Annular Pancreas in Neonates; Case Series and Review of Literatures

Abstract

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.

Annular pancreas: Beneath the intestinal obstruction—A case report

“…Moreover, infants with annular pancreas are prone to have other congenital anomalies like Down's syndrome, esophageal and duodenal atresia, heart abnormalities, Meckel's diverticulum, pancreas divisum, and imperforate anus [2] . A plain radiograph showing double air demonstrating duodenal obstruction that can be further evidenced by upper gastrointestinal series confirms the diagnosis of duodenal atresia; however, the etiology of it to ensure the proper treatment modality for annular pancreas is still a challenge [7] . The treatment for AP is surgical with duodenoduodenostomy being the most successful surgical option followed by division of a portion of the annular pancreas and gastrojejunostomy or duodenojejunostomy with Roux en Y loop [7] .…”

“…AP in utero presents mostly with maternal polyhydramnios due to intrinsic duodenal obstruction, whereas severe bilious vomiting presentation at a median age of 1 day after birth [ 2 , 7 , 10 ]. In neonates, more than two-thirds of AP will present with features of obstruction, that is, abdominal distention, bilious vomiting, decreased passage of meconium, and feeding intolerance [2] .…”

Mid-trimester dilated fetal bowel leading to diagnosis of interstitial duplication 46,XX,dup(8)(q21.13q21.2) associated with extensive neonatal jejuno-ileal atresia

“…Of the 27 cases of intestinal atresia, 8 (29.6%) manifested associated anomalies including 2 cases with anorectal malformation, 2 case with volvulus, one each with malrotation and meconium ileus, respectively [1] . Other rare etiologies of neonatal bowel (duodenal) obstruction include annular pancreas [2 , 3] . Small bowel atresia constitutes a congenital obstruction of the lumen of the duodenum, jejunum or ileum and is one of the most common causes of neonatal bowl obstruction occurring with an incidence of between 1.3 and 2.8 per 10,1000 live births [4] .…”

Obstrucción intestinal por páncreas anular en etapa neonatal: reporte de caso

“…El páncreas anular es una malformación congénita rara descrita por primera vez por Tiedemann en 1818 y nombrada bajo ese término por Ecker (1) . Puede manifestarse desde la etapa neonatal como obstrucción duodenal o permanecer asintomática hasta la edad adulta.…”

“…Puede manifestarse desde la etapa neonatal como obstrucción duodenal o permanecer asintomática hasta la edad adulta. La prevalencia exacta es desconocida, en la literatura se describe 1 caso por cada 20 000 recién nacidos vivos aproximadamente (1) . Se ha asociado con polihidramnios materno (2) y anomalías congénitas como atresia esofágica y duodenal, malformación anorrectal y divertículo de Meckel (3) .…”