1380

glucose. 19 The other possibility, that more rigorous exclusionof galactose may be necessary, was the main topic at an ad-hocmeeting earlier this year at Alder Hey Children’s Hospital,Liverpool. The main dietary restrictions in galactosaemia aremilk, milk products, and manufactured foods such as babycereals, soups, and malted milks. Dietitians need

comprehensive lists of the items to be avoided and foodmanufacturers have cooperated in supplying the information.The feeling is that restriction of these foods, which are theprincipal sources of galactose or lactose, should be lifelong;20indeed, most galactosaemic patients feel unwell if they havethem at any age. More uncertainty has existed about the needto exclude other possible minor sources of galactose. Manyfoods such as offal, soya, and vegetables contain smallamounts of galactose in the form of complex a-galactosides,but the weight of evidence suggests that, in man, this

galactose is not absorbed. However, it was felt that, as aprecaution, foodstuffs known to contain a-galactosidesshould be avoided for the first year of life. As regards thelactose used widely as an additive to drugs and

pharmaceutical preparations the position is less clear becausemanufacturers have been reluctant to reveal the compositionof their products. Such an attitude is not really excusable.The need for greater or lesser degrees of galactose restrictionmay be debated on theoretical grounds, but there have beenno careful studies correlating diet and long-term outcome.Biochemical monitoring, regarded as essential in

phenylketonuria, has seldom been used in galactosaemia.This is unfortunate since erythrocyte galactose-1-phosphatelevel seems to be a sensitive indicator of galactose intake andits possible toxicity.21Thus, despite initial optimism, the outcome of treated

galactosaemia has proved unsatisfactory. There is an urgentneed for more information on which to base management,and thirty years seems to have been lost in this respect. In theU.K. a thorough retrospective study of all known cases couldprovide some answers on the relation between age of

diagnosis, type of illness, diet control, and long-termoutcome. The main requirement, however, is a prospective.multicentre study to determine whether the various dietaryregimens do alter galactose-1-phosphate levels and whether,over a long period, biochemical control correlates withmental and physical development. The problemsencountered ’with galactosaemia have other importantimplications, since this condition is only one of many inbornerrors of metabolism presenting with an acute illness inwhich the baby’s life can be saved by intensive treatment.The long-term outcome for these babies is uncertain, andunless the matter is tackled by a collaborative effort we shallstill be in doubt about the answer thirty years hence.

SURGICAL STAPLERS

FOR at least twenty years surgical stapling machines havethreatened to take over from man. The history of theseingenious instruments goes back to a strong Austro-

Hungarian origin at the beginning of this century when thevon Petz instrument ultimately replaced the classic Fischer-

19 Gitzelmann R, Hansen RG. Galactose biogenesis and disposal in galactosaemics.Biochim Biophys Acta 1974; 372: 374-78.

20. Brandt NJ. How long should galactosaemia be treated? In: Burman D, Holton JB,Pennock CA. eds. Inherited disorders of carbohydrate metabolism Lancaster:MTP Press, 1980: 117-24.

21. Roe TF, Ng WG, Bergren WR, Donnell GN. Urinary galactitol in galactosemicpatients Biochem Med 1973: 7: 266-73.

Hultl stapler, named after a duet of engineer and surgeon, thelatter known in his native Budapest as the Paganini of theknife. In the 1950s a struggle of the new superpowersdeveloped. The Russian "gun ’"2 made an initial impact inthoracic and rectal surgery, but was rapidly superseded by therefined and wider-ranging American devices (’AutoSuture’).There are now beautifully designed and highly efficientinstruments (both reusable and disposable) for closing skinand fascia, for simultaneous ligation and division of bloodvessels, and for fashioning a bewildering range of

gastrointestinal anastomoses. The more ingenious surgeoncan now use "steel all the way" for any operation fromvaricose veins and appendicectomy, through cholecy-stectomy and gastrectomy, to lung resection and oesophagec-tomy ; but should he? Are metal staples better, safer, and morecost-effective than catgut and silk? In a retrospective survey3of 362 routine upper gastrointestinal operations done over asix-year period in a district general hospital, Lowdon et a1. 3

report a lower complication rate (anastomotic leak, woundinfection, and bleeding) with staplers than with conventionalsuturing, though the difference, 21% versus 16%, was notstatistically significant. They stress that they hadconsiderable experience of surgical staplers, and although allgrades of surgical staff took part in the study more "stapled"operations were done by the consultants-and why shouldthey not play with the new toys? There was a strikingimprovement in the leakage rate from traditionally difficultsuture lines (duodenal stump closure and oesophagealanastomosis). As with any machine there were mechanicalproblems, four "failures to fire" all recognised at once andcured with catgut. Presumably there are similar but

unrecognised mechanical failures with conventional

suturing, when the new senior house-officer ties hiscontinuous stitch with a series of half-hitches instead of a

surgeon’s (or even granny’s) knot.There was a reduction of about 50% in operating time,

particularly for those complex procedures requiring severalanastomoses, and herein might lie a reason for preferringexpensive staples to cheap sutures. But was this just areflection of surgical skill? In a randomised study Reiling etal. showed that staples made no difference to operating time;and lately the same conclusion has been reported from a non-randomised investigation of colon surgery (experiencedoperators only) in West Virginia. Scher et al. report nodifference in operating time, time to return of gastrointestinalactivity, or length of postoperative stay in their stapled andsutured groups, which in most respects had been similarbefore operation. For very low colorectal anastomoses,

operators did tend to choose staplers.The argument for a massive swing towards staplers is not

strong. By all means use them for difficult low resection of therectum, thereby inflicting fewer colostomies,6 and to

eliminate anastomotic leaks in oesophageal surgery,’ butsurely, during a simple gastroenterostomy, the surgeon-in-training must still learn how to sew a neat seam by hand.

1. Robicsek F. The birth of the surgical stapler. Surg Gynecol Obstet 1980, 150: 579-83.2. Ravitch MM, Brown IM, Daviglius GF Experimental and clinical use of the Soviet

bronchus stapling instrument. Surgery 1959; 46: 97-1083. Lowdon IMR, Gear MWL, Kilby JO. Stapling instruments in upper gastrointestinal

surgery: a retrospective study of 362 cases. Br J Surg 1982: 69: 333-35.4. Reiling RB, Reiling WA, Bernie WA et al. Prospective controlled study of

gastointestinal stapled anastomoses. Am J Surg 1980; 139: 147-525. Scher KS, Scott-Conner C, Jones CW, Leach M. A comparison of stapled and sutured

anastomoses in colonic operations. Surg Gynecol Obstet 1982; 155: 489-93.6. Heald RJ Towards fewer colostomies-the impact of circular stapling devices on the

surgery of rectal cancer in a district hospital. Br J Surg 1980, 67: 198-2007. Fabri B, Donnelly RJ. Oesophagogastrectomy using the end-to-end anastomosing

stapler Thorax 1982; 37: 296-99