Short-Bowel

Syndrome:

By M.H. Galea, H. Holliday, Nottingham,

syndrome.

L

ESS THAN 30 years ago death was inevitable following massive small bowel resection; surgical morbidity was contributory, but it was primarily because of the ensuing protein and calorific malnutrition. The situation is now very different; marked improvements in surgical and anaesthetic techniques and in neonatal intensive care units have occurred, but it is the introduction, development, and advances in total parenteral nutrition (TPN) and enteral feeds that have transformed the long-term outlook for children with short-bowel syndrome (SBS). TPN is able to provide all the nutrients essential for growth and development during the phase of postoperative adaptation of the remaining small bowel segment. This is thought to occur by bowel dilatation and lengthening (in the preterm neonate), heightening of the villi, and hyperplasia of the epithelium; the principal stimulus for adaptation is the presence of food in the gut lumen, endogenous secretions, and circulating hormones.’ However, management of this condition by any single surgeon is limited: it is against this background From the Department of Paediatnc Surgery Queen’s Medical Centre, Nottingham. England, and the Department Paediattic Surgery, Royal Hospital for Sick Children, Glasgow, Scotland. Date accepted: November 12, 1990. Presented in part at the 36th Annual International Congress of the British Association of Paediattic Surgeons, Nottingham, England, July 1989. Address reprint requests to M.H. Galea, FRCS, Depatiment of Surgery, City Hospital, Hucknall Rd, Nottingham NG5 IPB, England. Copyright o 1992 by W.B. Saunders Company 0022-3468/92/2705-0015$03.00/0 592

Review

R. Carachi, and L. Kapila

England and Glasgow, Scotland

l Neonates can now be expected to survive with very short lengths of bowel because of advances in pediatric neonatal care and in parenteral and enteral nutrition. Most pediatric surgeons have only a few patients with this problem under their care, so individual experience is limited. This collective survey, carried out by postal questionnaires to pediatric surgeons in the British Association of Paediatric Surgeons in the United Kingdom with an interest in the gastrointestinal tract, documents current techniques of management, the complications encountered, and reviews the clinical and economic consequences of prolonged total parenteral nutrition. We conclude that within the limitations of resources and our understanding of prognosis, neonates, especially if ~35 weeks’ gestation, with remaining jejunoileal segment of ~-20 cm with an intact ileocecal valve (ICV) or 230 cm without an ICV, should be considered salvageable. Copyright o 1992 by W.B. Saunders Company INDEX WORDS: Short-bowel

A Collective

that this survey was undertaken. The aim was to review the collective experience of SBS in the UK over the last 10 years. MATERIALS

AND METHODS

Review was for the lo-year period 1978 to 1988. It was undertaken using a postal questionnaire sent to all surgeons in the British Association of Paediatric Surgeons (BAPS) (UK) with a gastrointestinal tract interest. Data collection was onto a standard proforma. SBS has been defined based on the expected jejunoileal (JI) length for gestational age.2 The maximum acceptable length of JI was 25% of the mean length plus 1 standard deviation: thus, for neonates operated on between 27 and 3.5 weeks’ gestation, the remaining JI segment had to be < 50 cm and for neonates over 35 weeks, < 72 cm. After massive small bowel resection, TPN is almost always necessary, in order to provide the neonate with all the nutrients essential for growth and development during the period of postoperative adaptation of the remaining JI segment. When adaptation is complete and all the infant’s dietary requirements can be met enterally, TPN is no longer required. For the purpose of this survey we have equated the time period of TPN requirement with the time for postoperative adaptation to occur. RESULTS

Seventy-three completed proformas were returned; 64 were suitable for inclusion. There were 50 survivors and 14 deaths, giving an overall survival of 78%. There was no difference between the two groups for male:female sex ratio, mean gestational age, and mean birth weight (Table l), or the reason for surgery resulting in short bowel (Table 2). TPN was used for varying times in 58 of the 64 patients; in 4 patients who died it was withheld (reasons not given) and in 2 surviving patients it was not necessary. In those in whom TPN was withheld, the following patient details were noted: gestational ages 34,33,40, and 40 weeks; birth weights 2.3 kg, 2.3 kg, 2.3 kg, and 2.2 kg; remaining JI lengths 10 cm, 40 cm with an ileocecal valve (ICV), 20 cm, and unknown without an ICV, respectively. Both survivors not requiring postoperative TPN retained their ICV; patient 1, gestational age 38 weeks, had 65 cm of remaining JI and patient 2, gestational age 34 weeks, had 50 cm. Eight patients at the time of review were still on TPN; 5 had been on TPN for > 18 months. Their remaining JI lengths were 40 cm, 30 cm, and 22 cm, without an ICV and 20 cm and 5 cm with an ICV. Data are for survivors unless indicated. Figure 1 defines the number of survivors with specific JI lengths remaining after surgery: 33 patients (66%) had > 40 cm remaining after surgery. Figure 2 illusJournal ofPediatric Surgery, Vol 27, No 5 (May), 1992: pp 592-596

SHORT-BOWEL

SYNDROME:

593

A COLLECTIVE REVIEW

Table 1. Patient Details (n = 64)

No. of patients Male:female ratio Mean gestational age (wk) Mean birth weight (kg)

14

Survived

Died

50 (78%)

14 (22%)

27~22

717

35.7

35

2.2

2.2

trates that after exclusion of those patients still on TPN at the time of review most require TPN for short periods only: 56% for < 10 weeks, 70% for ~20 weeks, 81% for < 30 weeks, and 92% for < 40 weeks. One patient with 42 cm of remaining JI without an ICV required 208 weeks of TPN. Table 3 illustrates that the absolute length of remaining JI, whether greater or less than 40 cm, is not a predictor of TPN requirement; there is a trend toward a decreased time on TPN with a longer remaining JI segment but this is not significant for patients either with or without an ICV because of a wide distribution about the median value. Together with Fig 3, it demonstrates the importance of preservation of the ICV in promoting adaptation; no patient with an intact ICV required TPN for >40 weeks (excIudes those still on TPN at the time of review. Adaptation occurs sooner in the preterm infant. In neonates of < 35 weeks’ gestation when first operated on (n = 8) the median time on TPN was 6 weeks (range, 0 to 9). For those >35 weeks’ gestation (n = 42) me d’lan t.lme on TPN was 17 weeks (range, 2 to 108; P = .Ol[Mann-Whitney]). Survival and successful adaptation in neonates < 35 weeks’ gestation was recorded with remaining JI segments of 22 cm with an ICV and 30 cm without an ICV, and in neonates >35 weeks’ gestation with remaining JI segments of 25 cm with an ICV and 42 cm without an ICV. Survival is possible with shorter remaining lengths of JI segment, but in this review these patients are requiring long-term TPN because of failure of successful adaptation. In 80% of survivors TPN was commenced within 1 week of surgery; this had increased to 95% at 2 weeks. Despite the known advantages of early introduction of enteral feeds only 20% were on combined TPN and enteral feeding by 2 weeks: this improved to 60% by 1 month. The type of enteral feed used varied: progestimil 21 patients, Chix 8 patients, neocate 4

I

no. patients I

12 1

(20

21-30

31-40

41-50

length of remaining

51-60

61-72

JI (ems)

Fig 1. Lengths of remaining JI segmentsversus (survivors only).

number of patients

patients, galactimin 2 patients, Peptijunior 4 patients, milk and lactose-free preparation 9 patients, SMA 1 patient, Wysoy 1 patient, and expressed breast milk 1 patient. One survivor had a Bianchi bowel-lengthening procedure (the leaves of the mesentery are separated along the mesenteric border and the intestine divided with a stapling device down the middle longitudinally resulting in intestine twice as long and half its previous diameter); the patient had been on TPN for greater than 3 years prior to surgery and came off approximately a year after. Morbidity in these neonates was high. The major source of in-hospital morbidity was TPN-related catheter sepsis in 27 patients (54%) and acquired biliary dysfunction in 20 patients (40%). Other recorded sources of morbidity were anemia (50%), rickets (6%), endocarditis (4%), and symptomatic cholelithiasis (6%). Long-term morbidity was principally frequency of defaecation and short stature. At last review, and excluding those still on TPN, the median

% of patients

Table 2. Reason for Surgery Resulting in SBS Survived

Died

Midgut volvulus

16

4

Jejunal/ileal atresia

15

5

Necrotizing enterocolitis

12

2

7

3

50

14

Gastroschisis Total

0

10

30 20 time (weeks)

40

208

Fig 2. Percent remaining on TPN versus time (excludes those still on TPN).

594

GALEA

ET AL

Table 3. Results for Survivors Length of Remaining 40(n=33)

Median Time on TPN (range) (wk) 40cmb=33)

11

21

12 (7-104)

9 (O-52)

6

12

78 (4-118)

27 (7-208)

frequency of bowel action in children without ICV was 4 times per day (range, 2 to 10) and 50% of these children were taking antidiarrhea medication. Those with an ICV faired better; their median frequency of bowel actions was 2 times per day (range, 1 to 8) and only 30% of them required ongoing antidiarrhea medication. Fig 4 illustrates that short stature is common, even after coming off TPN and tolerating a normal diet; more than 90% of children at their last review were below the 50th percentile. Most children coming off TPN appear to be of normal intelligence. Only two were noted to have speech and learning delay. Figure 5 illustrates mortality data. In four patients TPN was withheld; death ensued soon after surgery. Of those on TPN three died of unrelated causes-one of ventriculoperitoneal shunt failure at 2.5 years having come off TPN at 3 months, one of progressive cerebral atrophy secondary to significant birth asphyxia, and one of microcephaly. Seven patients on TPN died of sepsis; three secondary to complications of surgical technique and postoperative morbidity and three secondary to complications of TPN alone. Therefore, only 3 of 58 (5%) of patients given TPN died solely of TPN-related problems. DISCUSSION

This review has looked at the UK results of massive small bowel resection over the last 10 years. It the first collective review since that of Wilmore in 1972,3 although others have reviewed their own experiences.4-6 It is a review of current practice in the United Kingdom, providing 64 patients for analysis.

1O-25

3-10

percentile Fig 4.

withoul

-

with ICV

at last

so-75

75-90

review

Short stature.

It is encouraging that many patients require TPN for only short periods; this must result in an overall reduced TPN-related morbidity. Interestingly though, TPN-related mortality appears to be independent of time on TPN; two liver failure deaths occurred at 10 and 11 weeks, respectively. Although it might be expected that the longer the remaining JI segment the shorter the time on TPN, this is not so in this survey. Of much more prognostic significance is the presence or absence of the ICV and gestational age at the time of initial laparotomy. Survivors with an intact ICV coming off TPN did so quicker than those without, such that by 40 weeks none required TPN. Others have reported similar findings.3-7 The ICV is important because it delays small bowel transit by a factor of 3, allowing prolonged contact between luminal nutrients and mucosa; it prevents reflux colonization of the remaining JI segment by colonic bacteria and it is likely that most patients with an ICV also have some remaining ileum. 14

A

X-50

Deaths

NO TPN II=4

ICV

Bowel Cause

lengths of death

(10 - 40 cm) - arrest x 2 sepsis "Ulrn. haFm

“nrelated:

I

,yJI7

Sepsis:7 /,

CL*.,.+ r-4 I.._^ AUAAVLS

Yl.“llL

Cerebral atrophy Microcephaly 2 liver

0

20

40

60

80

208

time an TPN(weeks1

Fig 3. Time on TPN versus percent of patients, with and without ICV (excludes those still on TPN).

1 subphrenic +DIC

1 pure

failure

sepsis

1 MOF

Fig 5. Mortality data. DIC, disseminated tion; MOF, multiple organ failure.

intravascular

coagula-

SHORT-BOWEL

SYNDROME:

595

A COLLECTIVE REVIEW

Animal studies indicate that ileum is better able to adapt than jejunum,s possibly because jejunum is already more specialized and, therefore, less able to alter its function. We have been unable to evaluate this factor in this survey because differential lengths of jejunum and ileum were incompletely recorded. Although the preterm neonate may appear a poor surgical risk, its chances of recovery from a massive small bowel resection are better. A small number of neonates with massive small bowel resection will require TPN over long periods. Six patients required TPN for more than 18 months, 5 of whom were still on TPN at the time of review. The question arises as to whether further surgical intervention could reduce the time on TPN. Methods that have been tried include reversal of the small intestine9x10recircmating intestinal loops11~‘2;Colon interposition1”,14; fashioning of sphincters and valve@; intestinal lengthening (Bianchi procedure16,17); and small bowel transplantation.18 A case can be made for considering surgery in those children who seem unlikely to be free of TPN, but this should never be contemplated before the age of 12 months. At about this time an infant’s caloric requirement per unit of body weight decreases; the chances of successful adaptation corresponding increase. This survey has shown survival and successful adaptation in neonates 35 weeks’ gestation with remaining JI segments of 25 cm with an ICV and 42 cm without an ICV. Other authors have reported survival in full-term neonates with 11 to 13 cm of remaining JI segment with an ICV,19,20but at the huge expense of prolonged TPN, hospital stay, significant morbidity, and questionable quality of life. It is stressed that quality of expected life must remain central to the debate in management of SBS; there are no medals for simply recording minimal JI segments compatible with crude survival. Cost can be reduced by use of home-based TPN (especially if TPN is likely to be prolonged); normal developmental milestones and successful outcome are being recorded using this treatment option.21 It is recognized that one of the basic stimuli promoting adaptation is the presence of food in the gut lumen and it is advocated that enteral feeding be commenced as soon as postoperative motility returns. Attention should be focused on quantity, rather than

strength, of the enteral feed. The choice of preparation usually reflects individual surgeon’s preference. Progestimil and lactose-free preparations are the most favored. The main in-hospital morbidity was TPN-related catheter sepsis, acquired hepatobiliary dysfunction, anemia, rickets, endocarditis, and gallstones. Catheter sepsis was the most common problem and was probably underreported in this survey. Continued improvement with catheter manufacture and the realization that an infected line can be salvaged by the use of high-dose antibiotics and urokinase down the line22 may reduce the number of line replacement procedures. Acquired hepatobiliary complications are well recognized, 23,24but fortunately most are selflimiting and nonprogressive. However, death may ensue from the poorly understood entity of TPNrelated liver failure. An increased susceptibility to cholelithiasis after ileal resection and loss of the ICV23,24is again recorded and in this survey three patients required cholecystectomy for cholelithiasis. Children successfully coming off TPN had reduced small bowel transit times and many required longterm antidiarrheal medication. Thirty percent with an intact ICV required some medication, compared with 50% without an ICV. Interestingly, in the group as a whole 90% were still below the 50th percentile for weight at their last review, many having been off TPN for a considerable time. Why they should fail after adaptation to regain a more normal distribution is unclear. The overall mortality was 22%, representing 14 deaths. Death should not be necessarily attributed to the complications of TPN; in this survey only 3 of the 14 deaths could be directly attributed to this factor. Thus, in 58 patients receiving TPN for varying periods the TPN-related mortality was 5%. The place of further surgical intervention is still undecided and more work on this should run parallel with efforts to reduce TPN morbidity and to enhance the adaptive process of the residual JI segment. Surgeons should be encouraged to persevere with enthusiasm because it is their leadership that will ensure maximum support from the health care team. ACKNOWLEDGMENT We are grateful to all the pediatric surgeons who contributed by filling in the data proformas, and to Penelope Browne for unerring secretarial support.

REFERENCES 1. Stand 2. term 3.

Dowhng RH: Small bowel adaptation and its regulations. J Gastroenterol74:53-74,1982 (suppl) Touloukian RJ, Smith GJ: Normal intestinal length in preinfants. J Pediatr Surg l&720-723, 1983 Wilmore DW: Factors correlating with a successful outcome

following extensive intestinal resection in newborn infants. J Pediatr 88:80-95,1972 4. Cooper A, Floyd TF, Ross AJ, et al: Morbidity and mortality of short-bowel syndrome acquired in infancy: An update. J Pediatr Surg 19:711-718.1984

596

5. Dorney SFA, Ament ME, Berquist WE, et al: Improved survival in very short small bowel of infancy with use of long term parenteral nutrition. J Pediatr 107:521-525, 1985 6. Caniano DA, Starr J, Ginn-Pease ME: Extensive short bowel syndrome in neonates: Outcome in 1980s. Surgery 105:119-124, 1989 7. Schwartz MZ, Maeda K: Short bowel syndrome in infants and children. Pediatr Clin North Am 32:1265-1279,1985 8. Dowling RH, Booth CC: Structural and functional changes following small intestinal resection in the rat. Clin Sci 32:139-149, 1967 9. Gibson LD, Carter R, Hinshaw DB: Segmental reversal of small intestine after massive bowel resection. JAMA 182:952-954, 1962 10. Hammer JM, Seay PH, Johnston RL, et al: The effect of antiperistaltic bowel segments and intestinal emptying time. Arch Surg 79537-541, 1959 11. Altman DP, Ellison EH: Massive intestinal resection: Inadequacies of the recirculating loop. Surg Forum 16:365-367, 1965 12. Budding J, Smith CC: Role of recirculating loops in the management of massive resections of the small intestine. Surg Gynecol Obstet 125:243-249,1967 13. Garcia VF, Templeton JM, Eichelberger MR, et al: Colon interposition for the short bowel syndrome. J Pediatr Surg 16:994995,1981 14. Carner DV, Raju S: Failure of antiperistaltic colon interposition to ameliorate short bowel syndrome. Am Surg 47:538-540, 1981

GAt-EA ET AL

15. Ricotta J, Zuidema GD, Gadacz TR, et al: Construction of an ileocecal valve and its role in massive resection of the small intestine. Surg Gynecol Obstet 152:310-314, 1981 16. Bianchi A: Intestinal loop lengthening-A technique for increasing small intestinal length. J Pediatr Surg 15:145-151, 1981 17. Bianchi A: Intestinal lengthening: An experimental clinical review. J R Sot Med 77:35-41, 1984 (suppl3)

and

18. Revilon Y, Goulet 0, Ricour C, et al: A one year follow-up after a small bowel transplantation. Presented at the 36th Annual International Congress of the British Association of Paediatric Surgeons, Nottingham, England, July 1989 (abstr) 19. Kurz R, Sauer H: Treatment and metabolic findings in extreme short bowel syndrome with 11 cm jejunal remnant. J Pediatr Surg 18:257-263, 1983 20. Holt D, Easa D, Shim W, et al: Survival after massive small intesatinal resection in a neonate. Am J Dis Child 136:79-80. 1982 21. Vargas JH, Ament ME, Berquist WE: Long term home parenteral nutrition in pediatrics: 10 years of experience in 102 patients. J Pediatr Gastroenterol Nutr 6:24-32, 1987 22. Walker J: Personal communication, 1989 23. Roslyn JJ. Berquist WE, Pitt HA, et al: Increased risk of gallstones in children receiving total parenteral nutrition. Pediatrics 71:784-789, 1983 24. King DR, Ginn-Pease ME, Lloyd TV, et al: Parenteral nutrition with associated cholelithiasis: Another iatrogenic disease of infants and children. J Pediatr Surg 22:593-596, 1987

Short-bowel syndrome: a collective review.

Neonates can now be expected to survive with very short lengths of bowel because of advances in pediatric neonatal care and in parenteral and enteral ...
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