Annular pancreas

Annular pancreas
Classification and external resources
Specialty	medical genetics
ICD-10	Q45.1
ICD-9-CM	751.7
OMIM	167750
MedlinePlus	001142

Annular pancreas is a rare condition in which the second part of the duodenum is surrounded by a ring of pancreatic tissue continuous with the head of the pancreas. This portion of the pancreas can constrict the duodenum and block or impair the flow of food to the rest of the intestines. It is estimated to occur in 1 out of 12,000 to 15,000 newborns.^[1] The ambiguity arises from the fact that not all cases are symptomatic.^[2]

Causes

It is typically associated with abnormal embryological development, however adult cases can develop. It can result from growth of a bifid ventral pancreatic bud around the duodenum, where the parts of the bifid ventral bud fuse with the dorsal bud, forming a pancreatic ring. It can also result if the ventral pancreatic bud fails to fully rotate, so it remains on the right or if the dorsal bud rotates in the wrong direction, such that the duodenum is surrounded by pancreatic tissue. Blockage of the duodenum develops if inflammation (pancreatitis) develops in the annular pancreas.

Presentation

Early signs of abnormality include polyhydramnios (an excess of amniotic fluid), low birth weight, and feeding intolerance immediately after birth.

Diagnosis

Postnatal diagnostic procedures include abdominal x-ray and ultrasound, CT scan, and upper GI and small bowel series.

Treatment

Treatment usually is bypassing the obstructed segment of duodenum by duodeno-jejunostomy. Another approach is laparoscopic gastrojejunostomy or duodenojejunostomy.^[3]

References

↑ Lainakis N; Antypas S; Panagidis A; et al. (2005), "Annular pancreas in two consecutive siblings: an extremely rare case", European Journal of Pediatric Surgery 15 (5): 364–8, doi:10.1055/s-2005-865838, PMID 16254852
↑ Ravitch, MM. (1975). "The pancreas in infants and children". Surg Clin North Am 55 (2): 377–85. PMID 165579.
↑ De Ugarte DA, Dutson EP, Hiyama DT (2006). "Annular pancreas in the adult: management with laparoscopic gastrojejunostomy". The American surgeon 72 (1): 71–3. PMID 16494188.

Congenital malformations and deformations of digestive system (Q35–Q45, 749–751)

Upper GI tract

Tongue, mouth and pharynx	Cleft lip and palate Van der Woude syndrome tongue Ankyloglossia Macroglossia Hypoglossia

Esophagus	EA/TEF Esophageal atresia: types A, B, C, and D Tracheoesophageal fistula: types B, C, D and E esophageal rings Esophageal web (upper) Schatzki ring (lower)

Stomach	Pyloric stenosis Hiatus hernia

Lower GI tract

Intestines	Intestinal atresia Duodenal atresia Meckel's diverticulum Hirschsprung's disease Intestinal malrotation Dolichocolon Enteric duplication cyst

Rectum/anal canal	Imperforate anus Rectovestibular fistula Persistent cloaca Rectal atresia

Accessory

Pancreas	Annular pancreas Accessory pancreas Johanson–Blizzard syndrome Pancreas divisum

Bile duct	Choledochal cysts Caroli disease Biliary atresia

Liver	Alagille syndrome Polycystic liver disease

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