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:
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:
