Case Report: Robotic pylorus-preserving pancreatoduodenectomy for periampullary rhabdomyosarcoma in a 3-year-old patient

Abstract

Periampullary neoplasm is rare in pediatric patients and has constituted a strict indication for pancreatoduodenectomy (PD), which is a procedure sporadically reported in the literature among children. Robotic PD has been routinely performed for periampullary neoplasm in periampullary neoplasm, but only a few cases in pediatric patients have been reported. Here, we report the case of a 3-year-old patient with periampullary rhabdomyosarcoma treated with robotic pylorus-preserving PD and share our experience with this procedure in pediatric patients. A 3-year-old patient presented with obstructive jaundice and a mass in the pancreatic head revealed by imaging. A laparoscopic biopsy was performed. Jaundice progressed with abdominal pain and elevated alpha-amylase leading to urgent robotic exploration in which a periampullary neoplasm was revealed and pathologically diagnosed as rhabdomyosarcoma by frozen section examination. After pylorus-preserving PD, we performed a conventional jejunal loop following a child reconstruction, including an end-to-end pancreaticojejunostomy, followed by end-to-side hepaticojejunostomy and duodenojejunostomy. Delayed gastric emptying (DGE) presented with increasing drain from the nasogastric tube (NGT) a week after the surgery and improved spontaneously within 10 days. In a 13-month follow-up until the present, our case patient recovered well without potentially fatal complications, such as pancreatic fistula. Robotic PD in pediatric patients was safe and effective without intra- or postoperative complications.

Keywords: case report; child; periampullary neoplasm; rhabdomyosarcoma; robotic pancreatoduodenectomy.

Figure 1

CT and MR results before radical surgery. (A,C,E) CT scan of the abdomen and pelvis with intravenous contrast agent showing an ill-defined 2.9 cm  × 2.8 cm mass with delayed enhancement of the uncinate process with proximal dilatation. (G,H) MR imaging showing a 2.6 cm × 3.6 cm × 2.1 cm mass originating from the common bile duct (CBD), presenting an equal signal on T1WI and an equal-high signal on T2WI. The common bile duct (CBD) was plugged up completely by the mass, resulting in the dilatation of the proximal biliary system. The dilatation of the common bile duct (CBD) reached 2.2 cm in diameter, and the pancreatic duct also presented a slight dilation of 0.1 cm. (B,D,F) CT scan for reevaluation showing the enlarged mass.

Figure 2

Pathological results confirmed the diagnosis of rhabdomyosarcoma. In hematoxylin–eosin staining, the lesion was located under the epithelium and formed a neoplastic layer parallel to the epithelium with small round atypic cells with obvious heteromorphism (A), and positive markers such as desmin (B), myoD1 (C), and myogenin (D) staining confirmed the diagnosis of embryonal RMS originated in the ampulla.

Figure 3

Robotic dissection in pylorus-preserving pancreatoduodenectomy (PPPD). (A) Port set up during surgery. (B) A neoplasm resembled a cluster of grapes inside the common bile duct (CBD). (C) The duodenum was transected at 2 cm distal to the pylorus. (D) The skeletonized SMV/SMA and dissected retroperitoneum with peripancreatic soft tissue and the nerve plexuses removed. (E) After the specimen was taken out, a stump of the common hepatic duct, pancreas, and duodenum. (F) A 3F ureteral stent tube was inserted into the MPD as an internal stent and fixed to the MPD with 4-0 Prolene.

Figure 4

Robotic child reconstruction and specimens. (A) End-to-end pancreatojejunostomy. (B) End-to-side single layer barbed suture hepaticojejunostomy. (C) The distal and free portion of omentum was fixed with 5–0 Prolene suture around the pancreatojejunostomy anastomosis as a mattress. (D) End-to-side single-layer barbed suture duodenojejunostomy. (E) Specimens removed with the Endo Bag.