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Annular pancreas

MedGen UID:
56211
Concept ID:
C0149955
Congenital Abnormality
Synonym: Pancreas, annular
SNOMED CT: Annular pancreas (40315008)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
HPO: HP:0001734
Monarch Initiative: MONDO:0008183
OMIM®: 167750
Orphanet: ORPHA675

Definition

A congenital anomaly in which the pancreas completely (or sometimes incompletely) encircles the second portion of duodenum and occasionally obstructs the more proximal duodenum. [from HPO]

Clinical features

From HPO
Annular pancreas
MedGen UID:
56211
Concept ID:
C0149955
Congenital Abnormality
A congenital anomaly in which the pancreas completely (or sometimes incompletely) encircles the second portion of duodenum and occasionally obstructs the more proximal duodenum.
Duodenal stenosis
MedGen UID:
66761
Concept ID:
C0238093
Anatomical Abnormality
The narrowing or partial blockage of a portion of the duodenum.
High intestinal obstruction
MedGen UID:
870770
Concept ID:
C4025227
Disease or Syndrome

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAnnular pancreas
Follow this link to review classifications for Annular pancreas in Orphanet.

Conditions with this feature

Annular pancreas
MedGen UID:
56211
Concept ID:
C0149955
Congenital Abnormality
A congenital anomaly in which the pancreas completely (or sometimes incompletely) encircles the second portion of duodenum and occasionally obstructs the more proximal duodenum.
11q partial monosomy syndrome
MedGen UID:
162878
Concept ID:
C0795841
Disease or Syndrome
Jacobsen syndrome (JBS) is a contiguous gene deletion syndrome with major clinical features of growth retardation, psychomotor retardation, trigonocephaly, divergent intermittent strabismus, epicanthus, telecanthus, broad nasal bridge, short nose with anteverted nostrils, carp-shaped upper lip, retrognathia, low-set dysmorphic ears, bilateral camptodactyly, hammertoes, and isoimmune thrombocytopenia (Fryns et al., 1986, Epstein, 1986).
Martinez-Frias syndrome
MedGen UID:
318628
Concept ID:
C1832443
Disease or Syndrome
The Martinez-Frias syndrome is characterized by pancreatic hypoplasia, intestinal atresia, and gallbladder aplasia or hypoplasia, with or without tracheoesophageal fistula. There is considerable phenotypic overlap between Martinez-Frias syndrome and Mitchell-Riley syndrome (MTCHRS; 615710), the latter being characterized by neonatal diabetes in addition to the features of the Martinez-Frias syndrome, but without tracheoesophageal fistula (Smith et al., 2010).
Hypoplastic pancreas-intestinal atresia-hypoplastic gallbalder syndrome
MedGen UID:
411637
Concept ID:
C2748662
Disease or Syndrome
Mitchell-Riley syndrome is characterized by neonatal diabetes, pancreatic hypoplasia, intestinal atresia, and gallbladder aplasia or hypoplasia. There is considerable phenotypic overlap between Mitchell-Riley syndrome and Martinez-Frias syndrome (601346), the latter being characterized by the features of the Mitchell-Riley syndrome except for neonatal diabetes, and including tracheoesophageal fistula in some patients (Smith et al., 2010).
Alveolar capillary dysplasia with pulmonary venous misalignment
MedGen UID:
755478
Concept ID:
C2960310
Congenital Abnormality
Congenital alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) is characterized histologically by failure of formation and ingrowth of alveolar capillaries that then do not make contact with alveolar epithelium, medial muscular thickening of small pulmonary arterioles with muscularization of the intraacinar arterioles, thickened alveolar walls, and anomalously situated pulmonary veins running alongside pulmonary arterioles and sharing the same adventitial sheath. Less common features include a reduced number of alveoli and a patchy distribution of the histopathologic changes. The disorder is associated with persistent pulmonary hypertension of the neonate and shows varying degrees of lability and severity (Boggs et al., 1994). Affected infants present with respiratory distress resulting from pulmonary hypertension in the early postnatal period, and the disease is uniformly fatal within the newborn period (Vassal et al., 1998). Additional features of ACDMPV include multiple congenital anomalies affecting the cardiovascular, gastrointestinal, genitourinary, and musculoskeletal systems, as well as disruption of the normal right-left asymmetry of intrathoracic or intraabdominal organs (Sen et al., 2004).
Fanconi anemia complementation group D2
MedGen UID:
463627
Concept ID:
C3160738
Disease or Syndrome
Fanconi anemia (FA) is characterized by physical abnormalities, bone marrow failure, and increased risk for malignancy. Physical abnormalities, present in approximately 75% of affected individuals, include one or more of the following: short stature, abnormal skin pigmentation, skeletal malformations of the upper and/or lower limbs, microcephaly, and ophthalmic and genitourinary tract anomalies. Progressive bone marrow failure with pancytopenia typically presents in the first decade, often initially with thrombocytopenia or leukopenia. The incidence of acute myeloid leukemia is 13% by age 50 years. Solid tumors – particularly of the head and neck, skin, and genitourinary tract – are more common in individuals with FA.
Neurodevelopmental disorder with or without anomalies of the brain, eye, or heart
MedGen UID:
934739
Concept ID:
C4310772
Disease or Syndrome
RERE-related disorders are characterized by neurodevelopmental problems with or without structural anomalies of the eyes, heart, kidneys, and genitourinary tract and mild sensorineural hearing loss. Hypotonia and feeding problems are common among affected individuals. Developmental delay and intellectual disability range from mild to profound. Behavior problems may include attention-deficit/hyperactivity disorder, self-injurious behavior, and autism spectrum disorder. A variety of eye anomalies (coloboma, optic nerve anomalies, microphthalmia, and/or Peter's anomaly) and vision issues (myopia, anisometropia, astigmatism, exotropia, esotropia) have been reported. Congenital heart defects, most commonly septal defects, have also been described. Genitourinary abnormalities include vesicoureteral reflux, and cryptorchidism and hypospadias in males. Sensorineural hearing loss can be unilateral or bilateral.
Feingold syndrome type 1
MedGen UID:
1637716
Concept ID:
C4551774
Disease or Syndrome
Feingold syndrome 1 (referred to as FS1 in this GeneReview) is characterized by digital anomalies (shortening of the 2nd and 5th middle phalanx of the hand, clinodactyly of the 5th finger, syndactyly of toes 2-3 and/or 4-5, thumb hypoplasia), microcephaly, facial dysmorphism (short palpebral fissures and micrognathia), gastrointestinal atresias (primarily esophageal and/or duodenal), and mild-to-moderate learning disability.
Spondyloepimetaphyseal dysplasia, Krakow type
MedGen UID:
1648323
Concept ID:
C4748455
Disease or Syndrome
Krakow-type spondyloepimetaphyseal dysplasia is characterized by severe skeletal dysplasia, severe immunodeficiency, and developmental delay (Csukasi et al., 2018).
Rothmund-Thomson syndrome type 2
MedGen UID:
1684753
Concept ID:
C5203410
Disease or Syndrome
Rothmund-Thomson syndrome (RTS) is characterized by a rash that progresses to poikiloderma; sparse hair, eyelashes, and/or eyebrows; small size; skeletal and dental abnormalities; juvenile cataracts; and an increased risk for cancer, especially osteosarcoma. A variety of benign and malignant hematologic abnormalities have been reported in affected individuals. The rash of RTS typically develops between ages three and six months (occasionally as late as age two years) as erythema, swelling, and blistering on the face, subsequently spreading to the buttocks and extremities. The rash evolves over months to years into the chronic pattern of reticulated hypo- and hyperpigmentation, telangiectasias, and punctate atrophy (collectively known as poikiloderma) that persist throughout life. Hyperkeratotic lesions occur in approximately one third of individuals. Skeletal abnormalities can include radial ray defects, ulnar defects, absent or hypoplastic patella, and osteopenia.

Professional guidelines

PubMed

Engwall-Gill AJ, Zhou AL, Penikis AB, Sferra SR, Jelin AC, Blakemore KJ, Kunisaki SM
J Pediatr Surg 2023 Jun;58(6):1090-1094. Epub 2023 Feb 17 doi: 10.1016/j.jpedsurg.2023.02.029. PMID: 36907770
Yin C, Tong L, Ma M, Tan X, Luo G, Fei Z, Nie D
BMC Pregnancy Childbirth 2020 Jul 3;20(1):387. doi: 10.1186/s12884-020-03078-5. PMID: 32620086Free PMC Article
Yang B, He F, He Q, Wang Z, Fang Q, Zhong W, Wang H
Eur Radiol 2019 Jun;29(6):2902-2909. Epub 2019 Jan 7 doi: 10.1007/s00330-018-5922-0. PMID: 30617479

Recent clinical studies

Etiology

Zhou Y, Li X
J Appl Clin Med Phys 2022 Jan;23(1):e13487. Epub 2021 Nov 29 doi: 10.1002/acm2.13487. PMID: 34845829Free PMC Article
Alkhayyat M, Bachour S, Abou Saleh M, Abureesh M, Almomani A, Simons-Linares CR, Mansoor E, Sandhu D, Bhatt A, Chahal P
J Clin Gastroenterol 2022 Feb 1;56(2):186-191. doi: 10.1097/MCG.0000000000001531. PMID: 33769394
Ravel A, Mircher C, Rebillat AS, Cieuta-Walti C, Megarbane A
Arch Pediatr 2020 Jan;27(1):53-60. Epub 2019 Nov 26 doi: 10.1016/j.arcped.2019.11.008. PMID: 31784293
Gromski MA, Lehman GA, Zyromski NJ, Watkins JL, El Hajj II, Tan D, McHenry L, Easler JJ, Tirkes T, Sherman S, Fogel EL
Gastrointest Endosc 2019 Feb;89(2):322-328. Epub 2018 Sep 18 doi: 10.1016/j.gie.2018.09.008. PMID: 30240880Free PMC Article
Connelly TM, Sakala M, Tappouni R
Surg Radiol Anat 2015 Jul;37(5):431-7. Epub 2015 Jan 28 doi: 10.1007/s00276-015-1436-5. PMID: 25626884

Diagnosis

Yakoubi C, Atri S, Maghrebi H, Kacem MJ
Br J Surg 2022 Sep 9;109(10):1012. doi: 10.1093/bjs/znac167. PMID: 36084336
Ravel A, Mircher C, Rebillat AS, Cieuta-Walti C, Megarbane A
Arch Pediatr 2020 Jan;27(1):53-60. Epub 2019 Nov 26 doi: 10.1016/j.arcped.2019.11.008. PMID: 31784293
Wood CG, Lopes Vendrami C, Craig E, Mittal PK, Miller FH
Abdom Radiol (NY) 2020 May;45(5):1316-1323. doi: 10.1007/s00261-019-02197-8. PMID: 31468154
Jovani M, Lee LS
Clin Gastroenterol Hepatol 2020 Jun;18(7):A26. Epub 2019 Jan 30 doi: 10.1016/j.cgh.2019.01.036. PMID: 30710695
Oi I
Pancreas 1998 Apr;16(3):402-7. doi: 10.1097/00006676-199804000-00031. PMID: 9548686

Therapy

Parray AM, Nadkarni S, Chaudhari V, Shrikhande SV, Bhandare MS
Ann Surg Oncol 2023 Sep;30(9):5758-5760. Epub 2023 Jul 4 doi: 10.1245/s10434-023-13782-z. PMID: 37402974
Medjedovic E, Zvizdic Z, Cerovac A, Jonuzovic-Prosic S, Milisic E
Acta Medica (Hradec Kralove) 2022;65(4):149-152. doi: 10.14712/18059694.2023.6. PMID: 36942706
Pandrowala S, Parray A, Chaudhari V, Shrikhande SV, Bhandare MS
J Gastrointest Surg 2021 May;25(5):1332-1339. Epub 2021 Feb 8 doi: 10.1007/s11605-021-04927-0. PMID: 33555524Free PMC Article
Dalla Vecchia LK, Grosfeld JL, West KW, Rescorla FJ, Scherer LR, Engum SA
Arch Surg 1998 May;133(5):490-6; discussion 496-7. doi: 10.1001/archsurg.133.5.490. PMID: 9605910
Levy J
Prog Clin Biol Res 1991;373:245-56. PMID: 1838188

Prognosis

Ali Almoamin HH, Kadhem SH, Saleh AM
Afr J Paediatr Surg 2022 Apr-Jun;19(2):97-101. doi: 10.4103/ajps.AJPS_180_20. PMID: 35017379Free PMC Article
Yang B, He F, He Q, Wang Z, Fang Q, Zhong W, Wang H
Eur Radiol 2019 Jun;29(6):2902-2909. Epub 2019 Jan 7 doi: 10.1007/s00330-018-5922-0. PMID: 30617479
Kivilevitch Z, Achiron R, Perlman S, Gilboa Y
J Ultrasound Med 2017 Oct;36(10):1997-2005. Epub 2017 May 16 doi: 10.1002/jum.14233. PMID: 28509414
Levy MJ, Geenen JE
Am J Gastroenterol 2001 Sep;96(9):2540-55. doi: 10.1111/j.1572-0241.2001.04098.x. PMID: 11569674
McKenna LL
Neonatal Netw 2000 Jun;19(4):13-20. doi: 10.1891/0730-0832.19.4.13. PMID: 11949098

Clinical prediction guides

Zhang JY, Deng ZH, Gong B
J Dig Dis 2022 Nov;23(11):651-659. doi: 10.1111/1751-2980.13152. PMID: 36630003
Zhou Y, Li X
J Appl Clin Med Phys 2022 Jan;23(1):e13487. Epub 2021 Nov 29 doi: 10.1002/acm2.13487. PMID: 34845829Free PMC Article
Yang B, He F, He Q, Wang Z, Fang Q, Zhong W, Wang H
Eur Radiol 2019 Jun;29(6):2902-2909. Epub 2019 Jan 7 doi: 10.1007/s00330-018-5922-0. PMID: 30617479
Gromski MA, Lehman GA, Zyromski NJ, Watkins JL, El Hajj II, Tan D, McHenry L, Easler JJ, Tirkes T, Sherman S, Fogel EL
Gastrointest Endosc 2019 Feb;89(2):322-328. Epub 2018 Sep 18 doi: 10.1016/j.gie.2018.09.008. PMID: 30240880Free PMC Article
Kivilevitch Z, Achiron R, Perlman S, Gilboa Y
J Ultrasound Med 2017 Oct;36(10):1997-2005. Epub 2017 May 16 doi: 10.1002/jum.14233. PMID: 28509414

Recent systematic reviews

Pandrowala S, Parray A, Chaudhari V, Shrikhande SV, Bhandare MS
J Gastrointest Surg 2021 May;25(5):1332-1339. Epub 2021 Feb 8 doi: 10.1007/s11605-021-04927-0. PMID: 33555524Free PMC Article
Harnoss JM, Harnoss JC, Diener MK, Contin P, Ulrich AB, Büchler MW, Schmitz-Winnenthal FH
Pancreas 2014 Oct;43(7):981-6. doi: 10.1097/MPA.0000000000000186. PMID: 25207658Free PMC Article

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