560 CORRESPONDENCE

REFERENCES

1. MacSween RNM: Hepatic sepsis after liver trans- plantation in dogs and pigs. Arch Path 88:166, 1969

2. Brettschneider L, Tong JL, Boose DS, et al: Specific bacteriologic problems after orthotopic liver transplanta- tion in dogs and pigs. Arch Surg 97:313, 1968

3. Kasai M: Treatment of biliary atresia with special reference to hepatic porto-enterostomy and its modifica-

tions, in Bill AH, Kasai M (eds): Progress in Pediatric Sur- gery, vol VI. Baltimore, University Park Press, 1974, p. 5

4. Lilly JR, A l tman RP, Dubois R, et al: A cholangiographic investigation of Kasai's operation for bil- iary atresia. Proceedings, American Pediatr ic Surgical Association, Dorado Beach, Puerto Rico, 1975

Reply to Dr. Lilly."

We have read Dr. Lilly's letter commenting on our paper on "Exper imenta l Investigations Into the Etiology of Cholangitis Following Operat ion for Biliary At res ia" (Journal of Pediatric Surgery, 13:55, 1978) with interest, but cannot agree with any of his remarks.

Dr. Lilly states that cbolangitis develops in a high propor- tion of pigs on whom major abdominal operations of any kind are performed. We do not question that; this may be the case with the type of pig Dr. Lilly uses, but in over a hundred major abdominal operations of different kinds on the strain of pig that we employ, namely the Gottingen mini- pig, we have never seen a case of cholangitis although we have always looked for it and performed histologic investi- gations of the liver. As we have shown in our article, even when ligating the common bile duct, cholangitis never developed, but in the majority of pigs on whom the hilar lymphatics were resected, severe cholangitis with multiple liver abscesses leading to the death of the animal was ob- served. Furthermore, in a paper that we hope to punish soon, we have shown that if resection of the hilar lymphatics is combined with omentopexy to reestablish the lymphatic flow, cholangitis does not develop.

The operation of hepatic portocholecystostomy has only very infrequently been carried out, as the necessary patency of the distal bile ducts is only rarely present. Little is, therefore, known about the long-term follow-up. During the PAPS-Meet ing in Osaka, Japan, May 1978, several authors, among them Dr. Kasai, Dr. Altmann, and Dr. Peter Jones, reported that most of their hepatic portocho- lecystostomies did not work and had to be reoperated on

and replaced by hepatoportoenterostomies. In the only he- patic portocholecystostomy that we performed, cholangitis developed.

The argument brought forward by Dr. Lilly that transhe- patio cholangiography done 6-10 wk after operation in his pa t i en t s d e m o n s t r a t e d r e e s t a b l i s h m e n t of lympha t i c drainage of the liver is, in our opinion, not valid because it is well known that lymphatic drainage is reestablished within 4 wk after operation. ~ We believe that the severe attacks of cholangitis appear early after operation. These attacks will probably leave islands of infection in the liver in spite of anti- biotic treatment, thus, causing recurrent infections. In this connection, it is interesting to note that Akiyama et al. reporting on their 13 yr experience with 179 cases of biliary atresia at the International Symposium on Cholestasis in In- fancy held at Sendal, Japan, June 1978, 2 divided cholangitis into two types. The early type occurred within 1 mo after operation and has a very poor prognosis; all these patients died. On the other hand, late cholangitis occurring any time after this period responded well to antibiotic treatment.

At the same meeting, it was suggested by Mr. Peter Jones of Melbourne, Australia, that in view of our experi- mental and clinical work the name of ascending cholangitis should not be used anymore, as there is no proof that the in- fection ascends and as we have produced quite a lot of evi- dence that infection is spread by the blood stream. We, therefore, should instead use the te rm postoperat ive cholangitis.

J. Hirsig P. P. Rickham

REFERENCES

1. Kocandrle V, Harttuin E, Prohaska JV: Regeneration 2. Akiyama H, Sawaguchi S, Nakajo T: Late complica- of the lymphatics after autotransplantation and homotrans- tions after surgery for biliary atresia. Lecture given at the plantation of the entire small intestine. Surg Gynecol International Symposium on Cholestasisin Infancy. Sendal, Obstet 122, 587, 1966 Japan, 1978

To the Editor:

The article by Noordijk and Bloemsma-Jonkman "Gas- troschisis: No Myth" February 1978 issue of Journal of Pediatric Surgery offers some practical guidelines for family counseling in the discussion of presumed genetic differences related to congenital defects of the abdominal wall? Un- fortunately, much of the discussion focuses on a misin- terpretation of a recent article of mine. 2 In the very first sentence, the authors state that " . . . Shaw qualified as a "myth" the distinction between gastroschisis mad ompha- locele." I do not consider omphalocele and gastroschisis to be the same entity as Noordijk and Bloemsma-Jonkman say

I do. Nor do I state, as these authors claim, that " . . . there is no difference in cause; only in timing:" Nor does a careful reading of my paper indicate, again as the authors claim, that "Shaw is convinced that there are no causal differences between omphalocele and gastroschisis and that the clinical differences can be explained by the age of the fetus at the time of the teratogenic agent." Indeed, my article does not speculate on what teratogenic factors are responsible for initiating the train of events which lead to ventral wall defects, although, I do point out some anatomic factors which probably contribute to the development of gastros- chisis.

In their criticism of my paper, Noordijk and Bloemsma-

CORRESPONDENCE 561

Jonkman miss the major point which I make no less than five times, i.e., " . . . that gastroschisis is the result of rup- ture of the amniotic membrane at the base of the umbilical cord . . . " and, thus, gastroschisis is, as is omphalocele, an anomaly of the developing umbilical ring and cord. Om- phalocele and gastroschisis, as currently defined, are clearly not the same clinical entity. Indeed, my paper reiterates the clinical differences originally pointed out by Moore. 3 What I refer to as "The Myth of Gastroschisis" is the widely ac- cepted belief that gastroschisis originates as a lateral ab- dominal wall defect, independent in its embryogenesis from the umbilical ring and cord. The article does not deny all clinical and embryologic differences between gastroschisis and omphalocele, but simply seeks to substitute a more ra- tional theory of embryogenesis for those currently in the literature. That Noordijk and Bloemsma-Jonkman misread my message is clearly suggested by their endorsement of the recently published observations of Thomas and AtwelP

whose conclusion about the origin of gastroschisis, published the year after my article appeared, is identical to mine, i.e., while clinical differences exist between gastroschisis and omphalocele and while genetic factors causing them may differ, the clinical evidence suggests that gastroschisis, like omphalocele and like hernia of the umbilical cord (or small omphalocele, if you will), is an anomaly of the umbilical ring and its covering rather than an independent malformation of the lateral abdominal wall.

Incidentally, why do Noordijk and Bloemsma-Jonkman exclude patients with small omphaloceles from their series? It would be interesting to know if these smaller ompha- loceles followed a genetic pattern more closely related to that of gastroschisis or to that of the larger omphaloceles.

Anthony Shaw, M,D. Dept. of Surgery

Univ. ofVa. Med. Center Charlottesville, Va. 22901

REFERENCES

1. Noordijk JA and Bloemsma-Jonkman F: Gastros- 3. MooreTC, Stokes GE: Gastroschisis. Surgery 33:112, chisis: No myth J Pediatr Surg 13:47, 1978 1953

4. Thomas DFM and Atwell JD: The embryology and 2. Shaw A: The myth of gastroschisis. J Pediatr Surg surgical management of gastroschisis. Br J Surg 63:893,

10:235, 1975 1976

To the Editor."

Recent work at The Rockefeller University has raised the question whether the intestinal absorption of cholesterol and plant sterols is super-normal in children with severe longstanding constipation. We have wondered whether the prolonged residence in the gut of unexcreted intestinal contents may promote the absorption of these sterols (that are ordinarily rather incompletely absorbed) as well as of the bile acids that are normally formed from these sterols.

This letter is written in order to appeal to physicians and surgeons who may be seeing children with behavioral or psy- chogenic cons t ipa t ion (or t odd le r s with unope ra t ed megacolon), who are on diets with ordinary intakes of fruits, vegetables, cereals, and/or nuts (all of which are rich sources of the various plant sterols). To answer the question we have posed, we need a single plasma specimen; with this we can measure the concentrations of total cholesterol, HDL-cholesterol, and the common plant sterols. Results will gladly be returned to the referring physician promptly.

Bloods should be drawn after an overnight fast in versene vacutainers (EDTA, purple topped), and the plasma col lec ted cleanly in a r u b b e r - s t o p p e r e d plain tube (chemically clean). Seal with tape and mail air mail (avoid- ing holidays and weekends) to Dr. Ahrens. Please include the patient's name and date, diagnosis, physician's name and address. Two milliliters plasma is satisfactory, five is preferred.

Thomas V. Santulli, M.D. Babies Hospital of New York 3975 Broadway New York, N.Y. 10032

E. H. Ahrens, Jr., M.D. The Rockefeller University Hospital 1230 York Avenue New York, N.Y. 10021