|Year : 2017 | Volume
| Issue : 2 | Page : 87-88
Annular pancreas causing duodenal obstruction in two consecutive sisters
Ruzaimie Noor, Zafran Mohamad, S Farhan, Tarmizi Nor
Department of Surgery, Paediatric Surgical Unit, Hospital Raja Perempuan Zainab II, Kota Bharu, Kelantan, Malaysia
|Date of Web Publication||26-Jul-2017|
Department of Surgery, Paediatric Surgical Unit, Hospital Raja Perempuan Zainab II, Kota Bharu 15586, Kelantan
Source of Support: None, Conflict of Interest: None
Congenital duodenal obstruction is a common cause of neonatal intestinal obstructions. The occurrence of duodenal obstructions in family is extremely rare. We operated on two consecutive sisters presented with symptoms of duodenal obstruction. In both of our patients, annular pancreas was identified as a cause of duodenal obstruction. Only less than twenty cases have been published in English literatures. To the author's knowledge, we are the first to report the cases of familial annular pancreas and duodenal stenosis in Malaysia.
Keywords: Annular pancreas, duodenal obstruction, familial intestinal atresia
|How to cite this article:|
Noor R, Mohamad Z, Farhan S, Nor T. Annular pancreas causing duodenal obstruction in two consecutive sisters. Saudi Surg J 2017;5:87-8
| Introduction|| |
Congenital duodenal obstruction is a common cause of neonatal obstruction and its occurrence in a family is extremely rare. Several genetic components have been identified, specifically the genes which are related to the expression of annular pancreas. The spectrums of small bowel atresia also have been observed to occur in family such as familial apple peel small bowel atresia and Feingold syndrome. We reported two consecutive siblings with congenital duodenal obstruction secondary to annular pancreas.
| Case Reports|| |
This girl was delivered at 32 weeks gestation to a para 2 mother with antenatal history of polyhydramnios. The parent was young, healthy, and nonconsanguineous. The child was nonsyndromic. Her birth weight was 1300 g. It was an accidental finding of double-bubble appearance on abdominal X-ray following umbilical catheterization. There was no evidence of prominent double-bubble appearance made during antenatal scan. Upper contrast study showed dilated stomach and duodenum with malrotation. This patient underwent surgery at day 3 of life. It was right upper transverse incision. Operative finding was duodenal stenosis with annular pancreas involving segment of the second part until third part of the duodenum and it was associated with malrotation. Side-to-side duodenojejunostomy anastomosis was performed using interrupted 5/0 Vicryl suture and completed with Ladd's procedure. The dilated proximal duodenum was not tapered or plicated. The patient was supported with parenteral nutrition after the surgery and enteral feeding started on day 11 after the surgery due to prolonged ileus. Full enteral bolus feeding was established on day 30 of life. She was discharged on day 60 of life after recovered from severe nosocomial chest infection.
Our second patient, who was the sister of Case 1, was born 2 years later. She was born at term with the birth weight of 2900 g and she was nonsyndromic looking. She presented at our center at day 12 of life due to persistent projectile nonbilious vomiting after feeding since birth. She passed motion every day and the abdomen was not distended. Upper contrast study of this patient showed dilated stomach and partial obstruction at the second part of the duodenum. She underwent surgery at day 15 of life. Surgical finding was an annular pancreas with duodenal stenosis at the second part of the duodenum without malrotation. Side-to-side duodenoduodenostomy anastomosis was performed using interrupted Vicryl 5/0. Enteral feeding was started on day 7 postsurgery. The patient was discharged on day 10 postsurgery after establishing full enteral feeding. The parenteral nutrition was not started. They are both well and asymptomatic after surgery and on yearly follow-up in our clinic for the past 10 years.
| Discussion|| |
Congenital duodenal obstruction is a common cause of intestinal obstruction in neonate. It occurs in 1/5000–10,000 live births and boys are affected more than girls. More than half of the affected patients have associated congenital anomalies such as malrotation and tracheoesophageal fistula. Thirty percent of congenital duodenal obstruction patients are trisomy 21. The accepted hypothesis of duodenal atresia is due to failure of recanalization of the duodenum. In general, congenital duodenal obstruction can be divided into intrinsic and extrinsic components or both. The extrinsic components contributing to duodenal obstructions are commonly due to defects in the development of adjacent structures such as a preduodenal portal vein, the pancreas, or secondary to malrotation. One-third of duodenal atresia or stenosis cases are associated with annular pancreas. The defect is due to fusion between ventral buds that failed to rotate with dorsal pancreatic primordium resulting in partial or complete obstruction of the duodenum.
There were only few published reports about the occurrence of annular pancreas and duodenal stenosis or atresia in family members.,,, As of this time, only less than ten cases of familial annular pancreas have been published in English literature. Berant et al. in 1970 were the first to report the occurrence of familial congenital duodenal obstruction in three consecutive siblings. In their report, all the three siblings were genetically nonsyndromic child. A genetic study by Ellen found the involvement of microduplication on chromosome 6q24.2 encompassing a single gene, named utrophin contributes to annular pancreas.
The current progress in modern medical imaging practice has shown a great impact in detecting congenital cases antenatally. The presence of polyhydramnios in second to third semester will bring the high suspicious of related congenital conditions in utero. The typical double-bubble appearance or dilated stomach is almost a mainstay finding in diagnosing duodenal obstruction antenatally. The diagnosis of duodenal obstruction is confirmed by air contrast abdominal radiograph showing a dilated gas-filled stomach and proximal duodenum. Intestinal gas beyond the duodenum indicates an incomplete obstruction or bile duct anomalies. Once the child is stabilized, a surgical procedure is performed to bypass the obstruction. We performed the duodenojejunostomy anastomosis for the first sister and duodenoduodenostomy for the second sister. The reported survival rates are above 90% of the cases and mainly the mortality is due to associated congenital anomalies.
| Conclusion|| |
Congenital annular pancreas causing duodenal obstruction can occur in family, and they may present in various presentation depending on degree of obstruction. It is emphasized for a surgeon encountering cases of suspected duodenal obstructions with positive family history to have a thought of dealing with similar inherited pathology during preoperative assessment.
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Conflicts of interest
There are no conflicts of interest.
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