First Report of Treatment with Pancreas-Sparing Duodenectomy in a Child with Primary Intestinal Lymphangiectasia.

Pancreas-sparing duodenectomy (PSD) is a known surgical technique used in patients with duodenal pathologies in the adult age group. We present a 3-year-old female patient with intestinal lymphangiectasia who underwent PSD. This is the first case in which this surgical technique was used in childhood. We believe that PSD can be used in the pediatric age group for benign pathologies. Introducing a stent to the common bile duct and the main pancreatic duct is not a requirement, especially if the ampulla is preserved as a "button" duodenal patch. Copyright:

INTRODUCTION

Primary intestinal lymphangiectasia (PIL) is a rare disorder, characterized by dilated intestinal lacteals resulting in lymph leakage into the small-bowel lumen, leading to protein-losing enteropathy. In rare cases in which the intestines are affected locally, resection of the diseased segment may provide relief.[1]

Pancreas-sparing duodenectomy (PSD) is a known surgical technique used in adult patients with benign pathologies of the duodenum like duodenal lymphangiectasia.[23] We present a 3-year-old female patient with intestinal lymphangiectasia who underwent PSD.

CASE REPORT

A 3-year-old female who had been followed with the diagnosis of PIL for 2 years was consulted to our department regarding the necessary surgical intervention for her anasarca-type edema, massive ascites, and refractory hypoalbuminemia. Her immune system was depressed, leading to frequent and severe infections. The diagnosis was made by the upper gastrointestinal system endoscopic examination that revealed duodenal lymphangiectasia [Figure 1]. She did not respond to dietary or medical (octreotide: 15–20 μg/kg, twice a day, subcutaneously) treatment. Partial intestinal resection had been performed twice before, leading to transient decreases in symptom severity. Two months after the last partial intestinal resection, the patient was hospitalized due to edema and hypoalbuminemia at first only once a month, then twice a month, and finally weekly. In addition, her immune system was depressed due to protein loss, which was leading to frequent and severe infections that jeopardized her life. Her brother, who also had PIL, had improved after partial intestinal resection and needed albumin supplement only once for 3.5 years. Our patient's last operation was done 7 months before which the affected small intestine segment of 50 cm was resected. Hence, we concluded that no pathology left in small intestines, and the only pathology remaining was at the duodenum. That's why, as a last opportunity for the treatment, excision of the duodenum was decided to decrease the lymphangiectatic load. As partial intestinal resection improved her brother's status significantly, the family agreed on duodenectomy.

Figure 1

Upper gastrointestinal system endoscopic examination of the patients; findings of duodenal lymphangiectasia

A right transverse laparotomy was performed. After mobilizing the hepatic flexure of the colon, the duodenum was mobilized extensively by Kocher's maneuver. The vessels from the anterior and posterior arcades of the superior and inferior pancreaticoduodenal arteries supplying the duodenum were individually ligated close to the bowel wall and divided. The duodenum was isolated from the pancreas and the retroperitoneum except for the ampullary region. The proximal duodenum was transected 1 cm distal to the pylorus. The duodenum and proximal jejunal segment, including the previous anastomosis region (20 cm jejunal segment), were isolated and excised. During excision, the ampulla with the surrounding mucosal patch of 1 cm in diameter was excised from the duodenum. The integrity of the bile duct was checked by inserting a catheter and administering fluid. The graft of the jejunal segment was mobilized by preserving the arcades and placed in the bed of the duodenum [Figure 2]. An end-to-end anastomosis between the jejunum and duodenal cuff was performed using a single layer of interrupted absorbable polyfilament 3/0 sutures [Figure 3]. A transmural incision (1 cm) was made at the optimal site on the jejunal wall facing the ampulla patch (5 cm distal to the pylorojejunostomy). The ampulla and its surrounding mucosal patch were anastomosed end-to-side to the jejunal segment by absorbable polyfilament 5/0 interrupted sutures [Figures 4 and 5]. No stent was left in either the bile duct or pancreatic duct. The postoperative follow-up was uneventful. Radiopaque examination of the upper gastrointestinal system that was done on the 6th postoperative day has shown normal passage without leakage. The patient was discharged without any complication. During the follow-up, she was hospitalized on the 40th postoperative day and thereafter, every week secondary to edema, ascites, tetany, central line infections, and thrombosis. She had hypocalcemia, hypoalbuminemia, hypophosphatemia, hypomagnesemia, deficiency of fat-soluble vitamins, and zinc that could not be corrected by oral replacement therapy. Despite parenteral and enteral nutrition support, she continued to have growth failure and malnutrition that could be explained by the widespread intestinal involvement of the primary disease. Two years after the operation, the patient died due to diarrhea, leading to electrolyte imbalance and accompanying pulmonary infection.

Figure 2

Schematic drawing showing the placement of the jejunum segment (neo-duodenum). The mesentery of the jejunum that was placed medially was not shown

Figure 3

The beginning of the anastomosis of the duodenal cuff (1 cm) and jejunal segment. The pylorus is seen (white arrow)

Figure 4

Schematic drawing showing the end-to-side anastomosis between the ampulla with duodenal patch and the incision made on the medial wall of the neoduodenum (jejunum). The ampulla with the duodenal patch is anastomosed to the incision made on the medial wall of the jejunum (neoduodenum)

Figure 5

Posterior view of the neoduodenum. The ampulla-jejunal anastomosis is observed (white arrow)

DISCUSSION

Previously, pancreaticoduodenectomy (PD) was used for all duodenal and ampullary pathologies, before Chung et al. defined the PSD in 1995.[3] As PSD preserves pancreatic tissue, it requires only two instead of three anastomoses and has a shorter operative time and it is preferred to PD.

Different operation techniques have been described as PSD. Most of the authors described anastomosis of the jejunum segment to stomach end-to-end like Billroth I,[34567] whereas others described anastomosis of the distal jejunum segment to stomach side-to-end after placing the jejunum to the duodenum bed-like Billroth II and called as Roux-en-Y reconstruction method.[7] We used the first technique which will be nominated as PSD at the rest of the text.

PSD was classified into four types: pancreas-sparing total duodenectomy (complete resection of the duodenum, including pylorus), pancreas-sparing subtotal duodenectomy (preserving the pylorus and duodenal bulb), pancreas-sparing proximal duodenectomy (resection of the proximal duodenum), and pancreas-sparing distal duodenectomy (resection of the distal duodenum).[4] Most authors define PSD as preserving the pylorus and duodenal bulb, as we did in our case.[3456]

Another classification based on the treatment of the major papilla was defined: type I preserves the major and minor papilla as well as either the upper or lower segment of the duodenal wall; type II leaves the major papilla as a button on the pancreatic head (as done at our operation); and type III excises the intraduodenal portion of the major papilla, exposing the terminal portions of the bile duct and the pancreatic duct separately.[5]

Most of the authors introduced a stent to the common bile duct and main pancreatic duct, but they also indicated that the introduction of a stent is not essential.[356] We did not leave any stent and we did not encounter any complication. However, when the ampulla is devoid of the duodenal patch, stenting is necessary to define the walls of both ducts and prevent obstruction.

Chung et al. defined intrajejunal ductal anastomosis by access enterotomy made opposite to the proposed site for the implantation.[3] Some authors defined anastomosis of the ampulla to the 1 cm transmural incision made at the jejunum facing the isolated ampulla, without additional enterotomy.[6] Most of the authors used interrupted one-layer pancreaticojejunotomy anastomosis,[6] whereas some authors defined two-layer pancreaticojejunotomy anastomosis.[7] We think that one-layer anastomosis is sufficient and two-layer anastomosis may increase the risk of stenosis of ductal anastomosis at children.

Although placing the mesentery of the neoduodenum posteriorly was advised,[6] we placed it anteromedially to decrease tension on the mesentery. We placed the ampullo-jejunostomy posteromedially to the jejunal wall facing the isolated ampulla. The ampulla-jejunal anastomosis which is made at the posterior wall after the jejunum is rolled medially was completed easily.

After PSD, the neoduodenum can be investigated by endoscopy routinely.[3] This is an advantage of the technique, especially in patients with mucosal pathologies such as familial adenomatous polyposis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient's family has given their consent for the patient's images and other clinical information to be reported in the journal. The patient's family understand that the patient's name and initial will not be published and due efforts will be made to conceal the patient's identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.