Complications Associated With Surgical Treatment of Congenital Intrinsic Duodenal Obstruction By Nitsana

Spigland Montreal,

6 Although survival rates for infants undergoing surgical treatment for congenital intrinsic duodenal obstruction are high, long-term follow-up suggests a high complication rate related to surgical therapy. We reviewed 33 neonates who underwent surgery for congenital intrinsic duodenal obstruction during the past 10 years. There were 20 girls and 13 boys: the mean gestational age was 36 weeks, and mean birthweight was 2,485 g. Bilious vomiting and intestinal obstruction were the most frequent presenting symptoms. Hydramnios was present in 75% of cases and 21% had associated Down’s syndrome. Findings at laparotomy included duodenal atresia (14). annular pancreas (11). and duodenal diaphragm (6). The most frequent surgical procedure was side-to-side duodenoduodenostomy (DD), followed by duodenojejunostomy and resection of web with Heineke-Mikulicr type duodenoplasty. Bowel transit was reestablished at a mean of 13.1 days (range, 6 to 45 days). Seventy-percent of patients developed postoperative complications, the most frequent being megaduodenum with blind loop syndrome or bile reflux gastritis (22%). cholestatic jaundice (17%). gastroesophageal reflux (17%). delayed transit (8%). and bowel obstruction (6%). Six patients (16%) required secondary surgical procedures for postoperative complications Be, megaduodenum, nonfunctioning anastomosis, missed intrinsic stenosis). Two patients died (6%). Stagnation and functional obstruction in the proximal duodenum is the main factor influencing the morbidity rate among these patients. Consideration should be given to the tapering duodenoplasty and diamond-shaped anastomosis in order to help reduce problems associated with megaduodenum and help restore earlier bowel transit. 0 1990 by W.B. Saunders Company. INDEX duodenal

WORDS: stenosis:

Duodenal annular

obstruction; pancreas.

duodenal

atresia;

A

LTHOUGH SURVIVAL rates for infants undergoing surgical treatment for congenital intrinsic duodenal obstruction are high, long-term follow-up suggests a high complication rate related to surgical therapy. In this retrospective analysis, we reviewed the charts of all patients who underwent surgery for congenital intrinsic duodenal obstruction during the past 10 years, with an emphasis on the early and late complications. MATERIALS

AND

METHODS

Thirty-three neonates were treated for congenital intrinsic duodenal obstruction at Hdpital Sainte-Justine, Montreal, between 1979 and 1989. There were 20 girls and 13 boys. The mean gestational age was 36 weeks. The mean birthweight was 2,485 g (range, 1,392 to 4,260 g). Twenty patients had associated congenital anomalies (Table 1). The most frequent associated anomalies included malrotation (21%). Down’s syndrome (21%). and biliary tract anomalies (12%). Journal

of Pediatric

Surgery,

Vol 25,

No

11 (November),

1990:

pp

1127-l

and

Salam

Yazbeck

Quebec Hydramnios was detected on prenatal ultrasound in I9 of 25 cases (75%) in which this information was available. In nine patients, the diagnosis of duodenal obstruction was made prenatally, based on a double-bubble image noted on maternal ultrasound. Vomiting and intestinal obstruction were the most frequent presenting symptoms. Vomiting was bilious in 29 patients (88%) and nonbilious in four patients (12%). Other associated pathological conditions are listed in Table 2. All infants had onset of symptoms at birth, except three patients who became symptomatic on the second day of life. Abdominal x-rays were performed in 31 patients. Twenty-seven of 31 (87%) had a double-bubble pattern with no distal air, suggesting the diagnosis of complete duodenal obstruction. Nonspecific gas patterns were found in the remaining four. Upper gastrointestinal studies were performed preoperatively in seven cases, and suggested the diagnosis of complete or incomplete duodenal obstruction. All patients underwent surgical intervention at a mean age of 4.6 days (range, 1 day to 16 months). RESULTS

Findings at laparotomy included duodenal atresia (14), annular pancreas (1 l), and duodenal diaphragms (8). In 32 cases the obstruction was at the level of the second portion of the duodenum, and in one case it was at the level of the third portion of the duodenum. The exact location of the obstruction in relation to the ampulla of Vater was clearly indicated in 28 charts. It was distal to the ampulla of Vater in 20 cases, at the level of the ampulla in four cases, and proximal to the ampulla in four cases. A variety of surgical procedures were performed (Table 3). The most frequent surgical procedure was side-to-side duodenoduodenostomy, followed by duodenojejunostomy, and partial web resection with HeinekeMickulicz-type duodenoplasty. Tapering duodenoplasty with duodenodudonostomy was performed in two cases, and a diamond-shaped duodenoduodenostomy was performed in one case. Reestablishment of bowel transit occured at a mean of 13.1 days (range, 6 to 45 days); 15 days for duodenojejunostomy, 12.4 days for duodenoduodenostomy, and 7.5 days for

From S&-Justine Hospital, and the Department of Surgery, University of Montreal, Montreal, Quebec. Presented at the 21st Annual Meeting of the Canadian Association of Paediatric Surgeons, Edmonton, Alberta, September 20-23, 1989. Address reprint requests to Salam Yazbeck. MD, Ste-Justine Hospital. 317.5 Ste-Catherine Rd. Montreal, Quebec H3T lC5, Canada. o 1990 by W.B. Saunders Company. 0022-3468/90/2511-0006$03.00/O 130

1127

1128

SPIGLAND

Table

1.

Distribution

of Associated (33 Patients)

Congenital

Anomalies

No. of Patients

Malrotation

Standard

side-to-side

Partial web excision duodenoplasty

Cardiac anomalies Jejunal atresia H-type tracheoesophageal Skull malformation

Procedures

(33 Patients) No. of Patients

duodenoduodenostomy

19

fistula

(8%).

Six patients required secondary surgical procedures for complications that arose in the postoperative period (Table 5). Among these patients, three patients required anastomotic revisions. Two patients were reexplored for symptoms related to megaduodenum 17 years and 15 months, respectively, after the initial surgical procedure. Both patients were found to have markedly dilated, atonic duodenal pouches, functional anastomotic obstruction, as well as missed intrinsic stenoses. One patient with duodenal, jejunal, and distal common bile duct atresias developed an ischemic stricture and necrosis at the choledochojejunal and duodenojejunal anastomoses. Two patients underwent repeat surgical exploration for postoperative intestinal obstruction, secondary to adhesions and volvulus, respectively. One patient, who had massive postoperative aspiration, was found to have an H-type tracheoesophageal fistula. Five of the six patients who required reinterventions had duodenojejunostomy as initial procedures. Four patients presented with persistent postoperative cholestatic jaundice. All four were found to have associated biliary tract anomalies. In one patient with Down’s syndrome and biliary atresia, a Kasai procedure was not performed and the infant died. A second patient had duodenal, jejunal, and extrahepatic biliary Pathological

Diagnosis Meconium aspiration Respiratory distress syndrome Chylothorax Renal insufficiency Macroscopic rectorrhagia

with Heineke-Mickulicz

6 1 type 4

Duodenoduodenostomy with tapering Diamond-shaped duodenoduodenostomy

partial web excision with Heineke-Mickulicz duodenoplasty. Seventy percent of these patients developed postoperative complications (Table 4). The most frequent complications included megaduodenum with blind loop syndrome or biliary reflux (22%), cholestatic jaundice (17%) gastroesophageal reflux (17%), delayed transit for more than 21 days (18%), and bowel obstruction

Jaundice

Surgical

YAZBECK

Retrocolic duodenojejunostomy Duodenojejunostomy and choledochojejunostomy

Down’s syndrome Biliary tract anomalies

2. Associated

3.

Procedure

Anomalv

Table

Table

AND

Conditions

atresia, and died of sepsis after anastomotic revision for ischemic stricture and necrosis. One patient with congenital absence of the gallbladder and common bile duct dilatation had jaundice that resolved spontaneously. The last patient had a liver biopsy suggesting extrahepatic biliary obstruction and periportal fibrosis, but no follow-up is available. One patient developed a postoperative abdominal wall abscess, after having an intraoperative duodenal injury as a consequence of a bovie burn that required partial resection of the duodenum. Four patients were found to have gastroesophageal reflux diagnosed from 3 months to 1 year after the initial surgical procedure. One patient is being treated with medical therapy for a duodenal ulcer that developed after 17 years of persistent biliary reflux. The overall mortality rate was 6% (2 deaths). Both of these patients had multiple associated congenital anomalies including biliary anomalies. Follow-up ranged from 1 month to 8 years with a mean of 2 years. DISCUSSION

Although there has been a marked improvement in survival for infants undergoing surgical therapy for congenital intrinsic duodenal obstruction, much controversy exists in the literature regarding the best technique of surgical repair. Modifications in surgical techniques over the past two decades were initially prompted by the high mortality rates associated with Table

4.

Postoperative

(with

or blind-

loop syndrome) Cholestatic jaundice Gastroesophageal reflux Intestinal obstruction

1 1 1 4

Cardiac failure Massive aspiration Septic arthritis TOTAL

(33 Patients) No. of Patients 1%)

biliary reflux

2 1

No. of Patients

Complications

Comclication Megaduodenum

Delayed transit lschemic anastomotic stricture, sis/sepsis Abdominal wall abscess Choking/swallowing difficulty

(33 Patients)

2 1

5 (22) 4tlJ) 4 (17) 2 (8) 2 (8) bowel

necro1 1 1 1 1 1

(4.6) (4.61 (4.6) (4.6) (4.6) (4.6)

23 (JO)

DUODENAL

OBSTRUCTION:

SURGICAL

Table

Postoperative Complication

1129

COMPLICATIONS

6. Secondary

Surgical

(6 Patients)

with

biliary reflux

Duodenojejunostomy

Megaduodenum

with

blind loop syn-

Duodenoduodenostomy

Partial excision missed membrane: take down of duodenojejunostomy; duodenoduodenostomy Partial web excision; duodenal

drome stricture

of anastomosis,

bowel necrosis

Duodenojejunostomy

Time Interval Between First and 9scendary Surgical Procedures

Secondary Surgical Procedure

lnltial Procedure

Megaduodenum

lschemic

Procedures

and choledo-

chojejunostomy

revision

anastomosis

duodeno-

Intestinal

obstruction

(adhesive)

Duodenojejunostomy

Intestinal

obstruction

volvuIus

Duodenojejunostomy Duodenojajunostomv

resection Repair H-type

aspiration

anastomotic complications in earlier series. However more recently, varied techniques have been proposed to diminish the early and late complication rates.‘-’ When evaluating the early postoperative results of the various repairs performed in this series, we found that the mean duration for reestablishment of bowel transit of 13.1 days, was significantly longer than the 3- to 7-day time interval reported in other series in which the diamond-shaped anastomosis or tapering duodenoplasty with duodenoduodenostomy were performed.sg7Y8 Additionally, partial web excision with Heineke-Mickulicz-type duodenoplasty, was associated with a more rapid reestablishment of bowel transit when compared with the bypass procedures. That’s why we favor this technique for the treatment of intrinsic duodenal webs, when the proximal duodenal pouch is not too dilated. Although the initial postoperative course following repair of congenital intrinsic duodenal anomalies may be uncomplicated, certain abnormalies are noted when long-term results are evaluated.‘*” Megaduodenum with functional obstruction and stagnation is prevalent among these abnormalities. In this series, megaduodenum was the most frequent postoperative complication, and it was associated with two basic patterns of clinical presentation: recurrent abdominal pain with postprandial vomiting, and diarrhea suggestive of a blind loop syndrome, or alkaline biliary reflux with resultant gastritis, duodenitis, and peptic ulceration. Ein et al9 reported three cases of functional obstruction following duodenoduodenostomy in patients who presented 6 to 18 months after the initial surgical procedure. In this series, two patients underwent anastomotic revisions for functional duodenal obstruction, at 15 months and 17 years of age. Both of these patients were found to have missed intrinsic stenoses and in one case, a blind duodenal cul-de-sac was

1 mo

redo choledochojejunostomy

and duodanojejunostomy Laparotomy; lysis of adhesions Laparotomy; reduction of volvulus

Massive

15mo

with tapering

duodenoplasty Take down of choledochojejunal and duodenojejunal anastomoses: jejunal resection;

17yr

tracheoesophageal

jejunal fistula

4mo 3mo 3 wk

created, which became obstructed intermittently and emptied retrograde through the stomach. The high incidence of gastroesophageal reflux and biliary reflux associated with megaduodenum in this series has already been reported.” Additionally, the fact that persistent biliary reflux can lead to peptic ulceration, was demonstrated by one of these patients who developed an anterior bulb duodenal ulcer after 17 years of documented persistent biliary reflux. Others have suggested the possibility of esophageal stricture formation and malignancy following long-standing biliary reflux.” The high morbidity associated with duodenojejunostomy parallels findings in other series.4 Five of the six patients who required reintervention, including those undergoing surgery for intestinal obstruction, had duodenojejunostomy as the initial procedure. Postoperative cholestatic jaundice that persists should alert the clinician to the possibility of associated biliary tract anomalies and not necessarily to a surgical complication.” Although a variety of tapering techniques have been advocated in the recent pediatric surgical literature, with emphasis on restoring earlier bowel transit and minimizing postoperative complications, the impetus for resection and tapering dates back to a review of small bowel atresias by Nixon and Tawes12 in 197 1, They showed that resection of the enlarged proximal bowel in jejunal atresias was associated with a marked improvement in survival when compared with direct anastomosis without resection. Despite the low mortality rate of 6% in this series, the high complication rate of 70% has led us to reevaluate the different surgical techniques for repair of these anomalies. This complication rate may in part be a reflection of the fact that duodenojejunostomy was performed among some of the earlier patients re-

1130

SPIGLAND

viewed. However, the delayed transit observed with standard lateral duodenoduodenostomy, as well as the development of megaduodenum and its associated complications, leads us to consider the more recently reported techniques including duodenoduodenostomy with tapering and the diamond-shaped anastomosis for duodenal atresia or annular pancreas. We favor partial web excision with Heineke-Mickulicz duodenoplasty for intrinsic webs when the proximal duodenum is not too dilated. Regardless of the surgical procedure, we can not

AND

YAZBECK

overemphasize the importance of checking the patency of the entire distal bowel, prior to construction of the anastomosis, to avoid missing additional intrinsic stenoses. It became obvious to us, that long-term follow-up of these patients is mandatory, because many of them will eventually develop abnormalities even if the initial postoperative period is uncomplicated. Although the results of early experiences with the newer techniques seem promising, it is only with long-term follow-up that we will be able to compare efficacy with respect to earlier techniques.

REFERENCES 1. Fonkalsrud EW, de Lorimier AA, Hays DM: Congenital atresia and stenosis of the duodenum. Pediatrics 43:79-83, 1969 2. Girvan DP, Stephens CA: Congenital intrinsic duodenal obstruction: A twenty-year review of its surgical management and consequences. J Pediatr Surg 9:833-839,1974 3. Weitzman JL, Brennan LP: An improved technique for the correction of congenital duodenal obstruction in the neonate. J Pediatr Surg 9:385-388,1974 4. Stauffer VG, Irving I: Duodenal atresia: Long term results. Prog Pediatr Surg 10:49-60, 1977 5. Kimura K, Tsugawa C, Ogawa K, et al: Diamond shaped anastomosis for congenital duodenal obstruction. Arch Surg 112: 1262-1263,1977 6. Weber TR, Lewis JE, Mooney D, et al: Duodenal atresia: A comparison of techniques of repair. J Pediatr Surg 21:1133-l 136, 1986

7. Weisberger G, Boureau M: Resultats imm6diats et secondaires des duod6no-duodCnostomies avec modelage dans le traitement des obstructions duodCnales congCnitales completes du nouveaun6. Chir Pediatr 23:369-372, 1982 8. Adzick NS, Harrison MR. de Lorimier AA: Tapering duodenoplasty for megaduodenum associated with duodenal atresia. J Pediatr Surg 21:311-312, 1986 9. Ein SH, Shandling B: The late non-functioning duodenal atresia repair. J Pediatr Surg 21:798-801, 1986 10. Kokkonen ML, Kalima T, Jaaskelainen J: Duodenal atresia: Late follow-up. J Pediatr Surg 23:216-220.1988 11. Reid IS: Biliary tract abnormalities associated with duodenal atresia. Arch Dis Child 48:952-957, 1973 12. Nixon HH, Tawes R: Etiology and treatment of small intestinal atresia: Analysis of a series of 127 jejuno-ileal atresias and comparison with 62 duodenal atresias. Surgery 69:41-51, 1971