Lethal short-bowel syndrome

Lethal Short-Bowel Syndrome By B.J. Hancock and N.E. Wiseman Winnipeg, 0 Infants with short-bowel syndrome are difficult to manage. Despite supportive measures with parenteral nutrition and surgery to lengthen remaining bowel or increase functional absorptive surface area, the outcome for many of these infants is poor. We have reviewed a series of seven infants diagnosed with severe short bowel. Causes included volvulus (3). multiple atresias (2). and total intestinal aganglionosis (2). Survival time ranged from 15 days to 8 months. During the hospital course, each infant underwent one to three operative procedures to diagnose and manage the short bowel and all received total parenteral nutrition (TPN) ranging from 10 days to 6 months. One infant died of liver failure and two others developed significant liver dysfunction secondary to TPN. Most infants remained hospitalized until their death. Death occurred at an average of g weeks following the diagnosis of short-bowel syndrome. This review suggests that infants with less than 6 cm of small bowel beyond the Ligament of Treitz will inevitably die of their disease or treatment complications. Until bowel transplant becomes a viable alternative, operative intervention and nutritional support may prolong survival but will not change the outcome of these infants and will only contribute to additional morbidity. A decision to withhold further therapy would be reasonable at the time the diagnosis is established. @ 1990 by W.B. Saunders Company. INDEX

WORDS:

Short-bowel

syndrome.

I

NFANTS WITH EXTREME short-bowel syndrome continue to be a challenge to the pediatric surgeon. Advances in providing nutrition through parenteral and enteral routes have changed the dismal outlook significantly. Infants with minimal functional small bowel length have survived with eventual intestinal adaptation sufficient to support the nutritional needs of the infant without parenteral supplements. In contrast, a significant number of infants ultimately die as a direct result of either the short bowel or complications of aggressive medical and surgical efforts to support life. Factors that influence management decisions include certainty in the diagnosis and prognosis, parental wishes, differing opinions among caregivers, and concerns of causing unnecessary prolongation of life and morbidity. In this series of seven infants with lethal short-bowel syndrome, the presentations, treatment, complications, and outcome were reviewed to address some of the issues involved in the decision to treat these infants. MATERIALS

AND METHODS

The charts of seven infants with lethal short-bowel syndrome were reviewed at the Children’s Hospital and St Boniface General Hospital, Winnipeg. The following information was obtained from Journal

of Pediatric

Surgery,

Vol 25,

No 11 (November),

1990:

pp 1131-l

Manitoba each chart: birth weight, gestational age, etiology of short-bowel syndrome, age at which the diagnosis was established, additional diagnoses, length of remaining small bowel, number and types of surgical procedures performed, use of parenteral and enteral nutrition, complications, and the age at which death occurred.

RESULTS

Table 1 summarizes the clinical data obtained for each patient with lethal short-bowel syndrome. Five infants were born at term and weighed over 2,500 g; one infant, the product of a twin gestation was 35 weeks’ gestation and weighed 2,000 g. Only one infants was premature at 26 weeks, weighing 500 g. Short bowel was the result of volvulus in three infants, total intestinal aganglionosis in two, and multiple congenital atresias in two. Six infants presented within 8 days of birth; the seventh presented with volvulus at the age of 4 months possibly related to the previous formation of a feeding jejunostomy. Additional medical problems were present in all but one infant. Three developed complications of total parenteral nutrition (TPN) including acute pancreatitis, fatty liver, hepatic cholestasis, liver failure, cholelithiasis, and thrombocytopenia necessitating discontinuation of parenteral support. Septic episodes related to parenteral nutrition occurred in three infants. All infants died: one of liver failure secondary to TPN and six after withdrawal of therapy when the situation seemed hopeless. Deaths occurred at an average of 9 weeks after the initial diagnosis of short-bowel syndrome was established and ranged from 15 days to 8 months. The lengths of functional small bowel remaining beyond the ligament of Treitz, as determined at the time of surgery, and the number and type of surgical procedures are outlined in Table 2. In four infants, no normal bowel remained, whereas in two, only 1 to 6 cm of small bowel was left intact. In the seventh infant, approximately 16 cm of bowel remained after resection for volvulus but was of questionable viability. Subse-

From the Department of Pediatric General Surgery, University of Manitoba, Winnipeg, Manitoba. Presented at the 2lst Annual Meeting of the Canadian Association of Paediatric Surgeons, Edmonton Alberta. September 20-23, 1989. Address reprint requests to N.E. Wiseman. MD. Winnipeg Children’s Hospital, 840 Sherbrook St, Winnipeg, Manitoba R3A ISI, Canada. 0 1990 by W.B. Saunders Company. 0022-3468/90/2511-0007$03.00/0 134

1131

1132

HANCOCK

Table Case No.

1 2

Birth Weight (a)

1. Summary

Gestation (wk)

2,950 500

of Patients

With

Volvulus

with malrotation

26

Volvulus

without

with malrotation

5d

malrotation

3

2,780

40

Volvulus

4 5

3,660 3,650

40 40

Total intestinal Total intestinal

6

2,000

35

Multiple small bowel cyst microcolon

7

2,580

36

Multiple

Additional Diagnoses

RUL atelectasis,

foregut Abbreviations:

RUL, right

Prematurity, RDS sepsis x 4, patent ductus, spontaneous enterostomy

2d

Dehydration/malnutrition, acute pancreatitis, fatty liver, hepatic

atresias,

enteric

to colon.

Volvulus

21 d Sepsis x 2, liver dysfunction, abdominal wall dehiscence

Id

Liver failure, thrombocytopenia, pneumococcal meningitis, pneumococcal sepsis/respiratory Perinatal asphyxia,

3d

with malrotation

arrest meconium

anemia,

aspira-

15d

2.

Surgical

continuity. The third patient with volvulus underwent initial resection and subsequent insertion of a central line for TPN. The two infants with aganglionosis had an initial laparotomy to establish the diagnosis and one underwent an additional laparotomy for enterolysis and the formation of a jejunostomy. Both infants with multiple congenital atresias had a single laparotomy that confirmed the diagnosis and each had bowel resections and enterostomies established. Table 3 summarizes the duration and complications of TPN as well as efforts to institute enteral nutrition. All infants received TPN ranging from 10 days to 6 Intervention

Length of Functional Small Bowel Distal to Ligament of Treitz (cm)

No. of Surgical Procedures for Short Bowel

None

3

Surgical Procedures

Ladd’s

procedure,

detorsion

720”

2ndlook lap, insertion prosthetic pouch; infarcted midgut resected, hoff catheter x 2 2

Volvulus

without

l-2

malrotation

3

4

5

Volvulus

with malrotation

Total intestinal

Total intestinal

None

aganglionosis

None

aganglionosis

6

Multiple

7

microcolon Multiple atresias-pylorus nonrotation

Abbreviations:

<16

small bowel

lap, laparotomy:

atresias,

enteric

to colon,

cyst foregut

TPN, total parenteral

nutrition.

volvulus; silo Tenck-

Patent ductus ligation; jejunal feeding tube, gastrostomy; detorsion 360” midgut volvulus; 2ndlook lap, midgut infarction; explore spontaneous

3

8mo

syndrome.

Table

1

cholelithiasis

tion pneumonia

distress

Diaanosis

1 mo

8d

nonrotation

quent barium study demonstrated a stricture at the junction of the second and third parts of the duodenum and a second stricture with complete obstruction of the bowel lumen 6 cm beyond the ligament of Treitz. Thus, all infants in this series were left with essentially 6 cm or less of normal small bowel. All seven infants underwent surgery. A total of 13 procedures were performed for short bowel, ranging from one to three per infant. Two infants with volvulus underwent three operations each to establish the diagnosis, reexamine the bowel at second-look laparotomy, and resect infarcted small bowel or restore bowel

C&?tl NO.

9wk 5mo

2d

atresias-pylorus

upper lobe; RDS, respiratory

Age at Death

coagulopathy

4mo

cholestasis. aganglionosis aganglionosis

WISEMAN

Syndrome

Age at Diagnosis

Diagnosis

40

Short-Bowel

AND

enterostomy,

2

Laparotomy,

1

mary anastomosis; central TPN line Laparotomy, large and small bowel biop-

2

sies x 9, in ileostomy 60 cm proximal to ileocecal valve Laparotomy, multiple biopsies to ligament of

6

1

None

1

small bowel resection

duodeno with pri-

Treitz, Stamm gastrostomy; enterolysis, jejunostomy Excision enteric cyst, duodenoileostomy, duodenoplasty, central TPN line Pyloroplasty. resection, ble-barrel

gastrostomy, duodenojejunal small bowel enterostomy, doujejunostomy

LETHAL

SHORT-BOWEL

1133

SYNDROME

Table Case No

10d

None

2 3

3mo 27 d

None Acute

4 5

11 d 45 d

None Liver dysfunction

7 Abbreviation:

and Enteral

Nutrition

Gnnpliitions of Parentera Nutrition

Time on TPN

1

6

3. Parenteral

6 mo

pancreatitis,

fatty

liver, hepatic

Progressive liver dysfunction, hepatic nodular cirrhosis, thrombocytopenia

10d

None

TPN. total parenteral

Enteral Nutrition

cholestasis,

No attempt No attempt All attempts

produced

profuse

watery

diarrhea

No anempt No attempt failure,

micro-

Glucose/water

expressed

breast

milk 53 kcal/kg/d

No attempt

nutrition.

months. Three infants developed significant liver dysfunction with the use of TPN for 27 days to 6 months. One infant died of progressive liver failure. The other two infants died of short-bowel syndrome before any change in liver function could be demonstrated with cessation of parenteral nutrition. Enteral feeding was attempted in two infants. One developed profuse watery diarrhea with each trial and, in the second case, adequate amounts of expressed breast milk to support the nutritional needs of the infant were not tolerated. DISCUSSION

In 1955, Potts’ published an account of the remarkable achievements taking place in the field of pediatric surgery. He stated that infants, due to their small size and substantial growth requirements, could not part with more than 38 cm of intestine and survive. Subsequent series of short-bowel syndrome indicated that survival was almost certain if more than 40 cm of small bowel remains in infants of adequate birth weight without associated anomalies who had uncomplicated surgery. Mortality was high (approximating 50%) in infants with 15 to 40 cm of remaining bowel, especially in the absence of an ileocecal valve. Very few infants survived with small intestinal lengths less than 15 cm.2-4 Massive intestinal loss results in severe metabolic complications.5W7 However, with time and support from parenteral and enteral nutrition, the small bowel has demonstrated a remarkable ability to grow and adapt. ‘-‘I In recent years, several authors have published reports of infants adapting to full enteral nutrition with as little as 8 to 15 cm of small bowel in the presence of an intact ileocecal valve.4*“*15 Survival has also been reported with only 29 cm of small intestine without an ileocecal valve.4 These significant successes have stimulated aggressive management protocols in the hope of improving survival in these unfortunate infants. The introduction of TPN revolutionized the management of short-bowel syndrome and is responsible for

the successful outcome of most infants while the small bowel adapts. However, considerable complications may result from TPN. Progressive cholestasis and abnormal elevation of liver enzymes have been shown to occur in up to 42% of infants on TPN.‘6.17 Deaths secondary to progressive liver dysfunction and catheterrelated sepsis are now the leading causes of death in infants with short-bowel syndrome dependent on parenteral nutrition.4*8’18”9 This was also observed in this series, as significant liver dysfunction developed in three infants and catheter-related sepsis occurred in three. Liver failure secondary to TPN resulted in one death. A variety of surgical procedures have been introduced to attempt to improve tolerance to enteral feedings. Vagotomy and pyloroplasty, recirculating loops, reversed segments of small bowel, colonic interposition, and various valves, sphincters, and baffles have been designed to delay transit and prolong contact time between nutritional elements and the mucosal surface.20*2’ In infants with atresia and massive proximal dilatation, tapering enteroplasty has improved the function of the remaining bowel.” Bianchi’s bowel-lengthening procedure has proven successful in infants with dilated proximal bowel both in slowing transit time and preserving mucosal surface area.22-24 For long-segment Hirschsprung’s disease, a modified Duhamel ileocolic anastomosis, a side-to-side anastomosis of aganglionic to ganglionic small bowel and long-segment myectomy-myotomy have improved function by using the absorptive capacity of aganglionic bowel and the functional reserve of normally innervated bowe1.25-28However, inherent in all these surgical procedures is the requisite for sufficient length of normal small bowel to provide some propulsive action and absorptive surface area for enteral nutrition. In the series of infants presented herein, none had sufficient small bowel to consider these types of surgical intervention. Several authors have attested to the fatal outcome of infants with total intestinal aganglionosis.29-33 This is

1134

HANCOCK

supported by the two infants included in this series. Similarly, extensive intestinal atresia, as observed in two infants presented herein, has exhibited a uniformly poor outcome. 34 The remaining three infants suffered extensive intestinal infarction with less than 6 cm of normal bowel remaining. This suggests that aggressive management in infants with such extensive loss of functional small intestine is inappropriate. Certainly, all infants require laparotomy to establish a diagnosis and, in the case of volvulus, a second-look laparotomy to reassessquestionably viable bowel. However, once a diagnosis is estab-

AND

WISEMAN

lished with certainty, further surgical intervention and parenteral nutritional support are not warranted. In these seven infants, aggressive management resulted in four unnecessary operations and bowel resections with enterostomies in an additional four. Approximately 375 days of TPN were used for nutritional support with complications developing in three infants and death due to liver failure in one. An earlier decision to withhold therapy would have avoided excessive morbidity and unnecessary surgery in this group of infants whose death was inevitable secondary to lethal shortbowel syndrome.

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18. Grosfeld JL, Rescoria FJ, West KW: Short bowel syndrome in infancy and childhood: Analysis of survival in 60 patients. Am J Surg 151:41-46, 1986 19. Weber TR, Vane DW, Grosfeld JL: Tapering enteroplasty in infants with bowel atresia and short gut. Arch Surg 117:684-688, 1982 20. Mitchell A, Watkins RM, Collin J: Surgical treatment of the short bowel syndrome. Br J Surg 71:329-333, 1984 21. Garcia VF, Templeton JM, Eichelberger MR, et al: Colon interposition for the short bowel syndrome. J Pediatr Surg 16:994995,198l 22. Bianchi A: Intestinal loop lengthening-A technique for increasing small intestinal length. J Pediatr Surg 15: 145- 151, 1980 23. Bianchi A: Intestinal lengthening: An experimental and clinical review. J R Sot Med 77:35-41, 1984 24. Boeckman CR, Traylor R: Bowel lengthening for short gut syndrome. J Pediatr Surg 16:996-997, 1981 25. Martin LW: Surgical management of Hirschsprung’s disease involving the small intestine. Arch Surg 97:183-188, 1968 26. Coran AG, Bjordal R, Eek S, et al: The surgical management of total colonic and partial small intestinal aganglionosis. J Pediatr Surg 4:531-537, 1969 27. Kottmeier PK, Jongco B, Velcek FT, et al: Absorptive function of the aganglionic ileum. J Pediatr Surg 16:275-278, 1981 28. Ziegler MM, Ross AJ, Bishop HC: Total intestinal aganglionosis: A new technique for prolonged survival. J Pediatr Surg 22:82-83, 1987 29. Talwalker VC: Aganglionosis of the entire bowel. J Pediatr Surg 11:213-216, 1976 30. Walker AW, Kempson RL, Ternberg JL: Aganglionosis of the small intestine. Surgery 60:449-457, 1966 31. Caniano DA, Ormsbee HS, Polito W, et al: Total intestinal aganglionosis. J Pediatr Surg 20:456-460, 1985 32. Di Lorenzo M, Yazbeck S, Brochu P: Aganglionosis of the entire bowel: Four new cases and review of the literature. Br J Surg 72:657-658,1985 33. Rudin C, Ohnacker PJH, Heitz PU: Absence of the enteric nervous system in the newborn: Presentation of three patients and review of the literature. J Pediatr Surg 2 1:3 13-3 18, 1986 34. Guttman FM, Braun P, Garance PH, et al: Multiple atresias and a new syndrome of hereditary multiple atresias involving the gastrointestinal tract from stomach to rectum. J Pediatr Surg 8:633-640, 1973