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1 Ross. F P, Dunphy, J. E New Engl. J. Med. 1950, 242, 3592 Sparkman, R S Ann. Surg. 1959. 149. 746.3. Thudichum, J L. W. Brit. med. J. 1859. ii, 935.4 Bobbs, J. S. Trans Indiana State med. Soc. 1863, 18, 68.5 Kocher. T KorrespBl. Schweiz. Arz. 1878. 8, 577.6 Sims. J M Brit. med. J. 1878. 1, 811.7 Keen, W W Amer J. med. Sci. 1879, 77, 134.8 Allen, A W Surgery 1956, 39. 7109 Donald, J G. Fitts, W. T Amer J. Surg. 1949. 77. 596.10 Cutler C W., Jun. Surg. Clin. N Amer. 1949, 29, 361.11 Glenn, F Surg. Gynec. Obstet. 1950. 90. 643.12 Glenn. F. Kays D M ibid. 1955 100 1113 Lahey. F H Surg Clin. N Amer. 1952. 32, 83714 Orr T. G Amer J. dig. Dis 1947. 14. 89.15. Zollinger, R., Cutler, E. C. J. Amer. med. Ass. 1943, 121,

491.

ently safe for both mother and child, and, properlydeveloped, should not be too complex or costly to beused by any obstetric unit which is adequatelystaffed. If it fulfils its early promise it will allowus to shorten labour, reduce pain, and perhaps lowerintra-amniotic pressure. This could be a valuableaid in the management of foetal distress or of a

lahour in which the fœtus is at greater than normalrisk.

Operation for the Gallstones

CHOLECYSTOSTOMY has been surgical anathemafor so many years that, even if it had no

intrinsic merits, cynics might predict its imminentrediscovery. Together with some other historic

operations for gallstones, it is certainly due forreassessment. Ross and DUNPHY 1, two of itsexponents, described it some years ago as " an un-wanted stepchild in the family of operations(whose) presence is recognised but whose legiti-macy is suspect "-an accurate enough rendering ofsurgical opinion but possibly misleading in its

implication of youth. Cholecystostomy is nearly ahundred years old. As SPARKMAN recently re-

called 2, THUDICHUM suggested in 1858 thatsevere gallstone disease might be relieved by attta-

ching the fundus of the gallbladder to the peri-toneum at a first operation and removing thestones at. a second 3 ; and BOBBS of Indiana-polis actually opened the gallbladder ten yearslater under the impression that he was incisinga large ovarian cyst 4. Other pioneers—KOCHER,SIMS, and KEEN among them 5-7-explored icspotentialities and pitfalls and established it as a

planned procedure: they recognised that, thoughit rarely afforded complete and lasting relief, ithad at least a commendably low mortality. Itsdecline came with the advent of cholecystectomy :thought never wholly eclipsed, cholecystostomy soonbecame one of those operations which well-bred

surgeons might have to perform but would not

talk about. Some, it is true, continued to extol itsvirtues for poor-risk patients 8-15 ; but in theeyes of the majority it became a symbol of the

poor-risk surgeon, an admission of operative inexpe-ri&nce at its best, and of diagnostic blunder at itsworst. Few surgical registrars, boldly tackling anacute cholecystitis, have not found it on occasionsa last line of safe retreat ; but such retreats, whileundoubtedly saving lives, enhanced neither theirown nor the operation’s reputation.There will always be a place for second-thought

operations for patients whu should never have beenoperated on in the first place: but cholecystostomyhas other, more generally recognised, indications.It is the best treatment in many injuries to the

gallbladder and bile-ducts, even without or before

16. Sparkman. R S Ann Surg. 1952. 135, 863.17. Glenn, F. Wantz, G. E. Surg. Gynec. Obstet. 1956, 102, 145.18. Miangolarra, C Ann, Surg. 1959, 149, 760.19. Lancet, 1956, ii, 184

actual rupture ; in fulminating cholecystitis pro. ’gressing to gangrene, perforation, and general peri. tonitis ; and in a diminishing number of patients :

who must be judged too ill to withstand cholecysto. ‘

tectomy but who are yet in urgent need of biliarydecompression. This last group includes the occasional acute cholecystitis complicating convales-cence from some other operation 16-17. The valueof cholecystostomy is less firmly established in ;acute pancreatitis ; and whether old age alone is a valid indication is debatable. In all these groups, however, cholecystostomy has always retained its usefulness; and SPARKMAN 2, has done well to set out its advantages against its often elaborated and more-or-less obvious drawbacks.

Cholecystostomy is, above all, a simple opera. tion ; and though (like most simple operations) it may be exceedingly difficult to perform well, it isusually within the competence of relatively inexpe-rienced surgeons. If need be, it can be performedunder local anaesthesia. It provides " prompt effect-ive decompression " of the biliary tract. " therebyrelieving the immediate attack and circumven.

ting gangrene, rupture, and peritonitis "; and, if stones have entered the common bile-duct,

"

it relie. ves or prevents the development of obstructive jaun- dice or cholangitis and obviates the necessity for

exploration of the choledochus at a difficult time " 2. The citation sounds almost too good : andit is as well to add a few provisos. The operation relieves obstruction only when the obstructing agent is removed : and this may not be easy. A calculus Eimpacted in Hartmann’s pouch, or ulceratingthrough it, may resist many gentle and repeatednot-so-gentle attemps to dislodge it ; and downrightviolence is apt to end in disaster.With many smallcalculi, on the other hand, it may be hard to ensurethat all have been retrieved ; and if one has alrea.dy blocked the cystic duct, the operation will not,of course, relieve common-duct obstruction. Sev-eral other rare indications or remote advantagescould be added to SPARKMAN’S useful list : chole-

cystostomy, for example, preserves what is by farthe easiest route for palliative decompression,should the patient ever have malignant common-duct obstruction.Useful as it may be to bear in mind these advanta ’,

ges of cholecystostomy (and one could enlarge tooon the similar merits of partial cholecystectomy 18),they apply only to a. tiny minority of gallstonepatients. Over the majority, cho1cystectomy hasheld sway for so long that even to mention an

operation such as cholecystolithotomy as an alter.native may seem anachronistic. So long as gall.stone disease is interpreted as the texbook entityof longstanding cholecystitis, punctuated as a ruleby acute episodes of colic or obstruction, there islittle to be said for anything less than completeremoval of the offending viscus. But this may notbe the only true interpretation. Some years agowe suggested that the gallbladder that is very easyto remove is usually not worth removing 19 ; andmany patients could still testify that the function-ing gallbladder has a definite part to play in

maintaining physical wellbeing. The postcholecyst-ectomy syndrome is clearly not a myth, even

93

20 ibid. 1957, ii, 1269.21 Ogilvie. W H. J. R. Coll. Surg. Edin. 1956, 2, 49.22 Hunt, T. C Practitioner, 1954, 172, 146.

though its victims tend to drift away from hos-

pitals and even though accounts of relief mea-

sures seem quickly to acquire a mythologicalring. With the moral rehabilitation of the func-

tioning gallbladder, however, came the gradualrealisation, even among conservative physicians,that gallstones causing symptoms should be remov-ed ; and the sooner the better 20-21. Indeed, mostdoctors now admit (though many in tones of noenthusiasm) that theoretically all gallstones shouldbe operated on, irrespective of symptoms. In prac-

. tice both the family doctor and the patient are

often loth to turn to cholecystectomy. The opera-tion still has a considerable morbidity (its death-rate in this country is probably less than 2 %) ;and in some respects the best that can be said forit is "that though it has failures... medical treat-mant has more 22. HUNT estimated that pros-pects of lasting relief of proved gallstones " is pro-bably not higher than 75 ’o whatever treatmentis advised" 22.Fears of the postcholecystectomy syndrome on

the one hand and of recurrent attacks of chole-cystitis on the other can be reconciled when it comesto management of the patient, so long as calculi aretreated as magic therapeutic touchstones. For-many years surgeons and physicians have equatedgallstones with lack of gallbladder functions--a con-venient notion, since whereas stones stood for ope-ration, non-function guaranteed against unpleasantafter-effects. It may be time, however, to takenotice of a large group of ’patients, demonstratedby advancing radiological technique, to whom thisrule-of-thumb does not apply. The patients seemto have one or a few "acute stones in other-wise well-functioning gallbladders presumably theproducts of some recent and transient disturbancein the composition of bile rather than of langstand-ing gallbladder dysfunction. Since we do not howgallstones (or any stones for that matter) are for-med in the body, we are here in the realm of in-

verted commas ; but the more negative shadowswe can detect by X-rays, and the bolder we getin recommending operation irrespective of symp-toms, the harder pathologists have to look for

signs of "chronic inflammation", or of any other

disease, in the gallbladders surgeons remove.If sometimes pyelolithotomy is preferrable to

nephrectomy, why not cholecystolithotomy to chole-cystectomy ? Part of the answer is that, thoughthe choice of operation for renal calculi is dictatedlargely by the preciousness of functioning renal

tissue, most surgeons assume that patients willgladly surrender their gallbladders in exchange forthe certainty that they will have no gallbladdertrouble in the future. It is true, of course, that apatient without a gallbladder cannot develop stonesin it, though be may breed far more troublesomeones in his bileducts, and it is very probable that,once the gallbladder has ceased to function, it willprove a good riddance. But the question remains :how serious is the risk of new stones forming inan apparently normal and still well-functioningorgan after one set of recent stones has been removed? Many of the symptoms which we now attri-bute to gallstones may be more properly ascribedto lack of gallbladder function ; and it seems

absurd to try to relieve them by removing both

1. Schmid, R., Schwartz, S., Sundberg, R. D. (1955) Blood,10, 416.

2. Townsend-Coles, W. F., Barnes, H. D. (1957) Lancet, ii,271.

3. Goldberg, A. (1959) Quart. J. med., 28, 183.4. Waldenstrom, J. (1957) Amer. J. Med., 22, 758.5. Saint, E. G, Curnow, D., Paton, R., Stokes, J. B. (1954).

Brit. med. J., i. 11826. Watson, C. J. (1954) Advances in Internal Medicine; Vol. 6.

Chicago.7. Tio, T. H., Leijnse, B., Jarret, A., Rimington, C. (1957)

Clin, Sci. 16, 517,8. Holti, G., Rimington. C. Tate, B, C., Thomas, G. (1958).Quart. J. Med. 27, 1.

stones and gallbladder. If all gallstones do in factneed removing (and they are certainly safer outthan in) is not the loss of all gallbladders whichcontain them an excessive price for safety ?

Porphyria in South AfricaALTHOUGH the porphyrias are uf world-wide dis-

tribution, they are considered uncommon diseases.There is no doubt about the rarity of congenitalporphyria, a disease probably inherited as a recessivetrait, in which abnormal sensitivity to light, and theexcretion of uroporphyrin i, are usually presentfrom birth. SCHMID et al. 1 traced only 34 authenti-cated cases in 1955, since when two further cases inSudanese siblings have been reported 2. Acute inter-mittent porphyria has been regarded as the leastuncommon of the porphyria group. It is inheritedas a mendelian dominant trait with variable clinicalpenetrance; the patient’s urine contains much por-phobilinogen and &dgr;-aminolævulic acid, the precur-sors of the porphyrins; and the main clinical featuresare pain, chiefly in the abomen, and neuropsychiat-ric manifestations u. WALDENSTRÖM 4 has suggesteda frequency of 1.5 per 100,000 inhabitants of Sweden,though the rate was far higher (1 per 1000) in

Lapland, where he found a large family with 137 .cases. SAINT et al. in Australia put the frequencyat 1.5 per 100,000 adult females. Although manycases have been collected in the U.S.A. 6 and inGreat Britain 3, the prevalence in these countrieshas not been estimated.There has been much controversy over porphyria

cutanea tanla. and its genera! frequency is unknown.WALDENSTRÖM 4, believing that his original conceptof this group required revision. has defined two

types: (a) porphyria cutanea tarda symptomatica,and (b) porphyria cutanea tarcla hereditaria or proto-coproporphyria. In the former, photosensitisationusually occurs in adult life through non-hereditarymechanisms—e.g., alcoholic cirrhosis or (very rarely)a porphyrin-producing hepatic adenoma 7—anduroporphyrin and coproporphyrin are excreted inthe urine. In the latter, photosensitisation occursin youngish persons, and there are sometimes acuteattacks resembling those of acute intermittent por-phyria. The condition is transmitted as a mend-

etian dominant trait B RIMINGTON has demonstrated

reciprocity in the excretion of porphyrins of this

group: in remission increased quantities of proto-porphyrin and coproporphyrin are often excreted

only in the faeces, whereas during an active phaseof photosensitivity the urinary porphyrins may beincreased considerably but the faecal porphyrins notso much. In his tentative classification WALDENS-TRÖM does not include mixed porphyria 6, and hethinks that acute intermittent porphyria remainstrue to type and entirely separate from the cutaneousporphyrias.