Congenital Duodenal Atresia Research Articles

Factors associated with cholestasis after surgery for congenital duodenal atresia

Background: This study assessed the characteristics of neonates with duodenal atresia (DA) who developed transient postoperative cholestasis which resolved spontaneously, without identifiable congenital anomalies or surgical complications, and identified potential risk factors for cholestasis. Methods: Neonates with DA who underwent surgery at our institution between January 2009 and July 2022 were retrospectively reviewed. Demographic factors, intraoperative findings, placement of a trans-anastomotic tube (TAT), and postoperative outcomes were compared between patients who developed cholestasis (conjugated hyperbilirubinemia >2.0 mg/dL) after surgery (Group A) and those who did not (Group B). This report is a retrospective cohort study and complies with the STROBE statement. Results: Among 19 neonates with DA, 6 (31.6%) developed cholestasis after surgery, with the highest direct bilirubin value being 4.3 (2.4-6.5) mg/dL (median, [range]) on postoperative day 14.5 (2-23) that persisted for 67 (47-116) days until spontaneous resolution. Neonates in Group A had a significantly younger gestational age (36.6 vs. 38.0 weeks) (median) (p=0.038), a higher rate of Down syndrome (66.7 vs. 15.4%) (p=0.046), a higher rate of TAT placement (66.7 vs. 15.4%) (p=0.046), and longer administration of total parenteral nutrition (15.5 vs. 7.0 days) (p=0.027) than those in Group B. Conclusion: Transient cholestasis after surgery for DA seemed to be associated with prematurity, Down syndrome, parenteral nutrition, and TAT placement.

Retrospective evaluation of clinical and molecular data of 148 cases of esophageal atresia.

Developmental abnormalities provide a unique opportunity to seek for the molecular mechanisms underlying human organogenesis. Esophageal development remains incompletely understood and elucidating causes for esophageal atresia (EA) in humans would contribute to achieve a better comprehension. Prenatal detection, syndromic classification, molecular diagnosis, and prognostic factors in EA are challenging. Some syndromes have been described to frequently include EA, such as CHARGE, EFTUD2-mandibulofacial dysostosis, Feingold syndrome, trisomy 18, and Fanconi anemia. However, no molecular diagnosis is made in most cases, including frequent associations, such as Vertebral-Anal-Cardiac-Tracheo-Esophageal-Renal-Limb defects (VACTERL). This study evaluates the clinical and genetic test results of 139 neonates and 9 fetuses followed-up at the Necker-Enfants Malades Hospital over a 10-years period. Overall, 52 cases were isolated EA (35%), and 96 were associated with other anomalies (65%). The latter group is divided into three subgroups: EA with a known genomic cause (9/148, 6%); EA with Vertebral-Anal-Cardiac-Tracheo-Esophageal-Renal-Limb defects (VACTERL) or VACTERL/Oculo-Auriculo-Vertebral Dysplasia (VACTERL/OAV) (22/148, 14%); EA with associated malformations including congenital heart defects, duodenal atresia, and diaphragmatic hernia without known associations or syndromes yet described (65/148, 44%). Altogether, the molecular diagnostic rate remains very low and may underlie frequent non-Mendelian genetic models.

Laparoscopic Management of Congenital Intestinal Obstruction: Duodenal Atresia and Small Bowel Atresia.

Background: Congenital intestinal obstruction occurs in ∼1:2000 live births. Congenital duodenal atresia and duodenal stenosis are frequent causes of intestinal obstruction and occur in 1 per 5000-10,000 live births. Today, duodeno-duodeno anastomosis is still the treatment of choice, and it can be performed safely by minimally invasive surgery, although duodenojejunal anastomosis is surgically simpler and has equal results. Jejunum-ileal atresia or stenosis is a major cause of neonatal intestinal obstruction; its prevalence is 1:330-1:1500 live births. Nowadays, the ability of laparoscopic assisted identification of the atresia and repair by only exteriorization of the small bowel through the umbilicus makes this technique safe and feasible in almost every new born. Methods: This article will describe the operative technique of laparoscopic management of congenital duodenal and small bowel obstruction. Results: For congenital duodenal atresia patients, mortality rate is less than 5% and the majority are secondary to associated comorbidities. There is a low rate of anastomotic leaks, anastomotic stricture, delayed gastric emptying, and bacterial overgrowth. For small bowel atresia patients, complications include anastomotic leak, adhesions, small bowel obstruction, and short bowel syndrome in less than 5% of the patients. The rate of re-operations due to small bowel obstruction in laparoscopic assisted repair patients is less compared with laparotomy patients. Conclusions: Minimally invasive surgery for duodenal and small bowel atresia is safe and feasible and reduces the complications of open surgical procedures.

Congenital Duodenal Atresia Diagnosis, Treatment, and Influence on Further Development of Patients

Background: Congenital duodenal atresia (CDA) is a common and surgically treated digestive tract anomaly that develops in the early stage of pregnancy. It often coexists with trisomy 21 and other inborn defects. Surgery is the only way of treatment. This study aimed to investigate the relationship of CDA with early diagnosis, course of pregnancy, coexisting congenital defects, and further development of children. Methods: The data were collected using the medical history and a self-designed survey which consisted of 22 questions about the perinatal interview, coexisting inborn defects, after birth symptoms, time and methods of diagnosis, as well as the treatment outcome. Results: The surveys were sent to 31 patients who were diagnosed and treated because of CDA in our clinic between 2004 and 2019. According to the collected data, 73.7% of the patients were diagnosed prenatally with the mean time of diagnosis at 28th weeks of gestation. Moreover, 37% of the patients were preborn, and almost half of the patients had low and very low body weight. More than 2/3 of children presented coexisting inborn abnormalities. Among after birth symptoms, the most common ones were abdominal distention and lack of meconium. The majority of children were operated within 24 hours after birth. Conclusions: Prenatal diagnosis of CDA results in an earlier time of primary operation. Newborns diagnosed antenatally present fewer symptoms after the operation, fewer reoperations, and a shorter time of oral feeding toleration. The coexistence of other congenital defects increases patients’ mortality. The important factors that deteriorate the postoperative course are prematurity and low body weight. In order to avoid long-term complications, it is of utmost importance to provide follow-up.

A Case of Congenital Duodenal Atresia Associated With Biliary Atresia

A Case of Congenital Duodenal Atresia Associated With Biliary Atresia

MON-916 Pure Large Cell Neuroendocrine Carcinoma (LCNEC) of the Gall Bladder in a Patient with Down Syndrome - a Rare Case of an Aggressive Tumor of the Gall Bladder

Background: Pure large cell neuroendocrine carcinoma of the gall bladder (LCNEC-GB) is an extremely rare entity, with only 12 such cases reported in literature to date. None has been reported in a patient with genetic disorders. We describe the case of a patient with pure LCNEC-GB in the presence of Down syndrome and prior biliary atresia.Case:A 49-year-old female with Down Syndrome and history of neonatal surgery for congenital duodenal atresia presented with fever, vague abdominal discomfort and 6 month history of 15 pound weight loss. CT abdomen revealed a 5 X 4.2 cm exophytic, heterogeneously enhancing mass in the gall bladder fossa extending into segment 4B-5 of liver with mild intrahepatic biliary dilation, along with a 9 cm cystic lesion in continuity with the duodenum which was confirmed to a dilated duodenal anastomosis (from prior biliary surgery) on endoscopy. There was high suspicion for malignancy (gall bladder carcinoma versus intrahepatic cholangiocarcinoma) and subsequent metastatic workup including tumor markers, staging CT chest, MRI of the abdomen and diagnostic laparoscopy was negative.The patient underwent robotic converted to open en bloc resection of the gallbladder mass with segment 4B-5 liver resection and adherent loops of small bowel as well as resection of the dilated duodenal anastomosis followed by reconstruction with a gastrojejunostomy and Roux-en-Y/small bowel entero-enterostomy and closure of duodenotomy.Pathology demonstrated poorly differentiated “pure” large cell neuroendocrine carcinoma (G3), 6 cm in greatest dimension, with invasion of liver, duodenum and stomach, negative liver and gastric/small bowel margins. Lymphovascular invasion (LVI) was present with no perineural invasion (PNI), and 3/15 lymph nodes involved. Staging was determined to be pT4pN1.Patient was considered for adjuvant chemotherapy based on few case reports (platinum/etoposide) but unfortunately developed systemic complaints of fevers, fatigue, pain in bones and joints 2 months post operatively. Workup revealed metastatic disease which was confirmed on biopsy. Several small satellite liver lesions were also identified which was consistent with metastatic hepatic disease. Patient and family elected to proceed with hospice. Patient died within 4 months of surgery.Conclusion:Pure LCNEC-GB is an extremely rare and aggressive tumor with a poor prognosis as seen in our patient. This is the first reported case of pure LCNEC-GB in a patient with a genetic syndrome, although it is unknown if it had any causal relationship with the tumor. Prior biliary atresia/post -surgical inflammation may have also contributed to its pathogenesis by plausible development of metaplasia and expression of neuroendocrine cells which are normally absent in the gall bladder. Our case might help shed some light into pathogenesis and genetic basis if any of this rare entity.

2608 Teduglutide Therapy for a Patient With Chronic Intestinal Failure Following Multi-Visceral Transplantation

INTRODUCTION: The use of glucagon-like peptide-2 (GLP-2) agonists, such as Teduglutide, and recombinant human growth hormones (HGH), remain important for making advances in Short Bowel Syndrome and intestinal failure to reduce parenteral nutrition requirements. The efficacy of these agents have yet to be studied in the intestinal transplant population who experience decreased absorption with no evidence of acute rejection. We present a case of Teduglutide therapy for chronic intestinal failure following a multi-visceral transplant including stomach, pancreas and small bowel. CASE DESCRIPTION/METHODS: A 47-year-old Caucasian female presented with a history of corrected, congenital duodenal atresia, complicated by recurrent obstructions, requiring a Whipple procedure with bowel to bowel bypass and anastomosis. She developed short bowel syndrome and began to lose weight. She was placed on long-term total parenteral nutrition (TPN), with consequent hospital admissions for fungemia. She underwent a multi-visceral transplant including stomach, pancreas, and small intestine and placed on immunosuppressive therapy. Years later, she developed ongoing, severe watery diarrhea with recurrent need for TPN. She was placed on Pancrelipase and lipids, without any improvement. Intestinal biopsies repeatedly showed no signs of acute rejection or cytomegalovirus (CMV). Loperamide and Diphenoxylate were also trialed. Given a lack of progress, she was placed on Teduglutide. The patient experienced significant symptomatic improvement after 10 days of therapy. She was able to decrease her intravenous fluids (IVF) needs by 50%. After one month she again experienced worsening symptoms. She was placed on Linaclotide in addition to Teduglutide with the need to maximize TPN requirements. Repeat intestinal biopsies did not show evidence of infection or acute rejection. Teduglutide was discontinued after 10 months as it did not improve symptoms. DISCUSSION: Intestinal absorption can remain an issue in short bowel syndrome until transplant can be achieved. It remains problematic when multi-visceral transplant is performed and patients continue to experience malabsorption. Unfortunately, although our patient had a good initial response, this response did not persist and therefore therapy was discontinued. Research is ongoing to discover pharmacologic treatment options that significantly decrease the necessity of TPN and IVF. HGH and GLP-2 agonists are options at this time, however remain in question if they are effective long term.

Clinical characteristics and pregnancy outcomes of new onset epilepsy during pregnancy.

The occurrence of seizures during pregnancy is really a challenging situation which risks the health of both mothers and fetuses. However, new onset epilepsy is unpredictable in pregnancy, and its clinic feature is barely known. This study aimed to explore the clinical characteristics and pregnancy outcomes of new onset epilepsy during pregnancy.We screened consecutive women with epilepsy and reproductive history from June 2013 to November 2018 from 3 hospitals in West China. Detailed demographics, clinical features, neurological status, related tests, managements, seizure and pregnancy outcomes were recorded and followed-up. Within them, patients with first seizure during pregnancy and spontaneous recurrent seizures after delivery or abortion were defined as new onset epilepsy during pregnancy.We screened a total of 1041 consecutive women with epilepsy and reproductive history. Twenty-two of them (2.1%) had new onset epilepsy during pregnancy. The average age at seizure onset was 22.7 ± 3.0 years. All their first seizures occurred in pregnancy period, including 4 (18.2%) in the first trimester, ten (45.4%) in the second trimester and eight (36.4%) in the third trimester. Most patients delivered healthy babies, except one patient had to choose induced abortion because of the disappearance of fetal heart rate, one child was diagnosed with mild harelip and one was diagnosed with trisomy 21 syndrome, tetralogy of Fallot and congenital duodenal atresia. All 3 complications happened in patients with their first seizures in first trimester.Although the risk of new onset epilepsy during pregnancy was relatively low, accurate diagnosis and appropriate treatment were required to reduce the damage to both mothers and fetuses. New onset epilepsy during pregnancy mostly began in middle and late pregnancy. However, seizures occurred from early pregnancy had bad effects on the embryo or fetus.

Duodenal web presenting outside the neonatal period concurrently with malrotation and mid-gut volvulus

Abstract Fetal development of the intestinal tract involves multiple embryological events at the culmination of which the duodenojejunal junction is fixed in the left upper quadrant and cecum in the right lower quadrant. Developmental anomalies affecting the normal rotation of the fetal intestines occur as frequently as 1% of the population. Patients with malrotation are at risk for midgut volvulus, a surgical emergency with potential catastrophic outcomes with an incidence of 1:6000. Congenital duodenal atresia and stenosis is a frequent cause of intestinal obstruction in neonates and the incidence is estimated to be between 1:4000–15000 livebirths. Developmental failure of the recanalization of the fetal duodenum leads to an intrinsic web or atresia. Classically, malrotation with midgut volvulus is often diagnosed in a previously healthy term neonate with bilious emesis. While duodenal webs can have a similar presentation, the concurrence of both pathological entities leading to bowel obstruction is rarely reported. In this report we present a case of a 2 year old previously asymptomatic male child presenting with bilious emesis and found to have malrotation with volvulus and a concurrent duodenal web.

Down’s Syndrome ‑ An Independent Risk Factor of Outcomes in Isolated Congenital Duodenal Atresia

Introduction: There is no consensus in the available literature whether the coexistence of Down’s syndrome has adverse effect on the outcomes of neonates born with congenital duodenal atresia.Materials and Methods: A total of 29 neonates with congenital duodenal atresia were retrospectively studied for demographic details, sepsis parameters at admission, management, morbidity, and mortality. The neonates who were prematureResults: The sepsis parameters such as total leukocyte counts, erythrocyte sedimentation rate, serum procalcitonin levels, and serum C‑reactive protein levels were significantly increased, and the platelets were significantly decreased at admission in Group B subjects, as compared to Group A subjects. There were no complications noted in Group A, while Group B had significant morbidity. The neonates with congenital duodenal atresia with Down’s syndrome had 3.27 times more relative risk of mortality than those without Down’s syndrome. Down’s syndrome appears to be an independent risk factor for mortality in isolated congenital duodenal atresia with attributable risk of 37.8%.Conclusion: The presence of Down’s syndrome is a significant independent adverse risk factor of outcomes in isolated congenital duodenal atresia.

A Case Report of Anastomotic Ulcers, 11 Years After Diamond-Shaped Anastomosis for Congenital Duodenal Atresia

A Case Report of Anastomotic Ulcers, 11 Years After Diamond-Shaped Anastomosis for Congenital Duodenal Atresia

Рідкісний випадок успішного симультанного хірургічного лікування природженої атрезії дванадцятипалої кишки та омфалоцелє в недоношеної новонародженої дитини

Рідкісний випадок успішного симультанного хірургічного лікування природженої атрезії дванадцятипалої кишки та омфалоцелє в недоношеної новонародженої дитини

Laparoscopic repair of congenital duodenal obstruction is feasible even in small-volume centres.

INTRODUCTION It has been suggested that laparoscopic repair of congenital duodenal obstruction (CDO) should be restricted to a limited number of designated centres of expertise. After gaining extensive experience with intracorporeal suturing in other procedures, we evaluated the feasibility of this approach at the Royal Hospital for Sick Children (RHFSC; Edinburgh, UK). METHODS We conducted a retrospective review of all cases of CDO presenting to the RHFSC from 2012 to 2014. Cases were identified from our electronic database using standardised codes. Data comprised: gestation; birth weight; associated anomalies; patient age and weight at surgery; operative time; complications; postoperative course. RESULTS Five consecutive non-selected cases of isolated CDO were repaired laparoscopically, and all were carried out by the senior surgeon. The male:female ratio was 4:1. Corrected gestational age at surgery was 35-38 weeks, and the weight at surgery was 1.7-3.1 kg. None of our patients had significant associated anomalies. CONCLUSIONS The present study demonstrates the feasibility of laparoscopic repair of CDO in small-volume centres, and is the first report of laparoscopically managed congenital duodenal atresia in twins.

先天性十二指腸閉鎖症・狭窄症におけるtransanastomotic tubeの有用性の検討

先天性十二指腸閉鎖症・狭窄症におけるtransanastomotic tubeの有用性の検討

Laparoscopic management of congenital duodenal atresia or stenosis: A single-center early experience

BackgroundThe background is to review our experience with laparoscopic repair of congenital duodenal atresia or stenosis (CDAS) and compare postoperative outcome with a group control of laparotomy repair. MethodsRetrospective chart review of all cases of CDAS undergoing laparoscopic surgery at our institution between July 2013 and May 2014 and comparison with a group control of open operation performed between 2007 and 2010. Data were compared using Fisher's exact test for qualitative values and Mann–Whitney test for quantitative values. P values less than 0,05 were considered statistically significant. ResultsTen consecutive cases were identified in laparoscopic group (7 duodenoduodenostomy and 3 duodenojejunostomy) and 19 cases in laparotomy group (16 duodenoduodenostomy and 3 web excision). Median birth weight was lower in laparoscopic group (2125 grams Vs 2777 grams p=0,04). In laparoscopic group, there was no conversion and no intraoperative complication. Median duration of surgery was 90minutes (80–150). In both groups, the surgical morbidity rate was 10%. Median time to initiation of oral feeding was significatively shorter in laparotomy group (8days Vs 4 p=0,009). Median time to full oral feeding and length of stay were shorter in laparotomy but not statistically different. (36days Vs 16,5 p=0,14 and 45,5days Vs 25,5 p=0,09 respectively) After a median follow up of 149,5days (24–355) in laparoscopic group, 8 children had a full oral intake. Five children had a weight below the 10th percentile. ConclusionThe laparoscopic approach for CDAS is safe and reproducible with outcomes similar to open repair even in the beginning of a learning curve for pediatric surgeons with appropriate laparoscopic skills. In this small series, laparoscopy did not appear to decrease time to full oral intake or length of stay. Larger studies are suggested to provide more conclusive results.

Duodenal web resection via hybrid natural orifice transluminal endoscopic surgery (NOTES)

Congenital duodenal atresia, stenosis, and webs are a common cause of proximal obstruction in the newborn period. A variety of surgical techniques have been described for the treatment of duodenal webs. Open and laparoscopic duodenotomy with web resection have been well described; endoscopic resection has also shown to have promising success. We describe a case of a 2-year-old male with a duodenal web, which was resected via an existing gastrostomy site utilizing a modified hybrid natural orifice transluminal endoscopic surgery (NOTES). • Duodenal webs are a common cause of neonatal duodenal obstruction. • High index of suspicion is needed to diagnose duodenal webs after infancy. • Hybrid NOTES is a safe and effective approach for the excision of duodenal webs. • Approach can be performed without an endoscope.

Recurrent Biliary Obstruction Overcome With Metal Biliary Stent Placement in the Surgically Altered Down Syndrome Patient for Congenital Duodenal Atresia

Recurrent Biliary Obstruction Overcome With Metal Biliary Stent Placement in the Surgically Altered Down Syndrome Patient for Congenital Duodenal Atresia

Abdominal Swelling in a Newborn With Trisomy 21

A newborn with trisomy 21 presents with abdominal swelling (Figure 1). Figure 1. Abdominal Swelling 19 days after birth ### Prenatal and Birth Histories The spontaneous vaginal delivery in the hospital had no complications. ### Physical Examination at Birth ### Later Findings

Congenital duodenal atresia with ‘apple-peel configuration’ of the small intestines and absent superior mesenteric artery: A case report and review of literature

Embryo logically, congenital duodenal atresia is a primary malformation that results from errors in recanalization of the duodenum in early gestation. This is in contrast to other intestinal atresias which result from vascular accidents. We report an unusual and rare case of congenital duodenal atresia, loss of the third and fourth parts of the duodenum with apple-peel configuration of remaining small bowel and absent superior mesenteric artery in a preterm child. The possibility of Strømme syndrome must also be kept in mind. The literature on the subject is also reviewed.

Two Cases of Congenital Duodenal Atresia With Postoperative Anastomotic Ulcers

Two Cases of Congenital Duodenal Atresia With Postoperative Anastomotic Ulcers