1000

and echocardiography are complementary. The

frequency with which a completely non-invasiveassessment is adequate depends on the disease inquestion, the technical quality of the echocardiograms,and the attitudes of the cardiologist and cardiac

surgeon.

MECONIUM ILEUS

MECONIUM ileus, the earliest and most severe of theabdominal complications of cystic fibrosis, is not common.Cystic fibrosis occurs in between 1/1150 and 1/2000 1,2 livebirths among white people, and of these babies only 10-15%have meconium ileus, which affects 40-50 babies a year inEngland and Wales. Few paediatric surgical units treat morethan two or three cases a year and this small numbercombined with the early unsatisfactory results of treatment(e.g., only 15% alive after one year3) has made manypaediatricians pessimistic about their management. This wasnot the attitude of Winifred Young, who felt that the

prognosis of babies with meconium ileus should be betterthan that of others born with cystic fibrosis since prophylactictreatment could be started before the onset of lung damage.Her prediction is only now being borne out throughimproved medical management, total parenteral nutrition,non-operative relief of the obstruction by radiocontrastenemas, and improvements in surgical technique. A report4from the Children’s Hospital of Los Angeles, where 31children with meconium ileus were treated in the 18 years1963 to 1980, is encouraging. 3 of the babies were

successfully treated by meglumine diatrizoate enemas, 28required operation, and there were 25 early survivors. Theirsubsequent progress has also been good, with 84% alive at 3months and 71% (20/28) at one year (3 babies were still lessthan one year old at the time of the report). In 1972,McPartlin et al. from London reported a 70% early survivaland Dickson and Mearns6 confirmed these results. Santulli7and Chappell8 have also reported success rates of this order.A ’Gastrografin’ enema9 (meglumine and sodium

diatrizoate) should be tried first in uncomplicated cases and iseffective in up to half. Provided the baby’s condition remainssatisfactory this can be repeated. For complicated cases, thosenot relieved by the enema, and where the diagnosis is in

doubt, operation is still required. The actual technique usedseems less important than the precautions listed by the LosAngeles team-adequate resection of the dilated

hypertrophied segment, complete evacuation-of the bowelcontent proximally and distally, and.care to ensure that the

1. Prosser R, Owen H, Bull F, Parry B, Smerkinich J, Goodwin HA, Dathan J. Screeningfor cystic fibrosis by examination of meconium. Arch Dis Childh 1974; 49: 597-601

2. Stephan U, Busch EW, Kollberg H, Hellsing K. Cystic fibrosis detection by means of atest-strip. Pediatrics 1975; 55: 35-38.

3. Holsclaw DS, Eckstein HB, Nixon HH. Meconium ileus-a 20 year review of 109cases. Am J Dis Child 1965; 109: 101-13.

4. Mabogunje OA, Wang Chun-I, Mahour GH. Improved survival of neonates withmeconium ileus. Arch Surg 1982; 117: 37-40.

5. McPartlin JF, Dickson JAS, Swam VAJ. Meconium ileus. Immediate and long termsurvival Arch Dis Childh 1972; 47: 207-10.

6. Dickson JAS, Mearns MB. Meconium ileus. In: Wilkinson AW, ed. Recent advances inpaediatric surgery. Edinburgh: Churchill Livingstone, 1975: 143-55.

7. Santulli TV Meconium ileus. In Holder TM, Ashcraft KW, eds. Pediatric surgery.Philadelphia: W. B. Saunders, 1980. 356-73.

8. Chappell JS, Management of meconium ileus by resection and end-to-end anastomosis.S Afr Med J 1977; 52: 1093-94.

9. Noblett HR. The treatment of uncomplicated meconium ileus by Gastrografin enema.A preliminary report. J Pediatr Surg 1969; 4: 190.

bowel anastomosed has a good blood supply and is

undamaged by handling. Four different techniques are inuse: resection and double enterostomy, recommended byGross, resection and primary anastomosis, recommendedby Swenson," as used by Chappell and in Los Angeles;4resection and proximal to distal end-to-side anastomosis witha distal enterostomy, recommended by Bishop and Koop;12and the reverse arrangement with a proximal enterostomyand side-to-end anastomosis, recommended by Santulli.13The double enterostomy and Santulli techniques leave aproximal stoma which may require inconveniently earlyclosure. In the past, most surgeons avoided primary ana-stomosis because they feared breakdown in the anastomosisfrom the unsatisfactory nature of the anastomosed gut andbecause a distal obstruction might still be presentpostoperatively. The Bishop/Koop operation carries thetheoretical objection that the safety valve is distal to theanastomosis it is designed to protect, and seems, for no veryobvious reason, to work well for some surgeons and not forothers. The stoma does not cause trouble since it will onlyleak if there is a distal obstruction; closure is a minor

procedure which can be done at any time. Some 40-50% ofcases are complicated (14 out of the 31 in the Los Angelesseries) by perforation, atresias, volvulus, gangrene, andmeconium peritonitis. Where there is much gut damage orloss and healthy ends cannot be obtained without furtherextensive resection, a double enterostomy is probably safestafter the resection in this group. For the others, the bestprocedure is probably the one with which the surgeon ishappiest.

. A corollary to these observations is that, in any case of smallbowel atresia or meconium peritonitis, cystic fibrosis shouldbe excluded by a sweat test. Between 5% and 20%14 of babiesin some series of meconium obstruction prove not to have

cystic fibrosis. The aetiology of these is obscure, but a fulltreatment regimen should be started initially until the

diagnosis of cystic fibrosis has been confirmed or refuted.The standard for results in the treatment ofmeconium ileus

has now been set. Whichever regimen is followed, 90% ofbabies with uncomplicated meconium ileus should survivetheir initial treatment, 70-80% should be alive and well atone year, and more than half should reach their teens inreasonable health.

GIGGLE INCONTINENCE

PHRASES such as "I laughed till I wet myself’ have theirequivalent in many languages. Thus a causal relation betweenlaughing and uncontrolled voiding of urine has long beenaccepted. However, it was not until 1959 that Mac Keith’coined the term "giggle micturition", to separate this form of

10. Gross RE. The surgery of infancy and childhood. Philadelphia: W. B. Saunders, 1953175.

11 Swenson P. Obstruction of the small intestine in the neonatal period. In: Swenson 0,ed. Pediatric surgery, 1st ed. New York: Appleton-Century-Crofts, 1958: 319.

12. Bishop HC, Koop CE. Management of meconium ileus: resection, Roux-en-Yanastomosis and ileostomy, irrigation with pancreatic enzymes. Ann Surg 1957;145: 410.

13. Santulli TV. Meconium ileus. In: Mustard WT, Ravitch MM, Snyder WH, Welch KJ,Benson CD, eds. Pediatric surgery, 2nd ed. Chicago: Year Book Medical

Publishers, 1969: 851.14. Rickham PP, Boeckman CR. Neonatal meconium obstruction in the absence of

mucoviscidosis. Am J.Surg 1965; 109: 173.1. Mac Keith RC. Micturition induced by giggling? cataplexy. Arch Dis Childh 1959; 34:

358.