Annular Pancreas: Unusual Presentation of a Rare Malformation Article - PDF document

See discussions, stats, and author profiles for this publication at: https://www.researchgate.net/publication/333118183 Annular Pancreas: Unusual Presentation of a Rare Malformation Article in Revista portuguesa de pediatria · May 2019 DOI: 10.25754/pjp.2019.14168 CITATIONS READS 0 49 5 authors , including: Sofia Amante José Estevão-Costa Hospital do Divino Espírito Santo de Ponta Delgada, EPE Centro Hospitalar Universitário São João, Faculty of Medicine of Porto 5 PUBLICATIONS 1 CITATION 119 PUBLICATIONS 901 CITATIONS SEE PROFILE SEE PROFILE Ana Catarina Fragoso Hospital de São João, Faculdade de Medicina do Porto, Portugal 40 PUBLICATIONS 368 CITATIONS SEE PROFILE Some of the authors of this publication are also working on these related projects: Artificial nutrition in children View project Minimal invasive access surgery View project All content following this page was uploaded by José Estevão-Costa on 17 February 2020. The user has requested enhancement of the downloaded file.

CASE REPORT Annular Pancreas: Unusual Presentation of a Rare Malformation Joana Fortuna 1 , Ana Luísa Rodrigues 1 , Sofia Amante 2 , Rui Amaral 2 , José Estevão-Costa 3 , Ana Catarina Fragoso 3 Port J Pediatr 2019;50:122-5 DOI: htups://doi.org/10.25754/pjp.2019.14168 Abstract that occurs in about one out of 10,000-20,000 live births. It is characterized by a band of pancreatjc tjssue that partjally or totally surrounds the second portjon of the A case of a 6-month-old female infant presentjng post - duodenum, causing a variable degree of obstructjon. 4 prandial vomitjng and poor weight gain is presented. Herein, we report a clinical case of complete annular She was malnourished and dehydrated with no abdom - pancreas that resulted in partjal duodenal obstructjon inal distension or neurological signs. Laboratory testjng presentjng at a late age. revealed hypochloremic hypokalemic alkalosis. Afuer the exclusion of infectjous disease and food intolerance, plain abdominal X-rays revealed a “double bubble” Case Report sign with distal air, thereby suggestjng partjal duodenal obstructjon. An upper gastrointestjnal contrast study We report on a case of a caucasian 6-month-old female, confjrmed this diagnosis. She was then referred to a as the second daughter of healthy parents. She was tertjary hospital for surgical treatment. At surgery, a born from a term gestatjon with adequate follow-up duodenum-duodenostomy was performed due to a and no intercurrences. The somatometry at birth was complete annular pancreas, with an excellent outcome. appropriate for gestatjonal age; the neonatal period had Annular pancreas is a rare entjty. When symptoms arise, no complicatjons with the passing of meconium in the they usually occur in the neonatal period. As the present fjrst 24 hours of life. case illustrates, it may be symptomatjc at later ages, At 4 months of age, when she started food diversifj - requiring a high grade of suspicion in order to diagnose catjon, intermituent postprandial vomitjng developed, and treat it early. predominantly afuer pasty meals (soup, baby cereals, or fruit). Episodes of vomitjng became progressively more Keywords : Duodenal Obstructjon/congenital; Duodenal intense (in frequency and content). At 6 months of age, Obstructjon/diagnosis; Duodenal Obstructjon/therapy; the weight decreased from percentjle (P) 50 to under Infant; Pancreas/abnormalitjes P3 and height from P50 to P15. The bowel habit was irregular but with normal consistency stools. Introduction At admission, she was malnourished with loss of mus - cle mass and subcutaneous fat. The abdomen was depressed with no palpable masses or organomegaly. Congenital intestjnal obstructjon of the duodenum The remaining examinatjon was unremarkable. Labora - occurs in one out of 2,500 to 10,000 births. 1 The typical tory testjng revealed hypochloremic hypokalemic meta - clinical presentatjon occurs in the neonatal period with bolic alkalosis (pH 7.59, chlorine 91 mmol/L, potassium early and frequent vomitjng. However, the associated 3.28 mmol/L). Renal and hepatjc functjons were within symptomatology depends not only on the degree of ste - the normal ranges. There was no evidence of infectjous nosis but also on the type of anatomical defect that may disease and no alteratjons in food specifjc immunoglob - be complete or incomplete, extrinsic or intrinsic. Duode - ulins or celiac disease specifjc antjbodies. nal atresia, including duodenal membrane, are classifjed Abdominal ultrasound showed the dilatatjon of the as intrinsic defects, whereas intestjnal malrotatjon, duo - stomach with water and gas content. Plain abdominal denal duplicatjon, annular pancreas, and preduodenal X-rays revealed a “double bubble” sign (gastric and portal vein are considered extrinsic defects. 1-3 duodenal dilatatjon) with distal air, suggestjng partjal The annular pancreas is a rare congenital malformatjon 1. Pediatrics Department, Hospital do Divino Espírito Santo, Ponta Delgada, Azores, Portugal 2. Radiology Department, Hospital do Divino Espírito Santo, Ponta Delgada, Azores, Portugal 3. Pediatrics Surgery Department, São João Hospital Centre, Faculty of Medicine, Oporto, Portugal Corresponding Author Joana Fortuna fortuna_joana@hotmail.com Rua Dr. Filipe Álvares Cabral, 21 R/C D, 9500-182 Ponta Delgada, São Miguel, Azores, Portugal Received: 12/04/2018 | Accepted: 13/12/2018 122 Portuguese Journal of Pediatrics

Annular Pancreas duodenal obstructjon (Fig. 1). The upper gastrointestjnal contrast study demonstrated an exuberant dilatatjon of the stomach and fjrst duodenal portjon, with the fjliform progression of contrast to the second portjon Weight of the duodenum afuer about 40 minutes of the begin - ning of the examinatjon, confjrming the diagnosis of partjal duodenal obstructjon (Fig. 2). The child was then referred to a tertjary hospital where she underwent surgical treatment that revealed a “complete” annular pancreas that was managed with a side to side duode - num-duodenostomy. Age (months and years) The postoperatjve course was uneventgul with oral tol - Figure 3. Weight percentjles curve. Decrease in weight at 4 erance from the fourth day. Regular surveillance by a months of age and subsequent recovery afuer surgery performed at 7 months of age. pediatrician has shown excellent weight improvement (Fig. 3). Discussion Intestjnal obstructjon is a common neonatal surgical emergency. 5 The annular pancreas is a rare malforma - tjon, accountjng for approximately 1% of the causes of bowel obstructjon in newborns. 6 The pancreas develops between the sixth and eighth week of gestatjon. Its fjnal locatjon and shape result from the fusion of two evaginatjons, the dorsal and ventral, of the primitjve duodenum. During this process of embryogenesis, the ventral evaginatjon, located to the right of the duodenum, follows a rotatjonal move - ment posterior to the second portjon of the duodenum and merges with the dorsal evaginatjon. The annular pancreas results from an error in the rotatjon of ventral Figure 1. Plain abdominal X-ray showing the “double bubble” evaginatjon in this process of morphogenesis, result - sign due to the dilatatjon of the stomach and the fjrst portjon of the duodenum (arrows); distal air suggests a partjal duodenal ing in the circular involvement of the second portjon obstructjon. of the duodenum by pancreatjc tjssue. 7 This ring may be complete or incomplete (in 25% and 75% of cases, respectjvely). 6,8 There are several theories proposed to explain the origin of the annular pancreas but there is no clear explanatjon of the mechanism underlying this malformatjon. 9 The prenatal diagnosis of this malformatjon is extremely rare, but it can be performed from the second trimester † of pregnancy through the identjfjcatjon of two charac - * teristjc fjndings on the obstetric ultrasound: the “double bubble” sign and the presence of hyperechogenic bands around the duodenum. 6 The age of the clinical presentatjon of annular pancreas depends on the degree of constrictjon that confers on the duodenum and on the coexistence of other mal - formatjons. 10 The degree of constrictjon is variable and there is ofuen an associated duodenum atresia. 2,3 Figure 2. Upper gastrointestjnal contrast study (posteroanterior In more than two thirds of cases, the diagnosis of view) showing the marked distension of the stomach (*) and the intestjnal obstructjon occurs in the neonatal period, fjrst duodenal portjon (†), with the fjliform progression of the con - trast to the second portjon of the duodenum (not clearly visible with patjents presentjng non-biliary vomitjng, localized in this image). 123 Portuguese Journal of Pediatrics