nerve, leading to atrophy and blind-by copyright. 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 complete at the age of four (Fig. 3) treatment at an early age gives the best cosmetic results and affords the best guarantee against any possible brain damage, too. In cases which have been diagnosed later on account of the symptoms caused by increased intracranial pressure the results are also satis- factory (Fig. 4). http://pmj.bmj.com/ on October 1, 2021 by guest. Protected ...........:::....... ..|l::: e....... > FIG. 2.-Brachycephaly. R s........!.t 9.-. ..... The short and broad, high brachycephalic ' tower head ' (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 intracranial commonest in combination with the FIG. 4.-A case of coronal synostosis. pressure, The coronal suture was opened brachycephalic type of craniosynostosis. Per- because of increased intracranial sistent pressure may result in retardation of mental pressure. Note the marked widen- and even physical development, The destruction ing of the artificial suture. Postgrad Med J: first published as 10.1136/pgmj.39.448.67 on 1 February 1963. Downloaded from February I963 SULAMAA: The Treatment of Some Skeletal Deformities 69 Trigonocephaly due to antenatal synostosis of the frontal suture is usually not accompanied by brain symptoms. As the deformity tends to diminish during growth, there is no indication for operative treatment. Hereditary craniofacial sysnostosis, the Crouzon syndrome (Fig. 5), usually leads to pressure symptoms sooner or later. Early treatment is indicated. 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 satisfactory results in our two patients. We have treated 82 cases of craniosynostosis by the method recommended by Ingraham and Matson (I954). The results have been satis- factory except for one death due to postoperative shock. One patient, a boy of 24, had been totally blind for two weeks. The fused coronal and FIG. 5.-Crouzon syndrome (hereditary sagittal sutures were opened. After the operation craniofacial sysnostosis). the boy's sight little-by-little returned to normal. The post-operative improvement itself is very good in early cases, but later less good. Fig. 8 shows a deformity and because attempts to ameliorate the child with typical scaphocephaly at the age of I retarded mental status by operation at school age month and Fig. 9 the same case after one and a half have by copyright. years. Thus, during the period of maximal given disappointing results. growth, i.e. before the age of4, we consider surgery Thoracic Deformities indicated in every case of craniosynostosis except Deformities of trigonocephaly. At a later age an operation is the thoracic wall are rather indicated only if there are pressure symptoms, common in infants and children. Small de- because cosmetically it has little effect on the formities are unimportant and the indications for http://pmj.bmj.com/ on October 1, 2021 by guest. Protected FIGS.yl- 6., andI 7.-Apert's syndrome (syndactylism with coronal craniosynostosis). Postgrad Med J: first published as 10.1136/pgmj.39.448.67 on 1 February 1963. Downloaded from 70 POSTGRADUATE MEDICAL JOURNAL February I963 FIG. 8.-Scaphocephaly at age one month. by copyright. http://pmj.bmj.com/ FIG. 9.-Scaphocephaly at age iI4 years. therapy are only cosmetic or psychological. De- (Fig. ii). The substitution of bone transplants formities involving a great part of the chest wall for the missing ribs is recommnended, but seldom and sometimes also anomalies of separate bones necessary, because the cosmetic effect is usually may to a greater or lesser extent disturb the due to missing muscles only. on October 1, 2021 by guest. Protected function of the chest organs. Our experience In sternal fissure the upper thoracic aperture clearly shows that severe symptoms are rare. is wide, allowing the heart to bulge up to the neck Therefore we think that operative correction is when the child is crying (Fig. I2). Because of indicated only if the treatment is relatively simple cardio-respiratory distress urgent treatment is and free of risk. needed. By freeing the sternal attachments of Congenital high scapula (Sprengel's deformity), both sternocleidomastoid muscles and suturing is often combined with vertebral and costal them together in the midline (Fig. 13) displace- anomalies. A limited abduction of the extremity ment of the heart is effectively prevented. is common and the deformity is cosmetically dis- A protruding sternum, or pigeon breast, does not tressing. Operation by the method of Schrock necessitate operation. Marked deformity has (1926) is easy and gives satisfactory results sometimes been considered an indication if the (Fig. Io). operation is free of risk. In cases where the chest In antero-lateral defect of the upper ribs the wall is depressed on both sides of the protruding pectoralis muscles are also missing or rudimentary sternum cardio-respiratory symptoms tend to be Postgrad Med J: first published as 10.1136/pgmj.39.448.67 on 1 February 1963. Downloaded from February I963 SULAMAA: The Treatment of Some Skeletal Deformities 71 _M>+. ..fi.fa.,i f..................~~~~~~~~~~................. .. -B--. FIG. I 2.-Sternal fissure, FIG. Io.-Result of Schrock's operation for congenital high scapula. by copyright. N11 http://pmj.bmj.com/ ' I FIG. 13.-Correction of sternal fissure. FIG. i I.-Antero-lateral defect of the upper ribs. more marked (Fig. 14). In such cases we have sternum with a steel strut passed through the on October 1, 2021 by guest. Protected made a subperichondral resection of the depressed sternum from side to side (Figs. i6 and I7). As costal cartilages. The perichondria are then we were practically never able to find a real elevated and fixed to steel struts, which perforate retrosternal ligament and as there is always a the sternum medially and are fixed laterally to the certain risk of pleural damage, we further modified ribs at the normal level (Fig. I5). our method by abandoning retrosternal dissection. The indications for the repair offunnel chest, the Our last 70 cases have been treated by this sim- commonest thoracic deformity, are nearly always plified method without complications. At follow relative. The operation must therefore be simple up the result has been considered good in 8o% and not attended by too much risk. Our operative and satisfactory in 20% of cases. method earlier consisted of subperichondral re- section of the deformed cartilages, retrosternal Congenital Bone Defects of the Limbs dissection of the diaphragmatic attachments and The congenital deformities of the limbs which maintenance of the elevated position of the are caused by primary defects of the bones are Postgrad Med J: first published as 10.1136/pgmj.39.448.67 on 1 February 1963. Downloaded from 72 POSTGRADUATE MEDICAL JOURNAL February I963 FIG.
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