Outcome and Intestinal Adaptation Short-Bowel Syndrome ByKeith

E. Georgeson

and Charles

in Neonatal

W. Breaux,

Jr

Birmingham, Alabama 6 We reviewed 52 consecutive patients with short-bowel syndrome (SBS) treated with long-term parenteral nutrition (PN) from 1978 through 1990. The SBS etiologies included necrotizing enterocolitis (NEC) in 28 patients (50%). abdominal wall defects in 11 (22%). jejunoileal atresia in 8 (12%). midgut volvulus in 4 (8%). Hirschsprung’s disease in 3 (8%). and segmental volvulus and cloaca1 exstrophy in 1(2%) each. The average initial small bowel length was 48.1 cm, and only 31% of the patients retained an ileocecal valve (ICV). The mean duration of PN therapy was 18.8 months, and 39 patients (75%) were successfully weaned from it. Forty-three patients (83%) survived. Significant differences between the initial 20 patients treated from 1978 through 1984 and the next 32 from 1985 through 1990 were duration of PN therapy (25.1 v 11.4 months; P = .04), incidence of PN-associated jaundice (80% Y 31%; P = .OOl), and survival (85% Y 94%; P = .02). NEC patients had a significantly lower mean birthweight than those with other etiologies (mean, 1,387 v 2,544 g; P < .OOOl) but did not differ in initial small bowel length, ICV retention rate, duration of PN treatment, incidence of successful PN weaning, or outcome. The presence of an ICV did not correlate with successful PN weaning but did affect the mean duration of PN therapy (7.2 months with ICV Y 21.6 months without; P = .03). Patients with 40 cm or less of initial small bowel spent a longer time on PN (24.1 v 10.6 months; P = .04) and had a lower weaning rate from it (57% v 88%; P = .02) than did those with more small bowel. The ratio of initial small bowel length to birthweight did not correlate with the duration of PN therapy in survivors successfully weaned from PN. We conclude that the outcome and morbidity of patients with SBS on long-term PN has improved. In addition, the absence of an ICV prolongs the length of PN therapy but does not determine successful weaning from it.

Copyright o 1992 by W.B. Saunders Company INDEX WORDS: Short-bowel syndrome; tion; intestinal adaptation; cholestasis.

parenteral

nutri-

S

HORT-BOWEL syndrome (SBS) in neonates does not lend itself to precise definition. Some authors have described neonatal SBS relative to the length of remaining jejunoileum after resection.‘.’

From the Department of Surgety The Children’s Hospital of Alabama, and the Division of Pediatric Surgery, the Department of Surgery, The Universiv ofAlabama School of Medicine, Birmingham, AL. Presented at the 22nd Annual Meeting of the American Pediatric SurgicalAssociation,Lake Buena l&a, Florida, May 15-18, 1991. Address reprint requests CO Keith E. Geotgeson, MD, Depatiment of Surgery, The Children’s Hospital of Alabama, 1600 7th Ave S, Birmingham, AL 35233. Copyright o 1992 by W. B. Saunders Company 0022-3468/92/2703-0014$03.OOlO

344

Others have defined neonatal SBS to be present if greater than 50% of the jejunoileum has been resected.3*4 Both of these definitions do not take into account the growth potential of the remaining small bowel, the presence or absence of the ileocecal valve (ICV), the length of retained colon, and the differences in diseases causing the SBS (eg, patients with necrotizing enterocolitis [NEC] may have patchy involvement of the remaining bowel that can affect absorption). For this patient review, we defined neonatal SBS as a symptom complex of malabsorption associated with substantial shortening of the jejunoileum and requiring total or supplemental parenteral nutrition (PN) for at least 3 months to achieve adequate growth and development.’ MATERIALS

AND

METHODS

The records of 52 consecutive patients treated from 1978 through 1990 for neonatal SBS as defined previously were reviewed. The first 20 patients were treated by the senior author (K.E.G.) at the Loma Linda University Medical Center from 1978 through 1984, and the subsequent 32 were treated by him at the Children’s Hospital of Alabama from 1985 through 1990 following his move to Birmingham. All patients with neonatal SBS managed by the senior author during this time interval were included in the study. During the diarrhea] stage of SBS, PN without enteral feedings was used. An elemental diet was introduced in increasing volumes as the patient’s small bowel adapted to enteral feedings. The PN was delivered through a cuffed Silastic central venous catheter, initially continuously and then converted to a daily cycle as soon as the patient stabilized and weighed at least 2,000 g. Appropriate calories, protein, fat, vitamins, and minerals were delivered as determined by each patient’s anthropometric measurements and adjusted according to growth and development. The parents were taught to deliver cycled PN, and the patients were discharged home when they were stable and weighed at least 3,000 g (usually aged 2 to 5 months). The patients were followed in the SBS/Home PN Clinic. Anthropometrics, intake and output, selected serum chemistries, proteins, liver enzymes, triglycerides, and hemograms were closely monitored. The patients were slowly weaned from PN as they increased their tolerance for enteral nourishment. Many patients were more tolerant of continuous drip feedings by nasogastric, nasoduodenal, or gastrostomy tubes than oral or bolus tube feedings. An attempt was made to keep the daily number of stools less than 10 and the stool pH above 5.0. Cholestyramine, cimetidine, loperamide, paregoric, and oral neomycin were administered when clinically appropriate. The majority of patients went through a period when they refused oral feedings but all such patients responded to a program of oral stimulation and feeding therapy. The central venous catheters were left in place for 2 to 3 months after cessation of all

PN as a precaution against an exacerbation of malabsorption. Statistical calculations were performed with True Epistat soft-

Journalof Pediatric Surgery, Vol 27, No 3 (March), 1992: pp 344-350

NEONATAL SHORT-BOWEL

SYNDROME

345

ware (EPISTAT Services, Richardson, TX) on an IBM Personal System/2 model 30-286 computer. Student’s t test was used to compare means of two samples. Fisher’s exact test was used to compare percentages between two samples. The degree of concordance between two matched samples was measured with Pearson’s correlation coefficient. All probabilities were calculated in a two-tailed fashion, and significance was set at the P = .05 level.

The diagnoses resulting in SBS among the 52 patients were NEC in 26 (50%), omphalocele or gastroschisis in 11 (21%), jejunal or ileal atresia in 6 (12%), midgut volvulus in 4 (8%), Hirschsprung’s disease in 3 (6%), and segmental volvulus and cloaca1 exstrophy in 1 (2%) each. The mean initial small bowel length was 48.1 + 20.6 cm (mean 2 SD), and only 16 patients (31%) retained an ICV. The duration of PN therapy averaged 16.6 rfr 18.7 months, and 39 patients (75%) were successfully weaned from it. Twenty-six patients (50%) developed PN-associated jaundice. Overall, 43 patients (83%) survived. The causes of death in the other nine patients are listed in Table 1. The 20 SBS patients treated from 1978 through 1984 are compared with the 32 treated from 1985 through 1990 in Table 2. Significant clinical differences between these earlier and more recent groups include average duration of PN (25.1 v 11.4 months), incidence of PN-associated jaundice (80% v 31%), and survival (65% v 94%). Survival curves for both treatment eras are shown in Fig 1. In Table 3, the 26 patients with SBS from NEC are compared with the 26 patients with SBS due to other causes. NEC patients had a significantly lower mean birthweight than did those with other diagnoses (1,367 v 2,544 g) but did not differ from them in average initial smaI1 bowel Length, ICV retention rate, mean duration of PN therapy, successful weaning from PN, incidence of PN-associated jaundice, or survival. The 16 SBS patients who retained an ICV are compared with the 33 who lost theirs in Table 4 (note that in three other patients the presence or absence of an ICV was not documented). Those patients

Causes

of Death

Versus 1985 to 1990

Birthweight (g)

Period

1978.1984

1985.1990

(n = 20)

(n = 32)

2,083 + 612

1.892 f 087

P Value

NS NS NS

Small bowel length (cm)

49.8 t 22.2

46.9 + 19.3

No. with retained ICV (%)

6 (30) 25.1 2 25.9

11.4 + 8.7

No. weaned from PN (%)

16 (80)

23 (72)

NS

No. with jaundice (%)

16 (80)

10 (31)

.OOl

No. of survivors (%)

13 (65)

30 (941

-02

Duration of PN therapy (mo)

RESULTS

Table 1. Causes of Death by Treatment

Table 2. SBS Patients Treated From 1978 to 1984

10 (31)

.04

NOTE. Data given as mean + SD unless otherwise indicated. Abbreviations: ICV, ileocecal valve; PN, parenteral nutrition.

without an ICV were no less successful in weaning from PN, but they did spend a significantly longer time on PN than did the patients with an ICV (mean, 21.6 v 7.2 months). In Table 5, the 21 patients with very short SBS (ie, 40 cm small bowel or less) are compared with the 26 with more jejunoileum (note that in 5 other patients the small bowel was not measured). Although not significantly different, there was a trend toward lower average birthweight and ICV retention rate in the patients with very short SBS. Their mean duration of PN therapy was longer than those with more small bowel (24.1 v 10.6 months), and there was a significant difference in successful weaning from PN between the two groups (57% v 88%). The 36 survivors who successfully weaned from PN and whose bowel length was documented were separately studied for concordance of their initial small bowel length to the duration of PN therapy (Fig 2). A trend toward a negative correlation between bowel length and time on PN was found (r = - .3 1, P = .09). However, when the initial small bowel length was indexed for the patient’s size by dividing small bowel length by birthweight, no correlation was found between this ratio and the duration of PN therapy (Fig 3; r = -.18, P = .37). 96 Survival 100 I

‘85’90

60 ‘78-‘04

No. of Patients

1978-1984

40

Sepsis

2

Hepatic failure

2

Acute abdomen

1

Vena caval injury

1

Voluntary withdrawal of treatment

1

20

c I 0

1985-1990 Sudden infant death syndrome

1

Respiratory failure

1

12

24

36

48

60

72

Months

Fig 1. SES patient survival by treatment eta.

84

96

346

GEORGESON AND BREAUX

Table 3. NEC Versus Non-NEC SBS Patients NEC (n = 26)

Birthweight (g)

Table 5. SBS Patients With +Kl cm Small Bowel Versus >40 cm Small Bowel

Non-NEC (n = 261

P Value

540 cm

>40cm

(n = 21)

(n = 26)

P Value

1,653 k 731

2,083 f 746

NS (.07)

1,367 + 506

‘2,544 +- 605

Small bowel length (cm)

49.5 + 20.1

46.8 +- 21.1

NS

Birthweight (g)

No. with retained ICV (%)

7 (27) 14.5 2 12.2

9 (35) 18.8 + 23.2

NS

Small bowel length (cm)

29.9 + 9.2

62.9 k 15.1

NS

No. with retained ICV (%)

12 (46)

NS (.07)

No. weaned from PN (%)

20 (77)

19 (73)

NS

Duration of PN therapy (mo)

4 (19) 24.1 + 27.0

10.6 2 7.3

.04

No. with jaundice (%)

13 (50)

13 (50)

NS

No. weaned from PN (%.)

12 (57)

23 (88)

.02

No. of survivors (%)

21 (81)

22 (85)

NS

No. with jaundice (%)

13 (62)

11 (42)

NS

No. of survivors (%)

17 (81)

28 (81)

NS

Duration of PN therapy (mo)

.OOOl

NOTE. Data given as mean f SD unless otherwise indicated. Abbreviations:

NEC, necrotizing enterocolitis; ICV, ileocecal valve;

PN, parenteral nutrition.

DISCUSSION

The majority of the patients were discharged from the hospital 2 to 5 months after birth. As soon as an infant was identified as malabsorbing secondary to a shortened small bowel, PN was initiated and cycled.6 Most infants cycled after achieving a weight of 2,000 g. Those infants who were intolerant of rate changes using a 20-minute cycle length frequently tolerated rate changes if they were extended to 40-minute intervals. Once a patient was stabilized on a cycling schedule, the parents were taught to administer the PN at home, based on a philosophy that early discharge from the hospital is advantageous because it separates the vulnerable infant from the hospital flora, integates the infant into the family, and is less expensive. If the infant was in the diarrhea1 stage of SBS at the time of discharge, enteral feedings were delayed and initiated as an outpatient. Most of the patients under review were given an elemental diet to optimize absorption in the remaining jejunoileum. There is now evidence that a diet containing complex carbohydrates and fat is tolerated as well as an elemental diet, is more palatable, and may stimulate the bowel to faster adaptation.7-9 Further studies to determine the ideal diet for neonates with SBS are needed. Bowel adaptation and somatic growth received priority in the early phases of nutritional support. Although many patients did not feed orally, this lack of interest in oral feeding was always reversed. Table 4. SBS Patients With an ICV Versus Those Without an ICV ICV (n = 16)

Birthweight (g) Small bowel length (cm) Duration of PN therapy (mo)

1,986 + 792

No

Enteral feedings were advanced as tolerated. Drip feedings were often better tolerated than bolus feedings.* A patient’s tolerance for enteral feedings was not steadily progressive. The progress made toward the end of intestinal adaptation was usually more rapid than in the earlier stages. Cimetidine was administered during the diarrhea1 stage of SBS. Once the patient was able to tolerate enteral feeds, the cimetidine was discontinued. Loperamide and cholestyramine were sometimes useful and were titrated appropriately.” The position of the central venous catheter used for PN was carefully monitored. A catheter that is improperly positioned in the internal jugular or subclavian vein can induce thrombosis of the vein and lead to superior vena cava syndrome. A fibrin sleeve left to enlarge around the catheter and attach to the caval or atria1 wall can also lead to this complication. Additionally, the presence of a fibrin sleeve increases the incidence of catheter-related sepsis.“.‘” Once initiated, such an infection can be difficult to clear in the presence of a fibrin sleeve. For these reasons, the development of a fibrin sleeve around the catheter was inhibited by the addition of heparin (1 U/mL) to the infused PN solutions. A patient was suspected of

100

60

Bowel /

length

(cm)

** * IIx *+ w

so4

-8 I

*

I I

Y

ICV

(n = 331

P Value

1,946 k 819

NS

49.3 f 14.4

46.5 + 22.8

NS

7.2 -c 4.5

21.6 t 21.6

.03

No. weaned from PN (%)

13 (81)

24 (73)

NS

No. with jaundice (%)

8 (50) 12 (75)

17 (52)

NS

28 (85)

NS

No. of survivors (%)

NOTE. Data given as mean -t SD unless otherwise indicated. Abbreviations: ICV, ileocecal valve; PN, parenteral nutrition.

NOTE. Data given as mean + SD unless otherwise indicated. Abbreviations: ICV, ileocecal valve; PN, parenteral nutrition.

0

c 0

) _---_i_b_

20

40

i

?io,

80

100

Time on PN Fig 2. Initial small bowel length versus duration of PN therepy in SBS survivors successfully weaned from PN [r = -.31, P = .09).

NEONATAL

** I Bowel

,Or-

5o 40

SHORT-BOWEL

IengthBirthweight

347

SYNDROME

(cm/kg) -__

*

*t

*



4

o

+

0

~~

-,-

10

___+_

_

20

_+_

_+_

30

Time

+._

40

50

_

60

on PN (mo)

Fig 3. Initial small bowel length/bitiweightversus duration of PN therapy in SBS survivors successfully weaned from PN (r = -.18, P = .37).

developing a fibrin sleeve if the catheter permitted infusion but not aspiration, and the diagnosis was confirmed with a cathetergram. If present, urokinase was used to lyse the sleeve in its early stages. Some patients with a prediliction for fibrin sleeve formation were given routine weekly or monthly instillations of urokinase. Those patients with a large fibrin sleeve usually required a 4-hour infusion of urokinase to lyse the sleeve.14 Three of the seven deaths in the earlier group of patients were associated with catheter complications, including sepsis (Table 1). Aggressive prevention and management of catheter complications seemed to eliminate catheter-related deaths in the more recent group of infants. This study demonstrated a sharp decline in the incidence of PN-associated cholestatic jaundice in the more recent group of patients. The clinical management of SBS in both time periods was similar. The primary difference in the PN was the use of a specific pediatric amino acid solution in the later time period as compared with a nonpediatric mixture in the earlier period. Balanced amino acid solutions specifically formulated for infants have been shown to decrease the incidence of cholestatic jaundice.15 We have recently considered the translocation of intestinal bacteria as one of the causes of cholestatic jaundice in patients with SBS. Infants receiving PN without entera feeding are especially vulnerable to the translocation of intestinal bacteria.” A dilated, hypomotile, small bowel with an absent ICV allowing colonic backwash into the small intestine may add to the risk of bacterial translocation. The translocated bacteria are carried to the liver by the portal circulation or by way of the lymphatics into the peripheral circulation. Early enteral feeding of nonelemental formulae, balanced parenteral protein sources rich in glutamine, efforts to improve intestinal motility, and

selective decontamination of the gastrointestinal tract may be helpful in decreasing colonization and mucosal attachment of hostile bacteria and thereby decreasing the incidence of cholestatic jaundice by deterring bacterial translocation.‘7-19 One of the patients in this series with a short, dilated small bowel developed progressive cholestatic jaundice. Selective decontamination with oral neomycin quickly ameliorated the jaundice which returned when the medication was inadvertantly discontinued. When the oral neomycin was resumed, the jaundice again resolved. Besides the use of a specific pediatric amino acid solution and prevention and treatment of intestinal bacterial translocation, other factors such as prevention and treatment of sepsis, early cycling of PN, and judicious early introduction of enteral feedings may have also contributed to the reduction in the incidence of cholestatic jaundice in the more recent group of infants. The decrease in this potential cause of death along with the elimination of deaths due to catheter complications account for the improvement in survival seen in the more recent group of infants, especially in the first 2 years of treatment (Fig 1). Several of the patients in the more recent group have undergone intestinal lengthening using the Bianchi technique.‘O.“’ This procedure has been most helpful in patients with a dilated, hypomotile jejunoileum. Dilatation and the associated sluggish intestinal motility seem to be related to the adaptation process. The Bianchi procedure improved motility in these patients, allowing them to resume enteral feedings. The duration of PN therapy averaged over twice as long in the earlier group of patients than in the later group. Two patients in the earlier group developed intractable intestinal pseudoobstruction and remained solely on PN therapy for many years. These two patients significantly lengthened the average time of PN therapy for the earlier group. The more recent group of patients include five infants who are expected to wean from PN but who have not yet completed their intestinal adaptation to enteral feedings. These patients shortened the average time on PN therapy for the later group. Patients without an ICV took 3 times as long to undergo intestinal adaptation than those with an intact ICV. Perhaps the association of a longer segment of colon with an ICV explains part of the faster adaptation. There is evidence that the colon absorbs some nutrients.“,” The inhibition of bacterial reflux from the colon into the small bowel and the slowing of small bowel motility may have also contributed to the faster adaptation in patients with an ICV.L4 The influence of the ICV relative to small

348

GEORGESON AND BREAUX

intestinal absorption and adaptation and the absorptive contribution of the remaining segment of colon in SBS patients deserve further study. Infants with very short SBS took longer to wean from PN. There was no increased incidence of cholestatic jaundice in this group of patients, and their mortality was no greater than SBS patients with more small bowel. Touloukian and Smith speculated that premature infants with SBS might do better than full-term infants with the same length of jejunoileum because of greater bowel growth potential.25 In the present patients who survived and weaned from PN, the ratio

of initial small bowel length to birthweight did not correlate with duration of PN therapy and, thus, we did not confirm their conjecture. In summary, the outcome and morbidity of patients with neonatal SBS on long-term PN is improving. Cholestatic jaundice and central venous catheter complications are no longer insolvable problems in these patients. The absence of an ICV prolongs the length of PN therapy required before adaptation occurs but does not determine the successful weaning from it. Infants with very short SBS take longer to wean from PN. The overall prognosis for most infants with SBS is good.

REFERENCES 1. Wilmore DW: Factors correlating with a successful outcome following extreme intestinal resection in newborn infants. J Pediatr 80:88-95,1972 2. Rickham PI? Massive small intestinal resection in newborn infants. Ann R Co11Surg Engl41:480,1967 3. Grosfeld JL, Rescorla FJ, West KW: Short bowel syndrome in infancy and childhood: Analysis of survival in 60 patients. Am J Surg 151:41-46, 1986 4. Cooper A, Floyd TF, Ross AJ III, et al: Morbidity and mortality of short-bowel syndrome acquired in infancy: An update. J Pediatr Surg 19:711-718,1984 5. Schwartz MZ, Maeda K: Short bowel syndrome in infants and children. Pediatr Clin North Am 32:1265-1279,198s 6. Young EA, Cioletti LA, Winborn WB, et al: Comparative study of nutritional adaptation to defined formula diets in rats. Am J Clin Nutr 33:2106-2110,198O 7. Levy E, Frileux P, Goldberg J, et al: Modification of GI transit comparing continuous enteral support versus a fractionated administration in a digestive ICU (60 cases). Presented at the Xth Aspen Congress, Dallas, TX, February 9-12,1986 8. Levy E, Frileux P, Sandrucci S, et al: Continuous enteral nutrition during the early adaptive stage of the short bowel syndrome. Br J Surg 75:549-553,1988 9. Maini B, Blackburn GL, Bistrian BR, et al: Cyclic hyperalimentation: An optimal technique for preservation of visceral protein. J Surg Res 20:515-525,1976 10. Tilson MD: Pathophysiology and treatment of short bowel syndrome. Surg Clin North Am 60:1273-1284,198O 11. King DR, Komer M, Hoffman J, et al: Broviac catheter sepsis: The natural history of an iatrogenic infection. J Pediatr Surg 20:728-733,198s 12. Ross P Jr, Ehrenkranz R, Kleinman CS, et al: Thrombus associated with central venous catheters in infants and children. J Pediatr Surg 24:253-256,1989 13. Grisoni ER, Mehta SK, Connors AF: Thrombosis and

infection complicating central venous catheterization in neonates. J Pediatr Sug 21:772-776,1986 14. Curnow A, Idowu J, Behrens E, et al: Urokinase therapy for Silastic catheter-induced intravascular thrombi in infants and children. Arch Surg 120:1237-1240,1985 15. Beck R: Use of a pediatric parenteral amino acid mixture in a population of extremely low birth weight neonates: Frequency and spectrum of direct bilirubinemia. Am J Perinatol7:84-86.1990 16. Alverdy JC, Aoys E, Moss GS: Total parenteral nutrition promotes bacterial translocation from the gut. Surgery 104:185190,1988 17. O’Dwyer ST, Smith RJ, Hwang TL, et al: Maintenance of small bowel mucosa with glutamine-enriched parenteral nutrition. J Parenter Enter Nutr 13:579-585, 1989 18. Burke DJ, Alverdy JC, Aoys E, et al: Glutamine-supplemented total parenteral nutrition improves gut immune function. Arch Sug 124:1396-1399,1989 19. Capron JP, Herve MA, Gineston JL, et al: Metronidazole in prevention of cholestasis associated with total parenteral nutrition. Lancet 1:446-447,1983 20. Bianchi A: Intestinal loop lengthening: A technique for increasing small intestinal length. J Pediatr Sug 15:145-151,198O 21. Aigrain Y, Cornet D, Cezard JP, et al: Longitudinal division of small intestine: A surgical possibility for children with the very short bowel syndrome. Z Kinderchir 40:233-236, 1985 22. Bond JH, Cuvier BE, Buchwals H, et al: Colonic conservation of malabsorbed carbohydrates. Gastroenterology 78:444-447, 1980 23. Bond JH, Levitt MD: Fate of soluble carbohydrate in the colon of rats and humans. J Clin Invest 57:1158-1164, 1976 24. Richardson JD, Medley ES, Griffen WO: Prevention of small bowel contamination by the ileocecal valve. South Med J 6:1056-1058,197l 25. Touloukian RJ, Smith GJW: Normal intestinal length in preterm infants. J Pediatr Surg 18:720-723,1983

Discussion T. Weber (St Louis, MO): This is an excellent paper on a very difficult problem and one with which we are all struggling almost on a daily basis. Short-bowel syndrome is an increasing challenge to the pediatric

surgeon primarily because of technological advances by many of the people in this organization. Drs Georgeson and Breaux are to be congratulated on their excellent overall results and, perhaps even more

NEONATAL

SHORT-BOWEL

SYNDROME

importantly, showing how survival has improved over the past 10 to 15 years. However, the medical, social, and financial implications of short-bowel syndrome can be staggering and quality of life in these patients must be kept in mind when assessing overall results. I have several questions. Among the therapies associated with increasing survival of short-bowel syndrome is total parenteral nutrition (TPN). This has been both friend and foe to the pediatric surgeon. Besides cholestatic liver injury, TPN is suspected to slow intestinal adaptation when used alone, perhaps by the inhibition of pancreatic secretion. Enteral feedings seem to ameliorate this affect of TPN, but Dr Georgeson stated that he holds the initiation of enteral feedings until the diarrhea phase of short-bowel syndrome has resolved. What is this usual time and wouldn’t it be better to initiate at least a small enteral feeding program even while the infants having diarrhea to attempt to ameliorate this affect of TPN? In our own series, with this very early initiation of enteral feedings, we have virtually no cholestatic liver injury anymore. He also described using the Bianchi procedure of lengthening and stated that improvement occurred, but could you give us more specific information of patient selection, timing of the operation, and exactly what was improved by the procedure? In our own series, we have utilized tapering enteroplasty rather than the Bianchi procedure in several patients, and have documented an improvement in stool number. Do you have similar data for your Bianchi patients? Have you performed any other surgical procedures, such as construction of an artificial iIeoceca1 valve or reverse segments of intestine? Finally, you have confirmed the fact that we and others have pointed out, that the presence of an ileocecal valve is not a determinant of survival in contemporary management of short-bowel syndrome? You also showed that the period of TPN is prolonged in infants without a valve. In our own series, we found an increased incidence of rehospitalization in infants without a valve due to viral gastroenteritis, particularly with rotovirus. Could you comment on rehospitalization and/or reoperative rates in your series of patients and whether viral-induced diarrhea was problematic? L. Spitz (London, England): I, too, would like to emphasize the importance of early enteral feeding in these patients with short-bowel syndrome. We have been singularly unsuccessful at Great Ormond Street with the Bianchi procedure, carried out both in the neonatal period and later when the bowel dilates. I would like the authors to elaborate on the use of this procedure.

349

W. Chwals (Winston-Salem, NC): John Grant has shown in rats and other authors as well have suggested that the use of glutamine in total parenteral nutrition preparations might have a sparing affect on the liver. I was wondering if Dr Georgeson would comment whether he has used these preparations with his patients and whether they have conferred that advantage? A.A. deLorimier (San Francisco, CA): One of the greatest advantages that we have, I think, is knowing when to stop. That is, resect what’s there and do an anastomosis. If the necrosis is all the way to the duodenum, is there anybody who hooks the duodenum to the colon? What is the lowest limit of viability independent of TPN that anyone has encountered with just residual jejunum. What is the lower limit? Douglas Wilmer, in his review of the literature, found 19 cm with independence of TPN. Has anybody experienced a shorter segment than that? P. Puri (Dublin, Ireland): I have a child who is now 18 months postoperation and had 12 cm of jejunum anastomosed to the midtransverse colon, and this baby did not get TPN, not even for 1 day. D.L. Schwartz (New York, NY): I’d like to know if the author could elaborate on how long he uses the Neomycin and is he concerned about any of the toxic manifestations of long-term use of Neomycin? K. Geotgeson (response): First of all, regarding the Bianchi technique, we have used it in four patients. Three of the patients had developed a very dilated small bowel and that made the Bianchi technique easier. All of these three patients had very poor motility and the Bianchi procedure improved motility in all to the point where before they were not able to take enteral feedings and afterward they tolerated them well. We have used a few other surgical procedures. At the present time we are looking at artificial ileocecal valves because of the data we generated out of this study and we don’t really have any information. One of the things that it appears to do is to help dilate the bowel and have it more easily lengthened by the Bianchi technique. As far as readmissions to the hospital, we have had a lot of problem in patients with short gut who develop viral disease such as rotovirus and we frequently tell parents that when their child develops significant diarrhea he/she will have to come back to the hospital. This is especially true after they have been weaned off parenteral nutrition. We are currently using trophamine as our amino acid source, which has high levels of glutamine in it and it has seemed to ameliorate the jaundice in many of the patients. We have one patient who we have attached the duodenum to the midtransverse colon and that

350

patient is now on 70% enteral feeds and 30% parenteral feeds about 2 years after surgery. His bowel had progressively dilated. He has had a Bianchi procedure that lengthened the bowel and now his bowel is dilated again and we are looking at another technique to see if we can further lengthen his bowel and also

GEORGESON AND BREAUX

improve motility. As far as what formula we use, we’ve gone through a number of formulas and there is no magic formula. One of the things we are not doing now is using Progestimil. I would say that most of our current patients are on Isomil.

Outcome and intestinal adaptation in neonatal short-bowel syndrome.

We reviewed 52 consecutive patients with short-bowel syndrome (SBS) treated with long-term parenteral nutrition (PN) from 1978 through 1990. The SBS e...
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