Congenital Intrinsic Duodenal Obstruction Research Articles

Congenital intrinsic duodenal obstruction: Clinical study and predictors of neonatal surgical mortality

Background: This study was aimed to analyze the clinical profile and the predictors of neonatal surgical mortality of congenital intrinsic duodenal obstruction (CIDO). Methods: This is a case series of 67 patients with CIDO (duodenal atresia and stenosis) admitted at our teaching institute, from October 2018 to June 2020. The demographic data, clinical presentation, diagnostic methods, location & type of duodenal atresia, the operative procedure performed, and neonatal surgical mortality (NSM) predictors such as birth weight, age on presentation, associated anomalies, duration of surgery, extubation after surgery, sepsis, and vasopressors support were analyzed. Results: Of 67 patients, 40(60%) were premature; mean weight and age at presentation were 2.06±0.6 (1-8 kg) and 6.7(1-240days), respectively. The majority of patients (59, 88%) presented with vomiting, and the most common finding on the x-ray abdomen was a double bubble sign (54, 81%). Twenty-four (36%) were associated with congenital heart disease and 19(28%) had Down’s syndrome. Type 3 duodenal atresia was the commonest finding in 43(64%) followed by type 1 in 22 (33%), and type 2 in 2(3%). In 49 (73%) Kimura’s duodenoduodenostomy, excision of web & duodenoplasty in 13 (19%), and side to side duodenoduodenostomy in 4 (6%), were performed. The mean hospital stay of survivors was 9 days. Overall survival was 63%. Conclusion: Type 3 duodenal atresia is the commonest variety observed. The prematurity, low birth weight, associated major congenital anomalies, surgery >90 minutes, delayed extubation after surgery, sepsis, and need for vasopressors support are significant predictors of NSM.

Abnormalities of intestinal rotation and congenital intrinsic duodenal obstruction

Background Congenital duodenal obstruction (CDO) is a unique anomaly with variable presentation depending on the site, type of obstruction, as well as the presence of associated anomalies. This is a review of our experience with 51 infants and children with CDO, stressing on the importance of associated rotational abnormalities of the gut. Methods Over a period of 18 years (January 1993– December 2011), 51 infants and children with CDO were treated at our hospital. Their medical records were reviewed retrospectively for age at diagnosis, sex, gestation, birth weight, clinical features, associated anomalies, method of diagnosis, treatment, and outcome. Results Fifty-one (27 boys and 24 girls) infants and children with intrinsic CDO were treated. Their birth weight ranged from 1.38 to 3.8 kg (mean 2.3 kg). Sixteen (31.4%) were premature. The mean maternal age was 25 years (range 18–40 years). Seven presented at 5 months, 18 days, 3.5 years, 1.8 years, 18 days, 1.5 years, and 2 months of age, respectively. Associated anomalies were observed in 34 (66.7%) of them. Sixteen (31.4%) had Down’s syndrome and 11 (21.6%) had congenital heart disease. Nine (17.6%) had rotational abnormalities of the gut, including two with situs inversus. The site of duodenal obstruction was located in the second part of duodenum in 48 (94%) of them. In two, the site of obstruction was in the fourth part of duodenum, whereas in the third it was located in the third part of duodenum. The causes of obstruction were duodenal atresia in 20 (39.2%), without a gap in eight (15.7%) and with a gap in 12 (23.5%), duodenal stenosis in 11 (21.6%), and duodenal diaphragm in 20 (39.2%). In 14 (27.5%) patients, an annular pancreas was observed. The different operative procedures were as follows: duodenoduodenostomy in 26; excision of the duodenal diaphragm and duodenoplasty in 12; Ladd’s procedure, excision of the duodenal diaphragm, duodenoplasty, and appendectomy in four; Ladd’s procedure, appendectomy, and duodenoduodenostomy in two; and duodenojejunostomy in seven. Two underwent reduction duodenoplasty to decrease the size of the duodenum. Three required reoperations, two because of an anastomotic leak and another because of duodenal dysfunction. Sixteen (31.4%) required total parenteral nutrition. Four died, resulting in an overall survival of 92.2%. Conclusion CDO is classified as intrinsic and extrinsic depending on the cause. Rotational abnormalities including situs inversus are among the rare but interesting anomalies associated with intrinsic CDO. The presence of malrotation or nonrotation with congenital bands does not exclude the presence of an associated intrinsic cause, which should be looked for intraoperatively. This is to obviate the possibility of further subsequent operative treatment. Keywords : congenital duodenal obstruction, duodenal diaphragm, malrotation, situs inversus

Late Presentation of a Congenital Intrinsic Duodenal Obstruction in a Patient with Anorectal Malformation

Anorectal Malformations are known to be associated with various other congenital anomalies including duodenal atresia. An association of congenital intrinsic duodenal obstruction causing partial duodenal obstruction in a patient with anorectal malformation is not described in literature. We describe a case of delayed presentation of congenital intrinsic duodenal obstruction in a child with high-type anorectal malformations causing diagnostic dilemma.

Influence of Down's syndrome on management and outcome of patients with congenital intrinsic duodenal obstruction

Purpose Children with Down's syndrome (DS) have been reported to have a high incidence of congenital heart disease (CHD) and respiratory tract infection. These anomalies and complications lead to poor outcomes, especially after treatment of congenital intrinsic duodenal obstruction (CIDO). The aim of this study was to review and compare the outcome of management of CIDO at a single tertiary institute for children in Thailand. Methods We reviewed the medical records of patients with CIDO, who were treated at Queen Sirikit National Institute of Child Health (Bangkok, Thailand) from 1997 to 2006. The patients were classified into 2 groups: A = CIDO with DS and B = CIDO with non-DS (NDS). Each group was then divided into 2 subgroups: A 1 = DS without CHD, A 2 = DS with CHD, B 1 = NDS without CHD, and B 2 = NDS with CHD. Comparisons of management and outcome between group A and group B, subgroup A 1 and subgroup B 1, and subgroup A 2 and subgroup B 2 were performed. Statistical differences were analyzed by the χ 2 test at a P < .05. Results A total of 227 patients (male-female = 108:119) underwent surgical correction of CIDO. Of 227 patients, 86 (37.9%) were shown to have DS (group A), and 141 (62.1%) were NDS patients (group B). Demographic information did not differ between the groups, except for the mothers' age at pregnancy and the operative procedure used for duodenoduodenostomy. Group A had a higher incidence of CHD than group B (66.3% vs 26.2%; P < .05). Early outcome regarding survival rate (SR) was not statistically different between groups (87.2% vs 92.9%; P = .15), but the rate of early postoperative complications in group A was higher than that in group B (45.3% vs 31.1%; P = .03). The overall SR of group A at long-term follow-up was worse than that in group B (68.6% vs 86.7%; P < .05). Late complications and sequelae in group A were also more frequent than in group B (34.6% vs 9.2%; P < .05). When comparing early outcome between DS and NDS patients without CHD, the SR of subgroup A 1 was lower than that of subgroup B 1 (86.5% vs 96.5%; P = .04), but the rate of early postoperative complications did not differ (41.5% vs 24.1%; P = .05). At long-term follow-up, the overall SR of subgroup A 1 was worse than that of subgroup B 1 (73% vs 95.6%; P < .05) because of a higher incidence of late death because of recurrent respiratory tract infection in the A 1 subgroup. In addition, late complications and sequelae were more common in subgroup A 1 than in subgroup B 1 (37.5% vs 8.9%; P < .05). In patients with CHD, there was no statistical difference between early and long-term results of SR or complications between subgroup A 2 and subgroup B 2. The overall SR at long-term follow-up was 63% in subgroup A 2 and 56% in subgroup B 2 ( P = .5). Conclusions Early outcome of CIDO management revealed minimal differences between DS and NDS patients. However, these differences were clearer in long-term outcomes. The DS patients had lower long-term SR and higher rates of complications and sequelae than NDS patients. It is concluded that DS negatively impacts the management and outcome of patients with CIDO.

The Modified Kimura's Technique for the Treatment of Duodenal Atresia

Background/Purpose. Kimura's diamond-shaped-duodenoduodenostomy (DSD) is a known technique for the correction of congenital intrinsic duodenal obstruction. We present a modification of the technique and review the advantages of this new technique. Methods. From 1992 to 2006, 14 newborns were treated for duodenal atresia. We inverted the direction of the duodenal incisions: a longitudinal incision was made in the proximal duodenum while the distal was opened by transverse incision. Results. Our “inverted-diamond-shaped-duodenoduodenostomy” (i-DSD) allowed postoperative oral feeding to start on days 2 to 3, peripheral intravenous fluids discontinuity on days 3 to 8 (median values 3.6); time to achieve full oral feeds on days 8 to 12 (median values 9.4); the length of hospitalisation ranged from 10 and 14 days (median value 11.2). No complications related to the anastomosis, by Viz leakage, dehiscence, biliary stasis, or stenosis were observed. Conclusions. The i-DSD provides a safe procedure to protect the ampulla of Vater from injury and avoids any formation of a blind loop. The results show that patients who have i-DSD achieve full oral feeds in a very short time period and, consequently, the length of hospitalisation is also significantly reduced.

Congenital duodenal diaphragm in eight children.

BackgroundCongenital duodenal obstruction (CDO) is a common and usually easy to diagnose cause of intestinal obstruction in the newborn, except when the cause of the obstruction is a duodenal diaphragm. We describe our experience with eight children who had intrinsic duodenal obstruction secondary to a duodenal diaphragm.MethodsThe medical records of 22 children with the diagnosis of congenital intrinsic duodenal obstruction were reviewed for age at diagnosis, sex, gestation, birth weight, clinical features, associated anomalies, method of diagnosis, treatment and outcome. Operative findings and procedures were obtained from the operative notes.ResultsEight of the 22 children (36.4%) had congenital duodenal diaphragm (CDD). In all children, the diagnosis was made from plain abdominal X-ray, which showed the classic double-bubble appearance, and barium meal, which showed duodenal obstruction. Four patients had associated anomalies, including two with Down’s syndrome. Intraoperatively, five patients were found to have duodenal diaphragm with a central hole, while the other three had complete duodenal diaphragms. Postoperatively, all patients did well. Six required total parenteral nutrition.ConclusionsThe 100% survival rate among these children is comparable to that in Western countries, and can be attributed to the lack of major associated abnormalities, good perioperative management, and the availability of total parenteral nutrition.

Radiological assessment of duodenal calibre in congenital duodenal obstruction.

A review of barium meal studies in 32 cases of congenital intrinsic duodenal obstruction (CDO) from the Adelaide Children's Hospital was performed to assess and measure pre and post operative duodenal dilatation. Comparisons were made with a group of 153 normal barium studies from the same Hospital with a view to quantifying normal and abnormal proximal duodenal calibre. Duodenal size was expressed as a ratio D1/L1, (diameter of 1st part of duodenum/height of first lumbar vertebra). In this study a normal range of duodenal diameter was found to be 0.90 to 1.62 (+/- 2 SD from mean), with a mean of 1.26. The CDO patients recorded a range of postoperative ratios on first follow-up barium studies of between 1.2 and 4.5 with a mean of 2.28. Where serial studies had been performed, duodenal diameter was found to show some decrease in size postoperatively. In patients who underwent surgery for membrane obstruction there was a trend towards normalisation of duodenal diameter over time, compared with the atresia group. However, this difference did not reach statistical significance.

Complications associated with surgical treatment of congenital intrinsic duodenal obstruction

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 (8). The most frequent surgical procedure was side-to-side duodenoduodenostomy (DD), followed by duodenojejunostomy and resection of web with Heineke-Mikulicz 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 (8%). Six patients (18%) required secondary surgical procedures for postoperative complications (ie, 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.

Diamond-shaped anastomosis for duodenal atresia: An experience with 44 patients over 15 years

During the period between 1974 and 1988 44 patients were treated for congenital intrinsic duodenal obstruction using a diamond-shaped anastomosis (35 atresias, including two multiple atresias and nine stenoses). Neither gastrostomy nor transanastomotic tube was used. Postoperatively, oral feeding was started on days 2 to 6 (3.66 +/- 1.41 day). Intravenous fluid administration was discontinued on days 3 to 20 (7.54 +/- 3.58 day). Fourteen patients died, none related to the operative procedure. Of 30 survivors, 21 patients have been followed from 6 months to 15 years. Body weight was within the normal range at the latest visit. Current barium study performed in 19 patients showed no blind loop, megaduodenum, or anastomotic malfunction. The diamond-shaped anastomosis provides the following advantages: (1) early recovery of anastomotic function, and (2) avoidance of later complications, such as formation of a blind loop or anastomotic stenosis.

Congenital intrinsic duodenal obstruction: Problems in the diagnosis and management

Nineteen infants with intrinsic duodenal obstruction are analyzed. Atresia was the most common lesion. An exceptionally high rate of associated anomalies (73.7%) were present; Down's syndrome, the single most common anomaly, was seen in 47% of the infants. In seven infants, the diagnosis was delayed and in another three it was made intraoperatively while establishing a gastrostomy for esophageal atresia. Three infants died without operation because of gross prematurity and multiple anomalies. Of the 16 operated on, three died, one due to peritonitis and the other two because of metabolic derangements. Of the various operative procedures used, no significant difference was found in the final outcome of treatment. A schematic approach to the diagnosis and management is proposed.

Congenital intrinsic duodenal obstruction: A twenty-year review of its surgical management and consequences

<h2>Summary</h2> We have presented our findings in 158 cases of congenital intrinsic duodenal obstruction seen at the Hospital for Sick Children, Toronto, between 1952 and 1971. Of the 120 undergoing operation, the survival was 67%. The frequency of associated anomalies, especially those involving the esophagus, kidney, and anorectum, was high. Vomiting in the newborn demands early investigation of the alimentary tract, in particular, a plain film or gastrointestinal series, so that these children will be seen and operated on early with the best chance for survival. The etiology of the obstruction may vary from pure atresia to anomalous portal structure, and the possibility of an additional lesion in the duodenum with malrotation and bands must always be kept in mind. Low birth weight and associated anomalies continue to keep the mortality high and present a further challenge to the pediatric surgeon in dealing with these interesting problems.

Congenital intrinsic duodenal obstruction: Treatment by duodenoplasty : W. P. Tunell and N. K. Connolly. Clin. Proc. Children's Hospital, Wash., D.C. 25:206–213, (August) 1969

Congenital intrinsic duodenal obstruction: Treatment by duodenoplasty : W. P. Tunell and N. K. Connolly. Clin. Proc. Children's Hospital, Wash., D.C. 25:206–213, (August) 1969

Congenital intrinsic duodenal obstruction in infancy: A series of 30 cases treated over a 6 year period

Summary Of 30 cases of intrinsic duodenal obstruction of the duodenum in the newborn, atresia was more common than stenosis and postampullary lesions more common than preampullary ones. The prematurity rate of 50 per cent was high compared to other reports. Mortality figures were also high at 43.3 per cent, but are accounted for, to a large extent, by the prematurity rate and the high incidence of other severe congenital anomalies (53.3 per cent). Duodenojejunostomy has proved to be the most successful operation but had to be replaced by gastroenterostomy in certain circumstances. Antiperistaltic anastomoses, while being anatomically incorrect, may be more practical and function as well as isoperistaltic anastomoses.

Congenital intrinsic duodenal obstruction; report of 32 cases.

Congenital intrinsic duodenal obstruction; report of 32 cases.