Neonatal duodenal obstruction due to a preduodenal portal vein associated with intestinal malrotation: A case report

Neonatal duodenal obstruction due to a preduodenal portal vein associated with intestinal malrotation: A case report

PEPO-543; No. of Pages 4 pediatria polska xxx (2017) xxx–xxx Available online at www.sciencedirect.com ScienceDirect journal homepage: www.elsevier...

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PEPO-543; No. of Pages 4 pediatria polska xxx (2017) xxx–xxx

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Case report/ Kazuistyka

Neonatal duodenal obstruction due to a preduodenal portal vein associated with intestinal malrotation: A case report Hechmi Ben Hamouda 1,*, Radhia Hadj Salem 1, Hatem Rabeh 1, Ilhem Ben Amor 1, Samia Belhassen 2, Hahib Soua 1, Abdellatif Nouri 2, Khaled Ben Rhouma 3, Hassine Hamza 3, Bahri Mahjoub 1, Mohamed Tahar Sfar 1 1

Department of Pediatrics and Neonatology, Tahar Sfar University Hospital, Mahdia, Tunisia Department of Pediatric Surgery, Fattouma Bourguiba University Hospital, Monastir, Tunisia 3 Department of Radiology, Tahar Sfar University Hospital, Mahdia, Tunisia 2

article info

abstract

Article history:

Preduodenal portal vein (PDPV) is a rare congenital vascular anomaly in which the portal

Received: 12.03.2017

vein passes unusually anterior to the duodenum rather than posteriorly. Generally

Accepted: 29.03.2017

asymptomatic, PDPV may rarely cause duodenal obstruction in newborn. We report a full

Available online: xxx

term 16-day-old girl who presented with recurrent non-bilious vomiting soon after birth. Clinically, she was dehydrated and the abdomen was not distended. Abdominal radio-

Keywords:  Congenital vascular anomaly  Maldevelopment of the portal vein  Gastrointestinal obstruction

graphy showed a dilated stomach and the first part of duodenum with some gas in the distal intestines. Abdominal ultrasound revealed distended stomach, dilatation of proximal duodenum and intestinal malrotation. Upper gastrointestinal contrast study showed severe gastroesophageal reflux with delayed passage of contrast to the small bowel, distended stomach and proximal duodenum confirming partial duodenal obstruction. Surgical exploration revealed a portal vein crossing over the duodenum anteriorly and intestinal malrotation. A duodenoduodenostomy was performed and Ladd's procedure was added to correct the intestinal malrotation. The post-operative course was uneventful and 6 months after the operation the patient is in good condition with normal development and no intestinal problems. © 2017 Polish Pediatric Society. Published by Elsevier Sp. z o.o. All rights reserved.

* Corresponding author at: Department of Pediatrics and Neonatology, Tahar Sfar University Hospital, 5111 Mahdia, Tunisia. Tel.: +00 216 98 688 475; fax: +00 216 73 671 579. E-mail address: [email protected] (H. Ben Hamouda). http://dx.doi.org/10.1016/j.pepo.2017.03.024 0031-3939/© 2017 Polish Pediatric Society. Published by Elsevier Sp. z o.o. All rights reserved.

Please cite this article in press as: Ben Hamouda H, et al. Neonatal duodenal obstruction due to a preduodenal portal vein associated with intestinal malrotation: A case report. Pediatr Pol. (2017), http://dx.doi.org/10.1016/j.pepo.2017.03.024

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Introduction Preduodenal portal vein (PDPV) is a rare congenital vascular anomaly in which the portal vein passes unusually anterior to the duodenum rather than posteriorly. This anomaly results from the embryonic maldevelopment of the portal venous system [1]. PDPV is generally asymptomatic, but in newborns and infants, it is usually associated with features of intestinal obstruction either due to compression of duodenum by the abnormal portal vein itself or by the coexisting congenital anomalies [2, 3]. PDPV is extremely rare in adults and is often found incidentally during laparotomy investigation for an unrelated condition [4]. There are few case reports in the literature about PDPV in newborns [2, 3, 5]. We report a neonate with partial duodenal obstruction who had a PDPV associated with intestinal malrotation diagnosed intraoperatively and we discuss diagnostic problems and the management about this anomaly with a review of literature.

Case report A 16-day-old girl presented with repeated non-bilious vomiting soon after birth. There was no significant antenatal history except gestational diabetes. She was born at 39 weeks of gestation by normal vaginal delivery with a birth weight of 3500 g. Clinically on admission, the neonate was dehydrated and the abdomen was not distended. She lost 650 g of his body weight. Biochemical results showed hyponatremia of 124 mmol/l, hypokalemia of 2.9 mmol/l, hypochloremia of 60 mmol/l, metabolic alkalosis pH of 7.6 and normal renal function. Abdominal radiography showed a double bubble sign, a dilated stomach and the first part of duodenum with some gas in the distal intestines signifying a partial obstruction of duodenum (Fig. 1). Abdominal ultrasound revealed distended both stomach and first part of duodenum with a normal pylorus. Besides, it showed a superior mesenteric vein located on the left side of the abdomen which may be related to intestinal malrotation. Upper gastrointestinal contrast study showed severe gastroesophageal reflux with delayed passage of contrast to the small bowel, dilated stomach and the first part of duodenum confirming partial duodenal obstruction (Fig. 2). The evolution after intravenous rehydration and electrolyte replacement is characterized by improving hydration status with good weight gain and correction of biological anomalies, but facing the persistence of early vomiting milk after each suckled, the patient was operated on 36 days of age. Surgical exploration revealed a vascular structure crossing over the duodenum which was distended cranially and narrowed distally. This vascular structure was confirmed to be a portal vein (Fig. 3). There was also intestinal malrotation. Subsequently, side-to-side duodenoduodenostomy was performed and Ladd's procedure was added to correct the intestinal malrotation. The postoperative course was uneventful and 6 months after operation the patient is in good condition with normal development and no intestinal problems.

Fig. 1 – Abdominal radiograph showing dilated stomach and the first part of the duodenum with some gas in the distal intestines

Fig. 2 – Upper gastrointestinal contrast study showed severe gastroesophageal reflux with delayed passage of contrast to the small bowel, dilated stomach and the first part of duodenum

Please cite this article in press as: Ben Hamouda H, et al. Neonatal duodenal obstruction due to a preduodenal portal vein associated with intestinal malrotation: A case report. Pediatr Pol. (2017), http://dx.doi.org/10.1016/j.pepo.2017.03.024

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Fig. 3 – Operative picture showing PDPV (arrow), distended stomach and the first part of duodenum

Discussion Since the first description of PDPV in 1921 by Knight [6], less than 100 cases have been reported in the literature [2, 5]. The majority of these cases are newborns and infants presenting with duodenal obstruction. PDPV was directly responsible for the duodenal obstruction in less than half of these cases [2, 3, 5, 7, 8]. The importance of this condition lies in the fact that the operations on the biliary tract and duodenum are fraught with danger of damage to the portal vein and the disastrous consequences due to its anterior placement [6]. PDPV results from the embryonic maldevelopment of the portal venous system. The development of this complex anomaly takes place between the 5th and 10th embryonal weeks. During this embryonic period, the portal vein is formed from the vitelline veins during liver development. The two parallel vitelline veins are joined by three interconnecting veins: the cephalad branch within the liver, the middle branch posterior to duodenum, and the caudal branch anterior to duodenum. During normal development, the middle branch, the caudal part of the left vitelline vein and the proximal portion of the right vitelline vein persist forming an S-shaped portal vein passing behind the duodenum. But if the caudal branch persists with the entire right vitelline vein, an L-shaped portal vein was formed what is known as the PDPV [1, 2]. Clinically, PDPV is generally asymptomatic, but in newborns and infants, this anomaly is usually associated with clinical and radiologic features of upper gastro-intestinal tract obstruction [8]. Approximately, 50% of cases of PDPV are asymptomatic discovered incidentally during surgery. This incidental finding should alert the surgeon to be familiar to deal with it to prevent unnecessary trauma during cholecystectomy, gastrectomy and pancreatectomy with serious outcomes [7]. PDPV has a high potential to

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cause preduodenal obstruction and the metabolic abnormalities observed in the present case were reminiscent of pyloric stenosis because the preampullary obstruction leads to nonbilious vomiting of gastric secretions. Symptomatic duodenal obstruction occurs in approximately 50% of patients with PDPV, caused by itself or coexisting anomalies such as duodenal web, duodenal atresia, intestinal malrotation, and annular pancreas [2, 3, 5]. PDPV can be associated with other anomalies such as situs inversus, cardiac anomalies, polysplenia, asplenia, biliary atresia, preduodenal common bile duct and jejunal atresia [1, 3, 5, 9, 10]. The association of biliary atresia with intestinal malrotation, preduodenal portal vein, and other structural abnormalities has been previously reported and is estimated to occur in 5–15% of cases [12]. As in our case, intestinal malrotation is frequently seen in PDPV patients, suggesting that there is a relationship between PDPV and intestinal malformation [1]. Prenatal diagnosis or preoperative identification of PDPV as a cause of duodenal obstruction is rarely made even in the cases of duodenal obstruction caused by the PDPV [7, 10, 11]. Our case has not been diagnosed either prenatally or preoperatively and was incidentally found during operation. However, if this possibility is suspected preoperatively, it can be outlined by noninvasive imaging using ultrasonography, computed tomography or magnetic resonance imaging [3]. Abdominal radiography may reveal distension of the stomach and proximal duodenum and a change in calibre of the small bowel distal to the level of partial obstruction, as was evident in our patient. These findings can be confirmed on contrast studies. Abdominal ultrasound can show a normal pylorus and a pre-pancreatic course of the portal vein on transverse or sagittal imaging. Similar findings may be found on computed tomography or magnetic resonance imaging [8, 13]. The treatment for duodenal obstruction cause by PDPV is bypass surgery. Duodenoduodenostomy or gastroduodenostomy that anteriorly bypasses the portal vein is the preferred method with good clinical outcomes [5, 7, 10]. In cases where PDPV is not causing obstruction and the obstruction is caused by an intraluminal web, excision of web and duodenoplasty is the treatment of choice [9]. Our patient showed an uneventful course after the duodenoduodenostomy performed with Ladd's procedure added to correct the intestinal malrotation. We determined that appropriate and timely surgical management of PDPV in infants led to good early clinical outcomes without complications. We conclude that the presence of PDPV should be considered in all newborns and infants presenting with duodenal obstruction, especially if associated with intestinal malrotation. The diagnosis is suspected if neonates present with clinical and radiologic features of upper gastro-intestinal tract obstruction. Even though PDVP apparently is the primary cause of duodenal obstruction in only a few of the reported cases, its mere presence always should be borne in mind to avoid inadvertent damage during surgery.

Authors’ contributions/Wkład autorów According to order.

Please cite this article in press as: Ben Hamouda H, et al. Neonatal duodenal obstruction due to a preduodenal portal vein associated with intestinal malrotation: A case report. Pediatr Pol. (2017), http://dx.doi.org/10.1016/j.pepo.2017.03.024

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Conflict of interest/Konflikt interesu [4]

None declared.

Financial support/Finansowanie None declared.

[5]

[6] [7]

Ethics/Etyka [8]

The work described in this article has been carried out in accordance with The Code of Ethics of the World Medical Association (Declaration of Helsinki) for experiments involving humans; EU Directive 2010/63/EU for animal experiments; Uniform Requirements for manuscripts submitted to Biomedical journals.

r e f e r e n c e s / p i s m i e n n i c t w o

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Please cite this article in press as: Ben Hamouda H, et al. Neonatal duodenal obstruction due to a preduodenal portal vein associated with intestinal malrotation: A case report. Pediatr Pol. (2017), http://dx.doi.org/10.1016/j.pepo.2017.03.024