Exploring the clinical characteristics and prevalence of the annular pancreas: a meta-analysis.

BACKGROUND: The annular pancreas (AP) is a rare gastrointestinal congenital malformation, in which malrotation of the pancreatic ventral bud in the seventh week of embryonic development manifests in a partial or complete ring of tissue around the second part of the duodenum. METHODS: The main online medical databases such as PubMed, ScienceDirect, Wiley online library, Web of Science, and EBSCO discovery service were used to gather all relevant studies on the AP. RESULTS: A total of 12,729,118 patients were analyzed in relation to the prevalence of AP. The pooled prevalence of AP was 0.0045% (95% CI: 0.0021%-0.0077%). The most frequent comorbidity in adults and children was duodenal obstruction, with a pooled prevalence of 24.04% and 52.58%, respectively (95% CI: 6.86%-46.48% and 35.56%-69.31%, respectively). The most frequent operation in adult patients with AP was duodenojejunostomy, with pooled prevalence established at 3.62% (95% CI: 0.00%-10.74%). CONCLUSION: The diagnostic complexity of AP is accentuated by its nonspecific clinical symptoms, making accurate identification reliant on imaging studies. Therefore, having a thorough knowledge of the clinical characteristics of the AP and its associated anomalies becomes paramount when faced with this rare congenital condition.

Dynamic ultrasonography for optimizing treatment position in superior mesenteric artery syndrome: Two case reports and review of literature.

BACKGROUND: Superior mesenteric artery (SMA) syndrome is a rare cause of duodenal obstruction by extrinsic compression between the SMA and the aorta (SMA-Ao). Although the left lateral recumbent position is considered effective in the treatment of SMA syndrome, individual variations in the optimal patient position have been noted. In this report, we present two elderly cases of SMA syndrome that exhibited rapid recovery due to ultrasonographic dynamic evaluation of the optimal position for each patient. CASE SUMMARY: Case 1: A 90-year-old man with nausea and vomiting. Following diagnosis of SMA syndrome by computed tomography (CT), ultrasonography (US) revealed the SMA-Ao distance in the supine position (4 mm), which slightly improved in the lateral position (5.7-7.0 mm) without the passage of duodenal contents. However, in the sitting position, the SMA-Ao distance was increased to 15 mm accompanied by improved content passage. Additionally, US indicated enhanced passage upon abdominal massage on the right side. By day 2, the patient could eat comfortably with the optimal position and massage. Case 2: An 87-year-old woman with vomiting. After the diagnosis of SMA syndrome and aspiration pneumonia by CT, dynamic US confirmed the optimal position (SMA-Ao distance was improved to 7 mm in forward-bent position, whereas it remained at 5 mm in the supine position). By day 7 when her pneumonia recovered, she could eat with the optimal position. CONCLUSION: The optimal position for SMA syndrome varies among individuals. Dynamic US appears to be a valuable tool in improving patient outcomes.

Esophageal Atresia Associated with Congenital Duodenal Obstruction: Turkish Esophageal Atresia Registry (TEAR) Evaluation.

INTRODUCTION: Coexistent congenital duodenal obstruction and esophageal atresia (EA) is known to have significant morbidity and mortality. Management strategies are not well-defined for this association. The data from the Turkish EA registry is evaluated. MATERIALS AND METHODS: A database search was done for the years 2015 to 2022. RESULTS: Among 857 EA patients, 31 (3.6%) had congenital duodenal obstruction. The mean birth weight was 2,104 (± 457) g with 6 babies weighing less than 1,500 g. Twenty-six (84%) had type C EA. The duodenal obstruction was complete in 15 patients and partial in 16. Other anomalies were detected in 27 (87%) patients. VACTERL-H was present in 15 (48%), anorectal malformation in 10 (32%), a major cardiac malformation in 6 (19%), and trisomy-21 in 3 (10%). Duodenal obstruction diagnosis was delayed in 10 (32%) babies for a median of 7.5 (1-109) days. Diagnosis for esophageal pathologies was delayed in 2. Among 19 babies with a simultaneous diagnosis, 1 died without surgery, 6 underwent triple repair for tracheoesophageal fistula (TEF), EA, and duodenal obstruction, and 3 for TEF and duodenal obstruction in the same session. A staged repair was planned in the remaining 9 patients. In total, 15 (48%) patients received a gastrostomy, the indication was long-gap EA in 8. Twenty-five (77%) patients survived. The cause of mortality was sepsis (n = 3) and major cardiac malformations (n = 3). CONCLUSION: Congenital duodenal obstruction associated with EA is a complex problem. Delayed diagnosis is common. Management strategies regarding single-stage repairs or gastrostomy insertions vary notably depending on the patient characteristics and institutional preferences.

Combined esophageal and duodenal atresia: A review of the literature from 1950 to 2020.

The combination of duodenal atresia (DA) and esophageal atresia (EA) is very rare. With improvements in prenatal sonography and the use of fetal magnetic resonance imaging (MRI), these malformations can be diagnosed in a more accurate and timely manner; polyhydramnios remains the most common sign despite having a low specificity. The high rate of associated anomalies (in 85% of cases) can also impact neonatal management and increase the morbidity rate; thus, it is of paramount importance to look for every possible associated malformation, such as VACTERL and chromosomic anomalies. The surgical management of this combination of atresias is not well defined and changes according to the patient's clinical status, the type of EA, and the other associated malformations. Management ranges from a primary approach for one of the atresias with delayed correction of the other (56.8%) to a simultaneous repair of both atresias (33.8%) with or without gastrostomy, or total abstention (9.4%). We suggest that a simultaneous approach can be safely performed on patients in good physical condition, with a birth weight over 1500 g, and with no major respiratory distress; this method begins by closing the tracheoesophageal fistula to protect the lung and then repairing the DA. The mortality rate has decreased over the years, dropping from 71% before 1980 to 24% after 2001. In this review, we present the available evidence on these conditions, focusing mostly on the epidemiology, prenatal diagnosis, neonatal management strategies, and outcome, with the aim of determining how the different clinical features and surgical approaches may impact on morbidity and mortality.

Obstrução duodenal por cálculo - síndrome de bouveret: um relato de caso / Duodenal obstruction by calculus - bouveret syndrome: a case report

Introdução: A Síndrome de Bouveret é uma complicação rara, causada por um cálculo biliar grande que obstrui o intestino por meio de uma fístula e tem como principais sintomas: náuseas, vômitos, dor abdominal e distensão. O diagnóstico é feito por meio de exames de imagem, o tratamento é desafiador e pode envolver cirurgia ou procedimentos endoscópicos. Tem morbimortalidade considerável, mas a falta de diretrizes padrão dificulta o diagnóstico e o tratamento. Objetivos: Apresentar um relato de caso de paciente com características fisiopatológicas e diagnóstico de Síndrome de Bouveret, discutir a sintomatologia clínica, diagnóstico, tratamento e o desfecho cirúrgico. Método: Relato de caso realizado por revisão de prontuário e exames de paciente com síndrome de Bouveret. Apresentação: Feminina, 62 anos, quadro clínico compatível com obstrução duodenal. Tomografia de abdômen: imagem ectópica radiopaca, distensão hidroaérea intestinal, sugerindo cálculo biliar, confirmada através de endoscopia digestiva alta. Submetida a gastrotomia, seguida de gastrorrafia, sem reparo da fístula colecistogástrica. Discussão: Síndrome de Bouveret é mais comum em idosos e mulheres com antecedentes de cálculos biliares grandes. O diagnóstico é feito por meio de exames de imagem, geralmente por tomografia computadorizada e o tratamento visa remover o cálculo e aliviar a obstrução intestinal de maneira menos invasiva possível. Por alto risco cirúrgico, o tratamento endoscópico por meio de métodos como retirada com cesta ou litotripsia é indicado, porém, requer múltiplos procedimentos e possui limitações. A abordagem cirúrgica pode ser realizada em um ou dois tempos, dependendo das condições do paciente. Conclusão: Exames de imagens aliados à clínica são essenciais para o diagnóstico e tratamento com gastrostomia e o reparo de fistula em dois tempos se mostra uma ótima opção

Introduction: Bouveret syndrome is a rare complication, caused by a large gallstone that obstructs the intestine through a fistula and has as main symptoms: nausea, vomiting, abdominal pain and distension. The diagnosis is made through imaging tests, the treatment is challenging and may involve surgery or endoscopic procedures. It has considerable morbidity and mortality, but the lack of standard guidelines makes diagnosis and treatment difficult. Objectives: To present a case report of a patient with pathophysiological characteristics and diagnosis of Bouveret syndrome, to discuss the clinical symptomatology, diagnosis, treatment and surgical outcome. Method: Case report performed by review of medical records and examinations of patients with Bouveret syndrome. Presentation: Female, 62 years old, clinical picture compatible with duodenal obstruction. Abdominal CT scan: radiopaque ectopic image, intestinal hydrorespiratory distension, suggesting gallstone, confirmed by upper digestive endoscopy. Submitted to gastrotomy, followed by gastrorraphy, without repair of the cholecystogastric fistula. Discussion: Bouveret syndrome is more common in the elderly and women with a history of large gallstones. The diagnosis is made through imaging tests, usually by computed tomography and the treatment aims to remove the stone and relieve intestinal obstruction in the least invasive way possible. Due to high surgical risk, endoscopic treatment through methods such as basket removal or lithotripsy is indicated, however, it requires multiple procedures and has limitations. The surgical approach can be performed in one or two times, depending on the patient's conditions. Conclusion: Imaging exams combined with the clinic are essential for diagnosis and treatment with gastrostomy and two-stage fistula repair is a great option

Introducción: El Síndrome de Bouveret es una complicación rara, causada por un gran cálculo biliar que obstruye el intestino a través de una fístula y tiene como síntomas principales: náuseas, vómitos, dolor abdominal y distensión. El diagnóstico se realiza a través de pruebas de imagen, el tratamiento es desafiante y puede involucrar cirugía o procedimientos endoscópicos. Tiene una morbilidad y mortalidad considerables, pero la falta de pautas estándar dificulta el diagnóstico y el tratamiento. Objetivos: Presentar un reporte de caso de un paciente con características fisiopatológicas y diagnóstico de Síndrome de Bouveret, discutir la sintomatología clínica, diagnóstico, tratamiento y resultado quirúrgico. Método: Reporte de caso realizado mediante revisión de historias clínicas y exámenes de un paciente con síndrome de Bouveret. Presentación: Mujer, 62 años, cuadro clínico compatible con obstrucción duodenal. Tomografía de abdomen: imagen ectópica radiopaca, distensión intestinal hidroaérea, sugestiva de cálculos biliares, confirmada por endoscopia digestiva alta. Sometido a gastrotomía, seguida de gastrorrafia, sin reparación de la fístula colecistogástrica. Discusión: El síndrome de Bouveret es más común en ancianos y mujeres con antecedentes de cálculos biliares grandes. El diagnóstico se realiza mediante pruebas de imagen, generalmente por tomografía computarizada, y el tratamiento tiene como objetivo eliminar el cálculo y aliviar la obstrucción intestinal de la forma menos invasiva posible. Debido al alto riesgo quirúrgico, está indicado el tratamiento endoscópico mediante métodos como la extracción con canastilla o la litotricia, sin embargo, requiere múltiples procedimientos y tiene limitaciones. El abordaje quirúrgico se puede realizar en una o dos etapas, dependiendo de las condiciones del paciente. Conclusión: Los exámenes de imagen combinados con la clínica son fundamentales para el diagnóstico y tratamiento con gastrostomía y la reparación de fístulas en dos tiempos demuestra ser una gran opción.

Incidental Preduodenal Portal Vein Causing Partial Duodenal Obstruction and Hypertrophied Pre-Pyloric Gastric Polyp.

Preduodenal portal vein (PDPV) is a rare congenital anomaly in which the portal vein lies anterior to the duodenum rather than its normal posterior position. It is a known rare cause of duodenal obstruction and can be associated with other anomalies such as malrotation with or without jejunal atresia. Presented is an incidentally found PDPV causing partial duodenal obstruction during exploration for the resection of a gastric mass and placement of open gastrostomy tube for feeding. This was managed with duodenoduodenostomy, re-creating normal anatomy with portal.

[Delayed complication of surgical treatment of duodenal atresia in neonatal period]. / Pozdnee oslozhnenie khirurgicheskogo lecheniya atrezii dvenadtsatiperstnoi kishki v neonatal'nom periode.

We report a 48-year-old woman who underwent surgery in early neonatal period for duodenal atresia and developed subsequent diseases of the upper gastrointestinal tract. Symptoms of gastric outlet obstruction, gastrointestinal bleeding and malnutrition developed over the past 5 years. Inflammatory and cicatricial lesions of gastrojejunostomy formed for congenital duodenal obstruction following annular pancreas required reconstructive surgery.

[Intraluminal endoscopy for biliary duodenal obstruction]. / Vnutriprosvetnaya endoskopiya pri biliarnoi duodenal'noi neprokhodimosti.

Cholelithiasis complicated by cholecystoduodenal fistula and small bowel biliary obstruction is an absolute indication for surgical treatment. Modern possibilities of intraluminal endoscopy (electrohydraulic lithotripsy) made it possible to avoid intra-abdominal access (laparotomy, laparoscopy) and postoperative complications. Finally, rapid rehabilitation was noted.

Duodenal Volvulus due to Duodenum Inversum.

Duodenum inversum, or inverted duodenum, is a rare congenital disorder resulting in an abnormal position of the third and/or fourth portions of the duodenum. Most patients are asymptomatic, but others can experience pain, nausea, vomiting, peptic ulcer disease, pancreatitis, and even intestinal obstruction. In this case, we report a patient who presented with acute abdominal pain and vomiting. He was diagnosed with duodenal volvulus and obstruction due to duodenum inversum. After failing nonoperative management, the patient was successfully treated with surgical resection.

[Surgical management of duodenal atresia due to annular pancreas and intestinal atresia IIIb]. / Manejo quirúrgico de atresia duodenal por páncreas anular y atresia intestinal IIIb.

Background: The presence of duodenal atresia related to type IIIb intestinal atresia is a rare association, with few cases reported in the literature, representing a surgical challenge considering that even isolated cases of type IIIb intestinal atresia are a challenge. The objective was to report the successful surgical management of a case of a complex intestinal malformation, characterized by duodenal occlusion secondary to annular pancreas and type IIIb intestinal atresia, with intestinal malrotation by definition and the presence of Meckel's diverticulum. Clinical case: We present the case report of a newborn sent to the second level of care with a diagnosis of duodenal obstruction not diagnosed prenatally, which resulted in duodenal atresia due to annular pancreas and type IIIb intestinal atresia according to the Grosfeld classification. The presence of duodenal atresia with type IIIb intestinal atresia is an extremely rare condition, even more so associated with annular pancreas. These cases are a challenge considering the short length of the small intestine and its consequent need for total parenteral nutrition for a prolonged period. Conclusions: The surgical management of this complex intestinal malformation resulted in a case with an adequate post-surgical evolution, based on the immediate start of enteral feeding with a short period of need for total parenteral nutrition that finally resulted in a short hospital stay.

Introducción: la presencia de atresia duodenal relacionada con atresia intestinal tipo IIIb es una asociación rara, con pocos casos reportados en la literatura, y representa un reto quirúrgico si se toma en cuenta que incluso los casos aislados de atresia intestinal tipo IIIb lo representan. El objetivo fue reportar el manejo quirúrgico exitoso del caso de una malformación intestinal compleja, caracterizada por una oclusión duodenal secundaria a páncreas anular y atresia intestinal tipo IIIb, con una malrotación intestinal por definición y la presencia de divertículo de Meckel. Caso clínico: reportamos el caso de un recién nacido enviado de segundo nivel de atención con un diagnóstico de obstrucción duodenal no diagnosticado prenatalmente, que resultó en atresia duodenal por páncreas anular y atresia intestinal tipo IIIb, según la clasificación de Grosfeld. La presencia de atresia duodenal con atresia intestinal tipo IIIb es una condición extremadamente rara y todavía lo es más asociada con páncreas anular. Estos casos son un desafío si se toma en cuenta la corta longitud de intestino delgado y su consiguiente necesidad de nutrición parenteral total por un periodo prolongado. Conclusiones: el manejo quirúrgico de esta malformación intestinal compleja resultó en un caso con una adecuada evolución postquirúrgica, basada en el inicio mediato de alimentación enteral con un periodo corto de necesidad de nutrición parenteral total que finalmente resultó en una corta estancia hospitalaria.

Small Bowel Congenital Anomalies: A Review and Update.

The small intestine is a complex organ system that is vital to the life of the individual. There are several congenital anomalies that occur and present most commonly in infancy; however, some may not present until adulthood. Most congenital anomalies of the small intestine will present with obstructive symptoms, whereas some may present with vomiting, abdominal pain, and/or gastrointestinal bleeding. Various radiologic procedures can aid in the diagnosis of these lesions that vary depending on the particular anomaly. The congenital anomalies of the small intestine discussed include Meckel diverticulum, duodenal web, duodenal atresia, jejunoileal atresia, and intestinal duplications.

Congenital duodenal obstruction - Advances in diagnosis, surgical management, and associated controversies.

Congenital duodenal obstruction (CDO) occurs due to intrinsic and extrinsic mechanisms but is most often caused by intrinsic duodenal atresia and stenosis. This review will summarize the history, epidemiology, and etiologies associated with the most common causes of CDO. The clinical presentation, complex diagnostic considerations, and current surgical repair options for duodenal atresia and stenosis will also be discussed. Finally, both historical and recent controversies which continue to affect the surgical decision-making in the management of these patients will be highlighted.

Dual duodenojejunostomies in the repair of megaduodenum, duodenal stenosis and prior missed diagnosis of annular pancreas.

Duodenal stenosis and atresia are some of the most common forms of congenital bowel obstruction. The gold standard approach to treatment is duodenoduodenostomy, while rare, gastrojejunostomy and duodenojejunostomies may still be used. We report a case of a 7-year-old male presenting with annular pancreas with duodenal stenosis that was diagnosed at birth as primary duodenal atresia and repaired by gastrojejunostomy with a Braun enteroenterostomy. Through successful reoperation with dual duodenojejunostomy and subsequent management, we treated the sequelae of the initial repair, including megaduodenum. Regions of duodenal obstruction must have direct anastomotic repair to prevent subsequent issues related to dysmotility. Otherwise, further surgical intervention and long-term medical management, such as the novel strategy reported, may be necessary.

Annular pancreas in neonates; Case series and review of literatures.

BACKGROUND: The annular pancreas is a rare congenital disorder of the pancreas first recognized in 1818. It is believed to result from faulty rotation of the ventral pancreatic bud in its course around the posterior aspect of the duodenal anlage. The duodenum is encircled and might be obstructed by normal pancreatic tissue. The management of the annular pancreas is still developing and under revision. CASE PRESENTATION: Six cases of neonatal intestinal obstruction secondary to the annular pancreas diagnosed, operated on, and involved in our study. Age, gender, maturity, age at referral, birth weight, clinical presentation, imaging findings, associated congenital anomalies, treatment, complications, and hospital stay were all studied. Polyhydramnios is found in 3 cases (50%). Down syndrome was diagnosed in one case. One patient has associated malrotation. Symptoms started earlier within the first 24 hours. Vomiting was bile stained in 4 cases (66.7%). Passing meconium, sometimes frequent, does not exclude the annular pancreas. Most patients show double bubbles sign on plain abdominal X-ray. All six neonates were treated with duodenoduodenostomy with excellent results. Survival was 100% and complications were minimum. CONCLUSION: The annular pancreas, although rare, is an important cause of neonatal duodenal obstruction. The accurate diagnosis is usually performed during laparotomy. Vomiting may contain bile or not, furthermore, passing meconium does not exclude this condition. The best and the excellent surgical option is diamond duodenoduodenostomy. This case series might be added to the registered cases of the annular pancreas to standardize the method of diagnosis and to define the best management.

Duodenal obstruction due to missed pre-duodenal portal vein in a patient with intestinal malrotation.

Pre-duodenal portal vein (PDPV) is a rare anomaly and a rare cause of duodenal obstruction (DO), with only a few cases reported in the literature. We present an infant whose bilious vomiting persisted despite having Ladd's procedure for intestinal malrotation due to a missed diagnosis of DO from PDPV that was found at re-exploration. The patient was diagnosed with malrotation and had Ladd's procedure at 12 weeks of age, but bilious vomiting persisted post-operatively. The patient presented to us after 4 weeks, was clinically malnourished and dehydrated, resuscitation was done and re-exploratory laparotomy performed, where an obstructing PDPV was found and a duodeno-duodenostomy was performed anterior to PDPV. However, the patient died on post-operative day 7 probably from severe malnutrition due to delayed diagnosis and absence of parenteral nutrition. We conclude that PDPV may be a cause of DO in infants with malrotation and should be properly sought for during Ladd's procedure for possible bypass surgery if found.

Neonate with Congenital Duodenal Obstruction and Ectopic Hepatic Parenchyma.

BackgroundThe presence of hepatic parenchyma at ectopic locations is infrequently reported in neonatal age. Case details: A male neonate presented with clinical signs and symptoms of duodenal obstruction. At exploration, an annular pancreas was found as the causative factor and he underwent a Kimura's duodeno-duodenostomy. A pedicled cyst was attached to the stomach's greater curvature, was excised, and histologically was a mesothelial-lined cyst with ectopic liver, complete with bile ducts, in the cyst wall. Conclusion: Ectopic liver tissue may be clinically silent and found within the wall of a mesothelial cyst. Long-term complications of this ectopic tissue are not known.

Diverticular disease of the small bowel: a rare cause of the duodenojejunal flexure obstruction (a case report).

The small bowel is the least common site for diverticula in the entire gastrointestinal tract. Chronic upper intestinal obstruction due to diverticula is very rare. We report a case of multiple small bowel diverticula causing mechanical obstruction of the duodenojejunal flexure.