capable of absorbing life-supporting nutrients. These data demonstrate ..... zen-section diagnoses have been frequent in this series. Therefore, it may be ...
ANNALS OF SURGERY Vol. 218, No. 4. 504-51 1 Oc 1993 J. B. Lippincott Company
Extended Myectomy Myotomy A Therapeutic Alternative for Total Intestinal Aganglionosis Moritz M. Ziegler, M.D., Richard E. Royal, M.D., Jody Brandt, Jeff Drasnin, and Lester W. Martin, M.D.
From the Division of Pediatric Surgery, Children's Hospital, Medical Center, and the Departments of Surgery and Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio
Objective That total intestinal aganglionosis (extended Hirschsprung's disease) is uniformly incompatible with life as reported in 1985, is challenged by this series of patients treated over the last 7 years with an alternative therapy, extended myectomy-myotomy of the small bowel.
Summary Background Data A total of 16 neonates worldwide presented with intestinal obstruction secondary to total (extending to the stomach) or near total (to 40 cm of jejunum) intestinal aganglionosis confirmed at one or more leveling operations.
Methods A patient questionnaire was answered by the surgeon of all 16 patients.
Results The sex distribution was eight boys and eight girls. The definitive operation included extending an antimesenteric myectomy-myotomy from the ganglionic-aganglionic transition zone for variable lengths, the operative design being to create sufficient small bowel length to support life (40-cm minimum, total small bowel maximum). The myectomized bowel was terminated as an endstoma or as an isolated jejuno-ileal segment. Ten of 16 patients have survived (62.5%) whose length of ganglionated bowel varies from 0 to 40 cm (mean, 12.4 cm; median, 6.0 cm). Six patients have died from 1 to 33 months after operation (mean, 9.5 months; median, 5.0 months) of gut-induced infection (n = 5) and respiratory failure (n = 1); their ganglionated bowel length was similar to survivors (range, 0-26 cm; mean, 9.2 cm; median, 8.0 cm). Of the 16 patients, 15 have received enteral nutrients through the myectomized bowel. Of ten survivors, strikingly two are totally gut nourished (2 cm, 7-cm length of ganglionated bowel), six receive from 1/5 to 4/5 of total calories enterally, and one receives minimal enteral feeding.
Conclusions From these patients we have learned that (1) extended myectomy-myotomy relieves the obstruction of extended Hirschsprung's disease; (2) aganglionic bowel after extended myectomy-myotomy acts as a passive conduit for proximally propulsed nutrients; and (3) aganglionic bowel after extended myectomy-myotomy undergoes adaptive change and is capable of absorbing life-supporting nutrients. These data demonstrate extended myectomymyotomy to be a therapeutic option for otherwise fatal extended Hirschsprung's disease, either as a potentially definitive therapy or as a putative bridge to intestinal transplantation.
504
Vol. 218-No. 4
Total or near-total intestinal aganglionosis is that variant of Hirschsprung's disease believed to be incompatible with life. Because of the uniformly fatal results in a series reported earlier,' we applied the unorthodox therapy of an extended small bowel myectomy-myotomy when caring for a similar patient.2 Strikingly, this child has done well and is completely enterally nourished. We have now gathered data on the application of this same operation to 15 additional patients afflicted similarly with small bowel Hirschsprung's disease, and they form the basis of this report.
METHODS In the Acknowledgments section, we list the names of the eight pediatric surgeons around the world whose ten cases, along with the six cared for by the authors, formed the basis of this report. Questionnaires were completed on each patient that included demographic data, presenting signs and symptoms, diagnostic procedures, operative techniques, and subsequent patient outcomes. The data were compiled, and numeric parameters were analyzed for their distribution.
RESULTS Tables 1 and 2 summarize the demographic, diagnostic, therapeutic, and current nutritional data of survivors and nonsurvivors, respectively. There were 13 white, 2 Asian, and 1 black child in this series. Eight were boys, and eight were girls. Only one patient had a family history of Hirschsprung's disease, a remarkable family in which the conditions of two brothers and the mother of this little boy had previously been diagnosed. All patients demonstrated symptoms as neonates, the median age at diagnosis being 7 days (mean, 16.4 days; range, 1 to 150 days). Bilious emesis, abdominal distention, and failure to pass meconium were the most frequent presenting signs and symptoms. Contrast enema radiographs gave variable results with demonstration of a small colon in only two patients. Upper gastrointestinal contrast radiographic results varied from a normal transit time to complete obstruction, and although a dilated small bowel was a frequent feature, visualization of a small bowel transition zone was not. A definitive histologic diagnosis was confirmed either by rectal suction biopsy or by operative biopsy, but appropriate "levelPresented at the 11 3th Annual Scientific Session of the American Surgical Association, Baltimore, Maryland, April 1-3, 1993. Address reprint requests to Moritz M. Ziegler, M.D., Division ofPediatric Surgery, Children's Hospital Medical Center, Elland and Bethesda Avenues, Cincinnati, OH 45229-2899. Accepted for publication April 9, 1993.
Myectomy for Extended Hirschsprung's Disease
505
ing" of the extent of aganglionosis was often not accomplished at an initial laparotomy and required as many as 3 procedures to confirm (median, 1 operation; mean, 1.4 operations; range, 1 to 3 operations). Three patients had no ganglion cells beyond the ligament of Treitz; ganglionated bowel below the ligament varied from 2 to 40 cm in the other 13 (mean, 13.8 cm; median, 10 cm). The myectomy-myotomy was of variable length, extending from the proximal to the aganglionic segment and continuing for variable distances of small bowel (range, 20-75 cm; median, 40 cm; mean, 44.4 cm) before ending in a stoma. This produced total small bowel conduit lengths of 38 to 80 cm (median, 50 cm; mean, 56.3 cm). One patient whose aganglionosis included the stomach had a total intestinal length myectomy, leaving the small and large bowel in situ and not forming an enterostomy. The myectomized segment was fashioned as a Thiry-Vella loop separated from the fecal stream in three cases. Patient outcomes included death in six children. The cause of death was secondary to gut-induced infection(s) and fulminant respiratory failure' and occurred 1 to 33 months after the operation (mean, 9.5 months; median, 5.0 months). The ganglionated intestinal length of nonsurvivors (mean, 9.2 cm mean; median, 8 cm) did not differ from that of the 10 children still alive (mean, 12.4 cm; median, 6.0 cm). Similarly, the length of the myotomy-myectomy segment did not differ between nonsurvivors (mean, 43.2 cm; median, 40 cm) and survivors (mean, 45.2 cm; median, 44.0 cm). Of the ten survivors, two are totally nourished by the enteral route, one who passed stools per rectum through her intact bowel and one who still has the excluded conduit. These children had only 2 and 7 cm of ganglionated jejunum at their initial leveling operation. Seven of the remaining eight receive from 20% to 80% of their calories by the enteral route, and only one child receives a minimal enteral intake.
DISCUSSION Although intestinal aganglionosis was first reported as a cause of intestinal obstruction by Harold Hirschsprung3 in 1888, the first report of total intestinal aganglionosis did not occur until 195 1.4 Thereafter, two reviews brought the world's experience to I 1 and 126 cases respectively, and the experience was extended to a 13th case in 1985.' This rare entity accounts for less than 5% to 10% of all reported cases of Hirschsprung's disease, whether in the United States or in Japan.7 8 The diagnosis and proper leveling of extensive small bowel aganglionosis is often elusive because of its rarity, the lack of diagnostic radiographs, and the lack of visible transition areas during exploration on the operating table. How-
506
Ann. Surg. * October 1993
Ziegler and Others
Table 1. SURVIVORS AFTER MYECTOMY-MYOTOMY FOR NEAR-TOTAL INTESTINAL AGANGLIONOSIS Current Patient No.
Sex
1 2 3 4 5 6 7 8 9 10
F M F M F M M M M F
Race
Age at Diagnosis
Age at Myectomy-Myotomy
Ganglionated Bowel Below LOT
Myectomy-Myotomy
W W W W W A W A W W
1 day 2 weeks 4 days 4 weeks 2 days 3 days 2 days 3 days 5 months 1 week
8weeks 5 weeks 16 weeks 8 weeks 18 days 27 days 32 weeks 8 weeks 20 weeks 8 weeks
5cm 5 cm 7 cm 0 2 cm 0 25 cm 10 cm 30 cm 40 cm
45 cm 75 cm 68 cm Total bowel 44 cm 70 cm 20 cm (Bianchi) 35 cm 20 cm 30 cm
ever, both histologic (aganglionosis) and histochemical (increased cholinesterase) tissue diagnostic criteria were preserved in those patients cared for by the authors, a contrast to the results of previous literature reports.' A male preponderance is seen in most series of Hirschsprung's disease; but with a greater extent of aganglionosis, the sex ratio is closer to 1: 1, a finding we observed and was reproduced in our review. Multifactorial inheritance in Hirschsprung's disease may give way to autosomal recessive inheritance in extended small bowel aganglionosis,9 but except for the one dominant kindred in this series, we could not confirm that observation. Gastrointestinal myotomy as a treatment for anatomic or functional bowel obstruction was first applied in the early part of this century at the level of the esophagus'0 or pylorus."12 In an article read before the American Surgical Association in 1927, segmental colonic rectosigmoid myotomy was described for the relief of the obstruction at what was incorrectly interpreted to be the transition zone rectosigmoid type of Hirschsprung's disease.'3 Intestinal myotomy as a technique to relieve anatomic obstructions in the small bowel has been reported.'4 In the same year, rectal myectomy was described as a useful technique for the management of short segment Hirschsprung's disease"5 and for func-
Extent of
Parenteral Enteral Calories
0/100% 55%/45%
66%/33% 66%/33% 0/100% 95%/5% 50%/50% 80%/20% 60%/40% 20%/80%
tional constipation.'6 One of us (L.W.M.) in 1968 reported on a series of five infants with Hirschsprung's disease involving the whole colon and extending for variable lengths into the small bowel.'7 The following observation was made, "The fact that a certain degree of success has been reported in treating Hirschsprung's Disease by means of a rectosigmoid myotomy suggests the possibility that the fecal stream might be adequately propelled by a gut in which a longitudinal strip is devoid of ganglion cells. It then follows that perhaps this longitudinal strip might be created with aganglionic colon which would then permit recapture of the reabsorptive qualities of the colon and result in a more nearly normal stool than could be expected from an anal ileostomy." This report formed the basis for the extended Martin-Duhamel procedure, such an elongated side-to-side anastomosis of ganglionated small bowel to aganglionic colon being a variant of a colon myectomy. Myectomy-myotomy was also coupled with partial colectomy as a technique for treating extended colonic Hirschsprung's
disease.'8 Many reports from the United States,'7"1920 Eu-
rope21,22 and the Far East23 have detailed the high mortality rate of Hirschsprung's disease involving the total colon and extending into the small bowel for variable
Table 2. NONSURVIVORS AFTER MYECTOMY-MYOTOMY FOR NEAR-TOTAL INTESTINAL AGANGLIONOSIS Patient No.
Sex
Race
Age at Diagnosis
Age at Myectomy-Myotomy
Ganglionated Bowel Below LOT
Extent of Myectomy-Myotomy
Age at Death
11 12 13 14 15 16
F F M F F M
W W W B W W
15days 8 days 9 days 11 days 2 days 7 days
17 days 23 days 5 weeks 11 days 4 weeks 4 weeks
3 cm 6 cm 10 cm 26 cm 0 10 cm
57 cm 32 cm 60 cm 30 cm 40 cm 40 cm
33 months 37 days 12 months 7 months 1 month 3 months
Vol. 218 d No. 4
lengths. These reports and those detailing the futility of still more extensive small bowel Hirschsprung's disease 46 have not recognized the importance of normally maintained absorptive function in the aganglionic colon'7 and aganglionic ileum24 ifthe intestinal obstructive component is relieved. With the additional knowledge learned from the short bowel syndrome literature, a minimal small bowel length and its adaptive component, which is compatible with survival, can be predicted,25'26 and a logical treatment plan can be devised for such children.
Diagnosis A high index of suspicion is indicated to make the diagnosis of total or near-total intestinal aganglionosis because there are no absolute clinical criteria that are reproducible except the presence of nonspecific signs of intestinal obstruction. Radiographs may demonstrate either dilated or normal-caliber small and large bowel, and a transition zone will rarely be seen. A family history of Hirschsprung's disease should raise the level of diagnostic suspicion.
Leveling A rectal punch biopsy will show positive findings for Hirschsprung's disease by either histologic (aganglionosis) or histochemical techniques (increased acetylcholinesterase) as will stepwise seromuscular biopsies obtained at laparotomy. However, interpretative errors from frozen-section diagnoses have been frequent in this series. Therefore, it may be prudent during surgery to perform multiple leveling seromuscular biopsies and await a definitive histologic and histochemical analysis before subjecting the patient to a bowel resection or an inadvertent stomal placement. The transition zones in this series varied from total aganglionosis to ganglionated bowel extending below the ligament of Treitz for up to 40 cm; however, the bowel within 2 to 15 cm of the ligament is the most fruitful site for such a transition. Closer sequential seromuscular biopsies coupled with accurate antimesenteric border length measurements are needed to locate the ganglionated-aganglionic transition zone accurately.
Myectomy for Extended Hirschsprung's Disease
507
permit proximal propulsive forces to be able to push luminal content through, resulting in a functional obstruction and or intraluminal stasis. At the selected site, the bowel should exit as a stoma, and venting of distal small bowel and colon may be optimal. Residual distal small and large bowel should not initially be discarded.
The Myectomy-Myotomy Whether a seromuscular 1-cm strip of tissue to the level of submucosa is excised or whether a Ramstedt type of myotomy is done is not critical because both are effective in relieving aganglionic segment obstruction (see Figures 1 and 2). The longitudinal elevation of an excised seromuscular strip whose plane ofdissection was augmented by the intramural injection of saline or 1:100,000 epinephrine was technically easier for us. In the authors' personal cases, although the myectomized bowel was left in continuity with the proximal bowel, there were three cases in this series where the myectomized conduit was isolated from the in situ bowel as a Thiry-Vella loop. It is one of these cases that has progressed to complete enteral nutrition. Enterotomies created during the myectomy-myotomy can be expected and must be recognized and closed. A final bowel length
\
~~7cm FROM LIGAMENT
10 cm,
.
MYECTOMY
Estimation of Needed Bowel Length Using data previously published25'26 and that from this series, it would be ideal to have a minimum of 20 to 40 cm of ganglionated small bowel (without the presence of an in situ ileocecal valve) in continuity in the fecal stream. More length would be preferable, but there will be a small bowel passive conduit length "too long" to
Figure 1. Techniques of a 10-cm jejunal myectomy as done in case 1 of this series. Reprinted with permission from J Pediatr Surg.
508
Ziegler and Others
Ann. Surg. * October 1993
END OSTOMY
t =4/400cm \
~~~~~~MYOTOMY
Figure 2. Techniques of a 40-cm jejunal myotomy coupled with a proximal myectomy as done in case 1 of this series. Reprinted with permission from J Pediatr Surg.
of proximal ganglionated bowel plus myotomy-myectomy segment theoretically should approximate 20 to 40
but in this series, this length was usually extended from 40 cm to 80 cm of total intestine before exiting as
cm, an
home care process or if severe complications oftotal parenteral nutrition, such as vascular access difficulty or progressive cholestatic liver disease supervene, then alternate operative procedures may need to be considered. Several side-to-side anastomotic options, which have been described,17'27'28 may take advantage ofthe absorptive function in aganglionic bowel and are a consideration. Similarly, we applied the intestinal lengthening operation,29 a form of "full-thickness" myotomy-myectomy in one patient with successful enhancement of enteral intake. Strikingly, the first case in this series underwent a Martin-Duhamel procedure20 in which a side-toside anastomosis was created between aganglionic small bowel treated by myectomy-myotomy and aganglionic rectosigmoid colon. The result was an enterally nourished, fecally continent child who is both physically and
psychologically healthy. Aganglionic small or large bowel undergoing myectomy-myotomy has the ability to adapt, as demonstrated by growth in both length and diameter and, therefore, mucosal absorptive area. Such bowel is capable of nutrient absorption. Although such bowel is not capable of effective peristalsis, the intraluminal content can be "pushed through" this passive conduit if a "normal" position of ganglionated bowel lies proximally. In the best circumstances, the myectomy-myotomy may be a definitive therapy, and in this series, 10 of 16 patients survived (62.5%) by contrast with no historic survivors. Furthermore, 2 of the 15 are currently normal and receiving total enteral nutrition. For a less optimal outcome, a myectomy-myotomy may carry the infant for several months or even years of good health and somatic growth, and it can serve as a functional bridge to intestinal transplantation.
ostomy.
Early Postoperative Care These patients require the best management of short bowel syndrome that can be provided, which care includes both aggressive parenteral and enteral nutrition. The best circumstance will see early function ofthe enterostomy with daily output, and in the face ofsuch output, early enteral nutrition is imperative to augment adaptive changes in both the ganglionic and aganglionic bowel and to stimulate bile drainage, thus preventing the cholestatic liver disease that characterizes parenterally fed infants with short bowel syndrome. Aggressive medical management of short bowel syndrome,25 including optimum parenteral nutrition, should be followed by a gradual transition to enteral caloric support, usually a transition requiring the constant infusion of an elemental formula. After 6 to 12 months have passed in this slow
Acknowledgments The following pediatric surgeons graciously contributed their cases to this review: Thomas Basil, M.D., Syracuse, New York; William
Brennom, M.D., St. Paul, Minnesota; Mary Fallat, M.D., Louisville, Kentucky; Howard Filston, M.D., Knoxville, Tennessee; George Holcomb III, M.D., Nashville, Tennessee; L. Lassaletta, M.D., Madrid, Spain; Eiji Nishijima, M.D., Kobe, Japan; and Joseph Zerella, M.D., Phoenix, Arizona. Drs. Nishijima and Zerella each contributed two cases.
References 1. Caniano DA, Ormsbee HS III, Polito W, et al. Total intestinal aganglionosis. J Pediatr Surg 1985; 20:456-460. 2. Ziegler MM, Ross AJ III, Bishop HC. Total intestinal aganglionosis: a new technique for prolonged survival. J Pediatr Surg; 1987;
22:82-83. 3. Hirschsprung H. Stuhltragheit neugeborener in folge von dilatation und hypertrophie des Colons. Jahrb Kinderheilkd 1888; 27:1-7.
Vol. 218 -No. 4
4. Bodian M, Carter CO, Ward BCH. Hirschsprung's disease. Lancet 1951; 1:302. 5. Talwalker UC. Aganglionosis of the entire bowel. J Pediatr Surg
6. 7.
8. 9. 10. 11.
12. 13. 14.
15. 16. 17.
18. 19.
20. 21.
22. 23. 24.
25. 26. 27. 28. 29.
1976; 11:213-216. Saperstein L, Pollack J, Beck AR. Total intestinal aganglionosis. Mt Sinai J Med 1980; 47:72-73. Kleinhaus S, Boley SJ, Sheran M, Sieber WK. Hirschsprung's disease: a survey of the members of the surgical section of the American Academy of Pediatrics. J Pediatr Surg 1979; 14:588-597. Ikeda K, Goto S. Diagnosis and treatment of Hirschsprung's disease in Japan. Ann Surg 1984; 199:400-405. MacKinnon AE, Cohen SJ. Total intestinal aganglionosis, an autosomal recessive condition? Arch Dis Child 1977; 52:898-899. Heller E. Extramukose cardioplastic beim chronischen cardiospasmus mit dilatation des oesophagus. Milt Grenspch Med Chir 1913; 27:141. Dufour H, Fredet P. La stenose hypertrophique du pylore chez le nourisson et son traitement chirurgical. Rev Chir 1908; 37:208253. Ramstedt C. Zur operation der angeborenen pylorus stenose. Med Klin 1912; 8:1702-1705. Martin E, Burden VG. The surgical significance of the rectosigmoid sphincter. Ann Surg 1927; 86:86-91. Tygart RH, Glass WW. Intestinal myotomy. Arch Surg 1966; 92:304. Lynn HB. Rectal myectomy for aganglionic megacolon. Mayo Clin Proc 1966; 41:289-295. Bentley JFR. Posterior excisional ano-rectal myotomy in management ofchronic fecal accumulation. Arch Dis Child 1966; 41:144147. Martin LW. Surgical management of Hirschsprung's disease involving the small intestine. Arch Surg 1968; 97:183-189. Kasai M, Suzuki H, Watanabe K. Rectal myotomy with colectomy: a new radical operation for Hirschsprung's disease. J Pediatr Surg 1971; 6:36-41. Walker AW, Kempson RL, Ternberg JL. Aganglionosis of the small intestine. Surgery 1966; 60:449-457. Martin LW. Surgical management of total colonic aganglionosis. Ann Surg 1972; 176:343-346. Coran AG, Bjordal R, Eek S, Knutrud 0. The surgical management of total colonic and partial small intestinal aganglionosis. J Pediatr Surg 1969; 4:531-537. N-Fekete C, Ricour C, Martelli H, et al. Total colonic aganglionosis (with or without ileal involvement): a review of 27 cases. J Pediatr Surg 1986; 21:251-254. Ikeda K, Goto S. Total colonic aganglionosis with or without small bowel involvement: an analysis of 137 patients. J Pediatr Surg 1986; 21:319-322. Kottmeier PK, Jongco B, Velcek FT, et al. Absorptive function of the aganglionic ileum. J Pediatr Surg 1981; 16:275-278. Cooper A, Floyd TF, Ross A, et al. Morbidity and mortality of short bowel syndrome acquired in infancy: an update. J Pediatr Surg 1984; 19:711-718. Wilmore DW. Factors correlating with a successful outcome following extensive intestinal resection in newborn infants. J Pediatr 1972; 80:88-95. Kimura K, Nishijima E, Marajit, et al. Extensive aganglionosis: further experience with the colonic patch graft procedure and longterm results. J Pediatr Surg 1988; 23:52-56. Stringel G. Extensive intestinal aganglionosis including the ileum: a new surgical technique. J Pediatr Surg 1986; 21:667-670. Bianchi A. Intestinal loop lengthening-a technique for increasing small intestinal length. J Pediatr Surg 1980; 15:145-151.
Myectomy for Extended Hirschsprung's Disease
509
Discussion DR. J. ALEX HALLER, JR. (Baltimore, Maryland): Mr. President, Ladies and Gentlemen, we would indeed be poor hosts in Baltimore if this excellent paper were not vigorously discussed. Although I did not have a chance to see the manuscript, I listened with great interest. One of the important principles that Dr. Ziegler has brought to us is that not only is this technique important in preventing obstruction in these small patients, but it adds an appropriate absorptive surface that is maintained. Dr. Ziegler, can you tell us whether any studies have been done to show the nature ofthat absorption? Whether it is occurring at the same level above in the small intestine, whether there is modification for absorption along the gastrointestinal tract of these children? My second question is, is it necessary to use any type of enteric antibiotics to decrease overgrowth of bacteria because of this prolonged transit time? DR. JAMES A. O'NEILL, JR. (Philadelphia, Pennsylvania): I think I have an advantage over the rest ofthe individuals in this room in that I saw the first three patients treated in Philadelphia. Now, despite the fact that Dr. Ziegler gave you a disclaimer and indicated that this was a reinvention of the wheel, it was only shortly before this meeting, I am sure, when he prepared that slide that it was termed that. It was well-conceived before it was first done: in fact, I can very well remember the clinical conference at which the little girl who was the first patient treated was discussed. I think it's a good example of courage in a surgeon to take a principle that basically was the anorectal myomectomy, a very short interruption of the external muscle of the bowel, and to conceptualize that perhaps this might work if extended throughout the gastrointestinal tract. Now, I think it's interesting in that while there has been a great deal of stress placed on the fact that absorption occurs, I think even more interesting is the fact that motility appears to be enhanced as well. As is obvious, all portions of the intestine that have aganglionosis have a failure to transmit peristalsis in a normal fashion, but somehow by interrupting the circumference of the muscle of the bowel there then comes an opportunity to at least improve it, even if it doesn't become entirely normal. If it wasn't at least improved to a clinically acceptable level, all of these patients would die of intestinal sepsis, which proves not to be the case. Now, as we look at the data, there appears to be one patient in eight who can get to the point of enteral independence. The first question I have for Dr. Ziegler is: As you go along in the course of management of such patients and see some improve and some not, do you have any ideas now as to when to give up and to move on to intestinal transplantation? That's the other obvious solution for these children, although obviously not as good if you can make the native bowel work satisfactorily. The second question is: Do you feel that the so-called Bianchi procedure is perhaps a better solution for many of these children? And if so, which ones would you select in this regard?
