2
663 operative series have been described from the USA, Japan, France, and England,"-" and enthusiasm for microvascular dissection has become so great as to replace other treatments for trigeminal neuralgia in some centres. Some scepticism remains because of occasional negative findings, difficulty in locating the compression, the finding of "arachnoiditis", occasional response failure, and the idea that patients selected for surgery may not be representative of the condition in general. The position has been clarified by a report from the Central Middlesex Hospital where Richards, Shawdon, and Illingworth18 found anatomical abnormalities in 46 of 52 patients with "idiopathic" trigeminal neuralgia. The most common finding was of a superior cerebellar arterial loop pressing on the nerve root entry zone, but there were also three clinically unsuspected tumours. Nerve decompression was followed by complete and prolonged pain relief. There is thus increasing evidence that the cause of trigeminal neur- algia is nerve compression, and, for young fit patients who wish to avoid unpleasant sensory symptoms, nerve decom- pression is probably the treatment of choice once medical treatment has failed. For the elderly or frail, injection or thermocoagulation remain valid alternatives. SURVIVAL IN CYSTIC FIBROSIS WHEN cystic fibrosis (CF) was first described nearly half a century ago1,2 survival was measured in months rather than years. Each successive decade has seen an improvement in life expectancy, and most patients in developed countries now survive childhood, the mean lifespan being between 20 and 25 years.3 A paper by Phelan and Hey4 reports that, while there has been a notable improvement in survival during the past 20 years, the factors causing this change are far from certain, and the prognosis differs greatly from centre to centre. Thus in the state of Victoria, Australia, where the incidence ofCF(l:2556 live births) is thought to be similar to that in England and Wales, mortality rates among children are two to three times lower in every age-group. An affected British newborn infant has an 80% chance of surviving until the age of 9, whereas in Victoria he or she would have the same chance of living to 20. The biggest difference in mortality is seen during early childhood, particularly the first year of life. The report does not indicate the proportions of infants in this age-group dying in the neonatal period from meconium ileus and its complications, on the one hand, and respiratory infection, on the other. In the older child and adult with CF, death is usually attributed to chronic respiratory infection, respiratory failure, and cor pulmonale. Excessive mucus secretion in the lungs produces obstructive changes and predisposes to 14. Jannetta PJ. Arterial compression of the trigeminal nerve at the pons in patients with trigeminal neuralgia. J Neurosurg 1967; 26: 159-62. 15 Provost J, Hardy J. Microchirurgie du trijumeau: anatomic functionelle. Neurochirurgie 1970; 16: 459-70. 16. Apfelbaum RI. Microvascular decompression for tic douloureux: Results. In: Brackmann DE, ed. Neurological surgery of the ear and skull base. New York: Raven Press, 1982: 175-80. 17. Adams CBT, Kaye AH, Teddy PJ. The treatment of trigeminal neuralgia by posterior fossa microsurgery. J Neurol Neurosurg Psychiatry 1982; 45: 1020-26. 18. Richards P, Shawdon H, Illingworth R. Operative findings of the cerebello-pontine angle in trigeminal neuralgia. J Neurol Neurosurg Psychiatry 1983; 46: 1098-101. 1 Fanconi G, Kehlinger E, Knauer C. Das Coeliakiesyndrom bei angeborener zysticher Pancreasfibromatose und Bronchiectasein. Wien Med Wschr 1936; 86: 753-56. 2. Andersen DH. Cystic fibrosis of pancreas and its relation to celiac disease: clinical and pathological study. Am J Dis Child 1938; 56: 344-99. 3 Warwick WJ. Prognosis for survival with cystic fibrosis: the effects of early diagnosis and cystic fibrosis center care. Acta Paediatr Scand 1982; 301: 27-31(suppl). 4. Phelan P, Hey E. Cystic fibrosis mortality in England and Wales and in Victoria, Australia 1976-80. Arch Dis Childh 1984; 59: 71-73. bronchial and peribronchial infection, the nature of which has changed over the years. Early accounts of post-mortem findings emphasised the importance of Staphylococcus aureus as a causal organism. 5,6 Pseudomonas aeruginosa has subsequently emerged as the predominant agent of chronic respiratory infection, being found in about 50% of all patients attending the Danish CF Centre in Copenhagen’ and in 70% of adults at the Brompton Hospital, London.8 At the time of death, Ps aeruginosa was cultured from 94% of the Danish patients.’ In persistent pseudomonas infections in CF patients, the organism undergoes a characteristic morphological change to a mucoid form, in which large amounts of an alginate are produced. Mucoid varieties of pseudomonas may be more resistant to phagocytosis and intracellular killing by leucocytes than other strains,9 although the mucoid change does not seem to increase bacterial resistance to antibiotics.10 The better prospects for the CF patient in Victoria are attributed by Phelan and Hey to the pattern of health care rather than to any environmental or climatic differences between Britain and Australia. They draw attention to the North American claim that management in a specialised centre is essential for optimum care, 1 and the data from Denmark12 reporting better survival among patients attending a specialised clinic compared with those looked after by their local paediatrician, "as happens for most children in England and Wales". Nevertheless the 80% survival figure for Danish patients is only 10 years (compared with 9 in England and Wales), rising to 12 years for those patients treated exclusively at the national CF centre, who comprise 77% of the total. Survival curves4,12 show that the probability of living to the age of 20 is 61 % for the Danish CF centre patients and 62% for England and Wales, and there seems to be little difference between the 50% survival figures for Denmark, England and Wales, and the USA and Canada. Whether centralisation of treatment would in itself substantially improve the prognosis remains to be shown, but the high mortality among young British infants leaves no room for complacency. Death from meconium ileus is now unusual in neonatal surgical centres and early diagnosis should ensure that most affected infants will receive prompt and adequate treatment for respiratory infections, whether at a special centre or at a district general hospital. The prevention of chronic lung disease poses a greater problem, since the underlying cause is poorly understood. However, Phelan and his colleagues have shown elsewhere that their patients have a remarkably low incidence of infection with Ps aeruginosa, which they suggest may be due to prophylactic treatment with inhaled gentamicin.13 The single most important factor influencing prognosis may be early 5. di Sant’Agnese PA, Andersen DH Celiac syndrome. iv. chemotherapy in infections of the respiratory tract associated with cystic fibrosis of the pancreas; observations with penicillin and drugs of the sulfonamide group with special reference to penicillin aerosol. Am J Dis Child 1946; 72: 17-61. 6. Bodian M, ed. Fibrocystic disease of the pancreas. London: Heinemann, 1952: 119-55. 7. Høiby N. Microbiology of lung infections in cystic fibrosis patients. Acta Paediatr Scand 1982; 301: 33-54(suppl). 8. Batten JC. Croonian lecture, Royal College of Physicians, London, 1983. 9. Baltimore RS, Mitchell M. Immunologic investigations of mucoid strains of Pseudomonas aeruginosa; comparison of susceptibility to opsonic antibody in mucoid and non-mucoid strains. J Infect Dis 1980; 141:238-47. 10. Makrowitz SM, Macrina FL, Phibbs PV. Antimicrobial susceptibility of mucoid Pseudomonas aeruginosa and their spontaneously occurring non-mucoid derivatives. J Antimicrob Chemother 1980; 6: 251-60. 11. Stern RC, Boat TF, Doershuk CF, Tucker AS, Primiano FO, Matthews LW. Course of cystic fibrosis in 95 patients. J Pediatr 1976; 89: 406-11. 12. Nielsen OH, Schiøtz PO. Cystic fibrosis in Denmark in the period 1945-81. Evaluation of centralised treatment. Acta Paediatr Scand 1982; 301: 107-19(suppl). 13. Phelan PD, Allan JL, Landau LI, Barnes GL Improved survival of patients with cystic fibrosis. Med J Austr 1979; i: 261-63.

SURVIVAL IN CYSTIC FIBROSIS

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operative series have been described from the USA, Japan,France, and England,"-" and enthusiasm for microvasculardissection has become so great as to replace other treatmentsfor trigeminal neuralgia in some centres. Some scepticismremains because of occasional negative findings, difficulty inlocating the compression, the finding of "arachnoiditis",occasional response failure, and the idea that patients selectedfor surgery may not be representative of the condition ingeneral. The position has been clarified by a report from theCentral Middlesex Hospital where Richards, Shawdon, andIllingworth18 found anatomical abnormalities in 46 of 52patients with "idiopathic" trigeminal neuralgia. The mostcommon finding was of a superior cerebellar arterial looppressing on the nerve root entry zone, but there were alsothree clinically unsuspected tumours. Nerve decompressionwas followed by complete and prolonged pain relief. There isthus increasing evidence that the cause of trigeminal neur-algia is nerve compression, and, for young fit patients whowish to avoid unpleasant sensory symptoms, nerve decom-pression is probably the treatment of choice once medicaltreatment has failed. For the elderly or frail, injection orthermocoagulation remain valid alternatives.

SURVIVAL IN CYSTIC FIBROSIS

WHEN cystic fibrosis (CF) was first described nearly half acentury ago1,2 survival was measured in months rather thanyears. Each successive decade has seen an improvement in lifeexpectancy, and most patients in developed countries nowsurvive childhood, the mean lifespan being between 20 and25 years.3 A paper by Phelan and Hey4 reports that, whilethere has been a notable improvement in survival during thepast 20 years, the factors causing this change are far fromcertain, and the prognosis differs greatly from centre tocentre. Thus in the state of Victoria, Australia, where theincidence ofCF(l:2556 live births) is thought to be similar tothat in England and Wales, mortality rates among childrenare two to three times lower in every age-group. An affectedBritish newborn infant has an 80% chance of surviving untilthe age of 9, whereas in Victoria he or she would have thesame chance of living to 20. The biggest difference in

mortality is seen during early childhood, particularly the firstyear of life. The report does not indicate the proportions ofinfants in this age-group dying in the neonatal period frommeconium ileus and its complications, on the one hand, andrespiratory infection, on the other.

In the older child and adult with CF, death is usuallyattributed to chronic respiratory infection, respiratoryfailure, and cor pulmonale. Excessive mucus secretion in thelungs produces obstructive changes and predisposes to

14. Jannetta PJ. Arterial compression of the trigeminal nerve at the pons in patients withtrigeminal neuralgia. J Neurosurg 1967; 26: 159-62.

15 Provost J, Hardy J. Microchirurgie du trijumeau: anatomic functionelle.

Neurochirurgie 1970; 16: 459-70.16. Apfelbaum RI. Microvascular decompression for tic douloureux: Results. In:

Brackmann DE, ed. Neurological surgery of the ear and skull base. New York:Raven Press, 1982: 175-80.

17. Adams CBT, Kaye AH, Teddy PJ. The treatment of trigeminal neuralgia by posteriorfossa microsurgery. J Neurol Neurosurg Psychiatry 1982; 45: 1020-26.

18. Richards P, Shawdon H, Illingworth R. Operative findings of the cerebello-pontineangle in trigeminal neuralgia. J Neurol Neurosurg Psychiatry 1983; 46: 1098-101.

1 Fanconi G, Kehlinger E, Knauer C. Das Coeliakiesyndrom bei angeborener zysticherPancreasfibromatose und Bronchiectasein. Wien Med Wschr 1936; 86: 753-56.

2. Andersen DH. Cystic fibrosis of pancreas and its relation to celiac disease: clinical andpathological study. Am J Dis Child 1938; 56: 344-99.

3 Warwick WJ. Prognosis for survival with cystic fibrosis: the effects of early diagnosisand cystic fibrosis center care. Acta Paediatr Scand 1982; 301: 27-31(suppl).

4. Phelan P, Hey E. Cystic fibrosis mortality in England and Wales and in Victoria,Australia 1976-80. Arch Dis Childh 1984; 59: 71-73.

bronchial and peribronchial infection, the nature of whichhas changed over the years. Early accounts of post-mortemfindings emphasised the importance of Staphylococcus aureusas a causal organism. 5,6 Pseudomonas aeruginosa has

subsequently emerged as the predominant agent of chronicrespiratory infection, being found in about 50% of all patientsattending the Danish CF Centre in Copenhagen’ and in 70%of adults at the Brompton Hospital, London.8 At the time ofdeath, Ps aeruginosa was cultured from 94% of the Danishpatients.’ In persistent pseudomonas infections in CF

patients, the organism undergoes a characteristic

morphological change to a mucoid form, in which largeamounts of an alginate are produced. Mucoid varieties ofpseudomonas may be more resistant to phagocytosis andintracellular killing by leucocytes than other strains,9although the mucoid change does not seem to increasebacterial resistance to antibiotics.10The better prospects for the CF patient in Victoria are

attributed by Phelan and Hey to the pattern of health carerather than to any environmental or climatic differencesbetween Britain and Australia. They draw attention to theNorth American claim that management in a specialisedcentre is essential for optimum care, 1 and the data fromDenmark12 reporting better survival among patientsattending a specialised clinic compared with those lookedafter by their local paediatrician, "as happens for mostchildren in England and Wales". Nevertheless the 80%survival figure for Danish patients is only 10 years (comparedwith 9 in England and Wales), rising to 12 years for thosepatients treated exclusively at the national CF centre, whocomprise 77% of the total. Survival curves4,12 show that theprobability of living to the age of 20 is 61 % for the Danish CFcentre patients and 62% for England and Wales, and thereseems to be little difference between the 50% survival figuresfor Denmark, England and Wales, and the USA and Canada.Whether centralisation of treatment would in itself

substantially improve the prognosis remains to be shown, butthe high mortality among young British infants leaves noroom for complacency. Death from meconium ileus is nowunusual in neonatal surgical centres and early diagnosisshould ensure that most affected infants will receive promptand adequate treatment for respiratory infections, whether ata special centre or at a district general hospital. Theprevention of chronic lung disease poses a greater problem,since the underlying cause is poorly understood. However,Phelan and his colleagues have shown elsewhere that theirpatients have a remarkably low incidence of infection with Psaeruginosa, which they suggest may be due to prophylactictreatment with inhaled gentamicin.13 The single most

important factor influencing prognosis may be early

5. di Sant’Agnese PA, Andersen DH Celiac syndrome. iv. chemotherapy in infections ofthe respiratory tract associated with cystic fibrosis of the pancreas; observations withpenicillin and drugs of the sulfonamide group with special reference to penicillinaerosol. Am J Dis Child 1946; 72: 17-61.

6. Bodian M, ed. Fibrocystic disease of the pancreas. London: Heinemann, 1952: 119-55.7. Høiby N. Microbiology of lung infections in cystic fibrosis patients. Acta Paediatr

Scand 1982; 301: 33-54(suppl).8. Batten JC. Croonian lecture, Royal College of Physicians, London, 1983.9. Baltimore RS, Mitchell M. Immunologic investigations of mucoid strains of

Pseudomonas aeruginosa; comparison of susceptibility to opsonic antibody in mucoidand non-mucoid strains. J Infect Dis 1980; 141:238-47.

10. Makrowitz SM, Macrina FL, Phibbs PV. Antimicrobial susceptibility of mucoidPseudomonas aeruginosa and their spontaneously occurring non-mucoid derivatives.J Antimicrob Chemother 1980; 6: 251-60.

11. Stern RC, Boat TF, Doershuk CF, Tucker AS, Primiano FO, Matthews LW. Course ofcystic fibrosis in 95 patients. J Pediatr 1976; 89: 406-11.

12. Nielsen OH, Schiøtz PO. Cystic fibrosis in Denmark in the period 1945-81.Evaluation of centralised treatment. Acta Paediatr Scand 1982; 301: 107-19(suppl).

13. Phelan PD, Allan JL, Landau LI, Barnes GL Improved survival of patients with cysticfibrosis. Med J Austr 1979; i: 261-63.

Page 2: SURVIVAL IN CYSTIC FIBROSIS

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diagnosis,14 but another Australian paper, from New SouthWales, reported a mean delay in diagnosis of about 21h yearsafter the earliest symptoms.l5 The development of reliableneonatal screening tests for CF, which can be done on driedblood spots,16,17 offers the opportunity for prospectivestudies to evaluate the importance of presymptomaticdiagnosis and specialised clinical care, as well as specificforms of treatment.

THE MONTEGGIA LESION IN CHILDREN

IN 1814 Giovanni Monteggia described the association ofanterior dislocation of the head of the radius with a fracture ofthe proximal third of the ulna.18 Bado19,20 named thecondition the Monteggia lesion when he classified thefracture dislocation into four types depending on thedirection of radial head dislocation and the presence of aradial fracture. The condition is rare, representing 1 - 7% ofall forearm fractures, 19 with Bado’s type 1 lesion

’(Monteggia’s original fracture) accounting for nearly two-thirds of all cases.20 This is the commonest lesion inchildren. 20-22 The type 3 lesion (lateral or anterolateraldislocation associated with an ulnar metaphyseal fracture)occurs exclusively in children20 but the type 2 (posteriordislocation) and the type 4 (anterior dislocation associatedwith ulnar and radial fractures) occur mostly in adults.20,21The condition is associated with local neural damage, 21-11ulnar non-union,26-29 and proximal radio-ulnar synostosis.24It is commonly missed on initial examination 29-3 ’ andchildren may present with a chronic radial head dislocationoften associated with an ulnar mal-union. Indeed, so

frequently is it missed that it has been suggested that allapparently congenital unilateral radial head dislocations arethe sequelae of missed Monteggia fracture dislocations.32While there is now general agreement that the Monteggialesion in adults is unstable and should be internallyfixed28,29,33,34 most authorities believe that it can be

adequately treated in children by closed methodS,22,21,29,34-36internal reduction and fixation being undertaken if this is

14. Shwachman H, Kulczycki LL. Long-term study of one hundred and five patients withcystic fibrosis; studies made over a five-to-fourteen year period. Am J Dis Child1958; 96: 6-15.

15. Wilcken B, Towns SJ, Mellis CM. Diagnostic delay in cystic fibrosis: lessons fromnewborn screening. Arch Dis Childh 1983; 58: 863-66.

16. Heeley AF, Heeley ME, King DN, Kuzemko JA, Walsh MP. Screening for cysticfibrosis by dried blood spot assay. Arch Dis Childh 1982; 57: 18-21.

17. Hammond KB, Ask CG, Watts DC. Immunoreactive pancreatic lipase test for neonataldetection of cystic fibrosis. Lancet 1984; i: 42.

18. Peltier LF. Eponymic fractures: Giovanni Battista Monteggia and Monteggia’sfracture. Surgery 1957; 42: 585-91.

19. Bado JL The Monteggia lesion. Springfield, Illinois: C. C. Thomas, 1959.20. Bado JL. The Monteggia lesion. Clin Orthop 1967, 50: 71-86.21. Pieró A, Andres F, Fernandez-Esteve F. Acute Monteggia lesions in children. J Bone

Joint Surg 1977; 59A: 92-97.22. Bryan R. Monteggia fractures of the forearm. J Trauma 1971; 11: 992-98.23. Jessing P. Monteggia lesions and their complicating nerve damage. Acta Orthop Scand

1975; 46: 601-09.24. Bruce HE, Harvey JP, Wilson JC Monteggia fractures. J Bone Joint Surg 1974; 56A:

1563-76.

25. Spinner M, Freundlich BD, Teicher J. Posterior interosseous nerve palsy as a

complication of Monteggia fractures in children. Clin Orthop 1968; 58: 141-45.26. Smith FM. Monteggia fractures. An analysis of twenty-five consecutive fresh injuries.

Surg Gynecol Obstet 1947; 85: 630-40.27. Holdsworth FW. Fractures ofthe radius and ulna. In: Clark JMP, ed. Modern trends in

orthopaedics vol 3. London: Butterworths, 1962: 84-99.28. Mobley JE, Jones JM. Monteggia fractures. Proc Staff Meet Mayo Clin 1955; 30:

497-504.

29. Boyd HB, Boals JC. The Monteggia lesion. A review of 159 cases. Clin Orthop 1969;66: 94-100.

30. Speed JS, Boyd HB. Treatment of fractures of ulna with dislocation of head of radius.(Monteggia fractures.) JAMA 1968; 115: 1699-1705.

31. Fowles JV, Noureddine S, Kassab MT. The Monteggia lesion in children. J Bone JointSurg 1983; 65A: 1276-83.

32 Lloyd-Roberts GC, Bucknill TM. Anterior dislocation of the radial head in children.Aetiology, natural history and management J Bone Joint Surg 1977; 59B: 402-07.

33 Benjamin A. Injuries of the forearm. In: Wilson JN, ed. Watson Jones fractures andjoint injuries. Edinburgh: Churchill Livingstone, 1982: 675-78.

unsuccessful. The management of the missed Monteggialesion is controversial. Some surgeons believe that the

deformity should not be treated;36 others feel that operativereduction should be undertaken only in the first three3’ orSix35 months; while a third group believe that open reductionwith repair or reconstruction of the annular ligament is

mandatory.31,32,38Despite a general acceptance of the conservative

management of the acute lesion in children there are fewseries comparing the results of conservative and operativetreatment. Reckling39 reported excellent function in fourtype 1 and three type 3 lesions treated conservatively. Bryan 12and Bruce et a124 both operated on 30% of their type 1 lesions,Bryan obtaining 93’4% and Bruce et al 64% overall

acceptable results. Piero et al2l operated on 11-1% of theirtype 1 fractures and reported excellent results irrespective ofthe treatment method. Smith26 operated on 16 of22 fracturesand claimed excellent results in 15 of the operated cases.Lately, Fowles et a131 have suggested that the prognosis of thelesion depends on the obliquity of the ulnar fracture. Theyfound that conservative management was adequate fortransverse ulnar fractures but 4 of their 5 closed obliquefractures required internal fixation and they recommendprimary internal fixation for all oblique and comminutedfractures. There were 5 previously missed lesions in thisseries, all with impaired elbow function. Operative reductionof the radial head with repair or reconstruction of the annularligament with a fascial loop improved function in all cases.Fowles and co-workers advocate this procedure in all cases oflate presentation. The rotational component of most type 20,40 and many type 3z° lesions means that transverse

fractures are uncommon, and the contention that all otherfractures require primary internal fixation is not supportedby other workers. However, Fowles et al are correct in

drawing attention to the unstable nature of this fracturedislocation in children and in pointing out that surgery isoften necessary. The notion of’.’uniformly good results" inchildren managed conservatively is unreasonable and theresults of Bruce et al,24 Smith,26 and Fowles et a13l in

particular give no room for complacency. All Monteggialesions require careful follow-up with internal reduction andfixation where conservative management fails.Chronic radial head dislocation should be treated by open

reduction and repair or reconstruction of the annular

ligament. Lloyd-Roberts and Bucknill32 have pointed out theefficacy and low morbidity of this procedure and recommenda modification of Bell Tawse’s38 original repair whereby thelateral portion of triceps is supplemented by Kirschner wirefixation. Persistent dislocation of the radial head can lead to

impairment of elbow function,31,32 degenerative arthritis,3land tardy nerve palsies;31,4l and reduction of the radial headwithout annular ligament repair or reconstruction can resultin persistent instability. 40,42

34. Anderson LD. Fractures of the shafts of the radius and ulna. In: Rockwood CA, GreenDP, eds. Fractures. Philadelphia: J. B. Lippincott, 469-77.

35. Salter RB. Textbook of disorders and injuries of the musculo-skeletal system, 2nd ed.Baltimore: Williams and Wilkins, 1983: 444.

36. Pollen AG. Fractures and dislocations in children. Edinburgh: Churchill Livingstone,1973: 61-68.

37. Blount WP. Fractures in children. Baltimore: Williams and Wilkins, 1954.38. Bell Tawse AJS. The treatment of malunited anterior Monteggia fractures in children.

J Bone Joint Surg 1965; 47B: 718-23.39. Reckling FW Unstable fracture-dislocations of the forearm (Monteggia and Galeazzi

lesions). J Bone Joint Surg 1982; 64A: 857-63.40. Evans EM. Pronation injuries of the forearm with special reference to the anterior

Monteggia fracture. J Bone Joint Surg 1949; 31B: 578-8841. Austin R. Tardy palsy of the radial nerve from a Monteggia fracture. Injury 1976; 7:

202-04.

42. Trillat A. Fractures complexes de Monteggia. Acta Orthop Belg 1958; 24: 114-24