Annals of Pediatric Surgery, Vol 2, No 2, April 2006, PP 130-135

Original Article

Congenital Duodenal Obstruction

Sherif N Kaddah, Khaled HK Bahaa-Aldin, Hisham Fayad Aly, Hosam Samir Hassan Departments of Pediatric Surgery, Cairo University & Tanta University, Egypt

Background/ Purpose: Congenital duodenal obstruction is a frequent cause of intestinal obstruction in the newborn. This study aimed to analyze various factors affecting the outcome of these cases at our institution. Materials & Methods: Seventy one cases of congenital duodenal obstruction were included in this retrospective review. Each case was studied as regard to: age at presentation, gestaional age, clinical data, other associated congenital anomalies, cause of obstruction, management, and outcome. Patients with abdominal wall defects (omphalocoele, gastroschisis) and diaphragmatic hernias were excluded from the study. Results: The causes of duodenal obstruction were: (n= 37), duodenal diaphragm (n= 12), malrotation (n= 14), and annular (n= 8). Age ranged from 2 days to 24 months. Bilious vomiting was the main presenting symptom. Plain radiography was the most valuable diagnostic tool in all cases except malrotation and partial obstruction. Gastrointestinal (GIT) contrast study was very valuable in that later group. Overall mortality was 15 cases (21.1 %). The causes of deaths were: prolonged gastric stasis and neonatal sepsis(n= 7), other associated cardiac anomalies (n=5), and extensive bowel gangrene due to neglected volvulus neonatorum(n= 3). Conclusion: This study showed that (1) the diagnosis of congenital duodenal obstruction is still delayed in many patients referred to our institutions.(2) early postoperative survival is still far from ideal ; (3) the mortality is related to delayed presentation, associated cardiac defects, and prolonged gastric stasis; and (4) late complications are more common than previously expected

Index Word: duodenal obstruction, duodenal atresia, duodenal stenosis, malrotation, volvulus neonatorum, annular pancreas

INTRODUCTION or even both. Atresia and stenosis are classed as ‘intrinsic’ obstruction, while annular pancreas and lthough neonatal duodenal obstruction is quite obstructions from errors of midgut rotation produce rare, the incidence has been variously A ‘extrinsic’ obstructions. Annular pancreas may be estimated as 1 in 10 000 to 1 in 40 000 births.1,2 In large combined with a complete atresia. Multiple atresias series the ratio of atresia : stenosis is 3:2 or 2:2.1,2 occur in about 15% of all cases. Although all of these Duodenal atresia and stenosis is considered frequent forms cause duodenal obstruction there are significant cause of intestinal obstruction in the newborn. 1-5 differences between them.1-5 Various types of duodenal obstruction exist; they may be either partial or complete, extrinsic or intrinsic

Correspondence: Sherif N Kadah M.D. Department of Pediatric Surgery, University of Cairo Children’s Hospital, Cairo , Egypt, Phone : + 20 12 326 7638, e-mail: [email protected]

Kadah SN et al

Duodenal atresia can be classified into three major The causes of duodenal obstruction were: types [Gray and Skandalakis 17]: A type I defect duodenal atresia (n= 37), duodenal diaphragm (n= represents a mucosal diaphragmatic membrane 12), malrotation (n= 14), and annular pancreas (n= 8) (atresia), and is the most common duodenal anomaly (Fig 1-3). Age ranged from 2 days to 24 months. observed. The muscle wall in these cases is intact.8 Patients who had abdominal wall defects The portion of the proximal to the atresia (omphalocoele, gastroschisis) and diaphragmatic is dilated, and that portion distal to the atretic area is hernias were excluded from the study. narrowed. The septum may be stretched distally Charts were reviewed for age at operation, sex, because of peristalsis and high intraluminal pressure gestational age, other medical problems or anomalies, (‘windsock anomaly’).2,9,10 Type 2 duodenal defects preoperative clinical data , type and results of have a short fibrous cord connecting the two ends of imaging studies, operative finding, details of surgical the atretic duodenum; it is less commonly observed. technique, duration of postoperative gastric The two blind ends of the duodenum are separated by decompression, time required for resumption of oral a gap, the mesentery showing a V-shaped defect in intake, hospital stay, and postoperative complications. type 3. Most of the unusual biliary duct anomalies Follow up period ranged from 5 to 33 months. that coexist in these cases are seen in babies with a type 3 defect.8 The first reported congenital obstruction of the duodenum by pyloric membrane was reported by Calder in 17332,10 and by duodenal atresia by Crosby- RESULTS Leonard in 1856 in London.11 Treatment does not The age of patients at time of presention was seem to have been attempted until the end of the 19th variable according to the cause of duodenal century. obstruction. Patients with complete duodenal obstruction presented during the first few days of life The only surgical interventions were a gastrostomy in contrast to those with chronic intermittent or with and a duodenostomy both of which carried a100% incomplete obstruction (Table 1) mortality. The first survivor was not recorded until 1916 by Ernst in Denmark. 12 Preoperative plain radiographs were done in 68 cases, and was sufficient to diagnose duodenal During the past 70 years gradual improvement in obstruction in all patients except those with the survival of babies with this condition has been malrotation and/or partial obstruction. Upper GIT achieved. contrast study was done in 21 cases with The aim of this study is to report 71 infants and incomplete duodenal obstruction. children with congenital duodenal obstruction treated Duodeno-duodenostomy was the operation of at 3 pediatric surgical units to find out the different choice in cases of duodenal obstruction, duodenal causes and to analyze various factors that have an atresia, duoedeal diaphragm and annular pancreas. impact on the outcome of this group of patients. Division of Ladd bands and proper alignment of intestinal mesentery and ilio-cecal valve was the treatment of choice in malrotation. Three patients with malrotation presented with volvulus MATERIALS AND METHODS neonatorum had gangrenous intestinal loop that requires resection and re-anastomosis

Seventy one cases of congenital duodenal The overall mortality was 15 cases (21.1 %). The obstruction were retrospectively reviewed. These causes of deaths were: prolonged gastric stasis and patients were treated by the authors at Departments neonatal sepsis (n= 7), other associated cardiac of Pediatric Surgery at Abuleesh Children’s Hospital, anomalies (n=5), and extensive bowel gangrene due to Benha Children’s Hospital, and Tanta University neglected volvulus neonatorum(n=3). The Hospital from June 2000 to June 2005. postoperative findings are summarized in Table 2.

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Fig 1A. Three-day old baby presented with neonatal Fig 1B. Construction of diamond anastomosis intestinal obstruction. Exploration revealed duodenal atresia type l

Fig 2A. Twelve-month old baby presented with Fig2 B. A duodenal diaphragm is bulging from inside intermittent vomiting and loss of weight. Operative photo showing the site of duodenal obstruction

Fig 3A. Upper GIT series showing duodenal obstruction Fig 3B. Abdominal exploration showing annular pancreas

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Table l. cases of duodenal obstruction

DA (n=37) DD (n=12) MR(n=14) AP(n=8) Mean age at presentation 3.3 days 117.6 days 48.4 days 3.7 days No of premature cases 10 (27.2%) 2 (16.7%) 3 (21.4%) 0 Other anomalies 12 (32.4%) 2 (16.7%) 0 0 Acute symptoms 37 (100%) 5 (41.7%) 11 (78.6%) 7 (87.5%) Chronic symptoms 0 7 (58.3%) 3 (21.4%) l (12.5%) Upper GIT study 2 (5.4%) 10 (83.3%) 6 (42.9%) 3 (37.5%)

DA: Duodenal atresia, DD: Duodenal diaphragm, MR: Malrotation, AP: Annular pancreas Table 2. Post operative course

DA (n=37) DD (n=12) MR(n=14) AP(n=8)

Mean time to full feeding (days) 7.7 4.7 3 5.6 The mean Postoperative hospital 17.4 7.5 6.3 6 stay (days)

Complications:

Wound infection 4 2 l 0 Gastric stasis 16 4 0 2 Neonatal septicemia 8 l 3 0 mortality 10 l 3 1 Anastomotic leak 0 0 0 0

DA: Duodenal atresia, DD: Duodenal diaphragm, MR: Malrotation, AP: Annular pancreas, NG: Nasogastric

DISCUSSION They are relatively uncommon proximal to the Approximately 40% of the atresias are found in ampulla of Vater, the most common site being just at the duodenum, 35% in the ileum and 25% in the the ampulla. Multiple atresias are rare.2 jejunum.2 Likewise, almost 75% of all intestinal Partial obstruction may be caused by a diaphragm stenosis are supposed to be in the duodenum, 20 % in with a central or eccentric opening, or a mucosal web. the ileum and 5% in the jejunum.2 The present series Both anomalies may cause neonatal obstruction, but does not represent the total number of patients with they may be diagnosed only later in childhood, congenital duodenal obstruction treated at the three depending on the size of the opening. Duodenal mentioned institutions during the period of the study; diaphragm represented about 17% (12/ 71) of patients it is rather an experience of the contributing authors. in this series. More than half of this subset group of Congenital duodenal obstruction may be patients (58.3%) presented as incomplete obstruction. associated with other GI and biliary tract The mean age at presentation was 117.6 days abnormalities, or some syndromes as (VACTERL) Partial obstruction may also be associated with an (vertebral, anal, cardiac, tracheal, esophageal, renal, extrinsic narrowing of the duodenal lumen by and limb) association.2 mesenteric bands in association with malrotation, or Usually the atresias as well as the stenoses are by an annular pancreas or by aberrant pancreatic limited to the first and second part of the duodenum. tissue in the duodenal wall.13,14 133 Vol 2, No 2, April 2006

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The most common form of extrinsic obstruction Upper GIT contrast study was done in 21 cases in consists of pancreatic tissue completely or partially series with incomplete duodenal obstruction. The surrounding the second part of the duodenum. All majority of these cases had incomplete or intermittent degrees of obstruction are possible, but in addition duodenal obstruction. We believe that no oral contrast quite often an atresia of the duodenum at the same materials are necessary in the evaluation of complete level is apparent. Symptomless annular pancreas may duodenal obstruction. However, a small amount of be noted by chance during laparotomy or autopsy. contrast material can be instilled through a feeding Annular pancreas is a recognized cause of tube into the distal stomach and duodenum to duodenal obstruction in all age groups. Patients most differentiate between the diaphragm and a long commonly present in infancy or early childhood.15 stenosis. Jimenez and his colleagues conducted a thorough review of the English-language literature, and found Occasionally, barium enema examination is only 5 series of pediatric patients with annular suggested as an adjunct study in the evaluation of pancreas since 1950. The total number of patients in duodenal atresia. To demonstrate a malpositioned these 5 series was 50 patients. Only one of these series cecum but this is not always diagnostic of malrotation included more than 10 patients and only one of these and volvulus.13 series was published within the last 20 years. Other large series of pediatric duodenal obstruction In our series diamond shaped duodeno- included patients with annular pancreas but did not duodenostomy was the standard treatment for analyze them separately.16 patients with intrinsic and annular pancreas. We Whatever the nature of the obstruction the results preferred this technique even in duodenal diaphragm are very similar: the stomach and proximal cases as it prevents any injury to duodenal ampulla or duodenum dilate, their walls become hypertrophied, pancreatic duct, further more there were no reports of and the distal duodenum is small and remains thin- re-stenosis compared to excision of the diaphragm. In walled. The stomach and duodenum may perforate in cases of annular pancreas duodenoplasty is preferred utero or postnatally.10 Annular pancreas presented by most authors to prevent re stenosis, and pancreatic 11.3% of total patients in this series (8/71). Associated fistulas, on attempt of annulus excision.16-18 duodenal atresia was noted in 6 of them. Weber and his colleagues compared different Bile-stained vomitus in neonates aged 24 hours or surgical techniques in treatment of duodenal atresia younger is the typical presentation of atresia or severe and suggested better results of diamond shaped stenosis. Minimal duodenal obstruction in mild duodeno-duodenostomy than other techniques.19 stenosis or membrane may have few symptoms. In a few cases, the atresia is proximal to the ampulla of Effective treatment of annular pancreas was Vater and the vomitus is free of bile. Down's accomplished only in the last half of the 20th century. syndrome occurs in approximately 30%of patients, In 1944, Gross and Chisholm18 reported first successful in 33% to 50%, and 45% are operation of duodenal by-pass instead of releasing the premature.6,7 annulus around the pancreas in a 3 days old girl with annular pancreas Morbidity and mortality using Preoperative plain radiographs were done in 68 bypass technique was clearly understood in later cases and was sufficient to diagnose duodenal reports explaining that annular pancreas may not obstruction in all patients except those with only cause extrinsic obstruction , but may lead , or be malrotation and/or partial obstruction. Plain associated with intrinsic narrowing as well.17 radiographs that demonstrate the double-bubble appearance with no distal gas are characteristic of The overall mortality rate in is very high in our duodenal atresia. The double-bubble sign represents series (n=15, 21.1 %) compared to other reports. dilatation of the stomach and duodenum. This Esscher and his colleagues reported 3% early configuration most commonly occurs with duodenal mortality.20 Our highest mortality rate was in cases of atresia and an annular pancreas. An annular pancreas duodenal atresia (n=10, 27%). This very high is almost always associated with duodenal atresia.17 mortality is attributed to prolonged stay in NICU, gastro- duodenal dysfunction from mega duodenum and gastric dilatation leading to neonatal sepsis. In our series gastric stasis manifested by mega

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Kadah SN et al duodenum with blind loop syndrome, gastro- 6. Chabra R, Suresh BR, Weinberg G, et al: Duodenal atresia esophageal reflux, and delayed transit that occurred presenting as hematemesis in a premature infant with in 22 (31%) patients of intrinsic duodenal obstruction, . J Perinatal 12:25-27, 1992 9 of them were complicated by neonatal sepsis. 7. Grosfeld JL, Rescorla FJ: Duodenal atresia and stenosis: Compared to our series, Spigland and Yazbeck,21 Reassessment of treatment and outcome based on antenatal reported gastric stasis symptoms in 77% of their diagnosis: pathologic variance and long-term follow-up. patients of congenital intrinsic duodenal obstruction, World J Surg 17:301-309, 1993 with mortality 6%. Weber and his colleagues reported 8. Gray SW, Skandalakis JH: The embryological basis for no mortalities in their series.19 Other causes of early treatment of congenital defects. In: Embryology for mortality were the presence of multiple congenital surgeons. Philadelphia, PA, Saunders, 1972, pp: 177-217 anomalies. Two of our patients with duodenal atresia 9. Richardson WR, Martin LW: Pitfalls in the surgical had associated congenital heart and TEF, the other management of the incomplete duodenal diaphragm. patient was premature (<30 weeks). In the current Journal of Pediatric Surgery 4:303—312, 1969 series, Mean hospital stay in cases of duodenal atresia 10. Irving IM, Rickham PP: Duodenal atresia and stenosis; was 17.4 days and the Mean time till onset of feeding annular pancreas. In: Rickham PP, Lister J, Irving IM (eds). was 7.7 days. These figures were higher compared to Neonatal surgery, 2nd edn. Butterworth, Boston 1978, pp: 19 Weber et al who repoted 16.2, 4.1 days respectively, 355—370 and Alexander et al22 who reported 9 and 6 respectively. Mean total hospital stay in cases of 11. Theremin E: Ueber congenitale Occlusionen des Dnndarms. Deutsche Zeitschrift für Chirurgie 1877, pp: 34— malrotation was 7 and 3 days, mean time till onset of 71 feeding was 3 days, compared to John et al13 which was 7 and 3 respectively, in our series there were 3 12. Ernst NP: A case of congenital atresia of the duodenum cases of volvulus neonatorum. The incidence of post treated successfully by operation. British Medical Journal operative wound infection in our series was (7.6%). 1916, 1:644—645 13. John R, Rachel L, Richard J, et al: Management of CONCLUSION Typical and Atypical . Journal of The morbidity and mortality rate of patients with Pediatric Surgery 37:1169-1172, 2002 congenital duodenal obstruction in this series are still far from ideal. Effective measures are urgently needed 14. Rescorla FJ, Grosfeld JL: Anomalies of rotation and fixation. Surgery 108:710-715, 1990 to improve the outcome. Early diagnosis and intervention, proper preoperative preparation, 15. Merrill JR, Raffensperger JG: Pediatric annular pancreas: nutrional support are among these measures. Twenty years' experience. J Pediatr Surg 11:921-925, 1976 16. Jimenez JC, Emil S, Y Podnos: Nam Nguyen Annular Pancreas in Children: A Recent Decade's Experience. J of REFRENCES Pediatr Surg 39:1654-1657, 2004 1. Nixon HH, Tawes R: Etiology and treatment of small 17. Irving IM: Duodenal atresia and stenosis: annular . Surgery 69: 4 1—51, 1971 pancreas. Neonatal Surgery 1990, pp:424-441 2. Schnauffer L: Duodenal atresia, stenosis and annular 18. Gross RE, Chisholm TC: Annular pancreas producing pancreas. In: Welch KJ, Randolph JG, Ravitch MM, et al duodenal obstruction. Ann Surg 119:759-769, 1944 (eds). Pediatric surgery, 4th edn. Year Book Medical, 19. Weber TR, Lewis JE, Mooney D, et al: Duodenal atresia: Chicago, 1991, pp:829—837 A comparison of techniques of repair. J Pediatric Surg 3. Calkins CM, Karrer F, Deodhar J, et al: Duodenal Atresia. 21:1133-1136, 1986 E Medicine World Medical Library, 2003 20. Esscher T: Preduodenal portal vein: A case of intestinal 4. Dalla Vecchia LK, Grosfeld JL, West KW, et al: Intestinal obstruction? J Pediatr Surg 15:609- 612, 1980 atresia and stenosis: A 25-year experience with 277 cases. 21. Spigland N, Yazbeck S: Complications associated with Arch Surg 133:490-497, 1998 surgical treatment of congenital intrinsic duodenal 5. Murshed R, Nicholls G, Spitz L: Intrinsic duodenal obstruction. J Pediatr Surg 25:1127-1130, 1990 obstruction: Trends in management and outcomeover 45 22. Alexander F, DiFiore J, Stallion A: Triangular Tapered years (1951-1995) with relevance to prenatal counselling. Br Duodenoplasty for the Treatment of Congenital Duodenal J Obstet Gynecol 1999, pp:1186-1197 Obstruction. Journal of Pediatric Surgery 37:862-864, 2002

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