Delayed presentation of the duodenal web with malrotation

Aditya P Singh; Dinesh K Barolia; Vinita Chaturvedi; Gurudatt Raipuria; Chetan Sharma

doi:10.4103/mmj.mmj_272_18

CASE REPORT

Year : 2020 | Volume : 33 | Issue : 4 | Page : 1418-1420

Delayed presentation of the duodenal web with malrotation

Aditya P Singh, Dinesh K Barolia, Vinita Chaturvedi, Gurudatt Raipuria, Chetan Sharma
Department of Pediatric Surgery, SMS Medical College, Jaipur, Rajasthan, India

Date of Submission	12-Sep-2018
Date of Decision	13-Oct-2018
Date of Acceptance	14-Oct-2018
Date of Web Publication	24-Dec-2020

Correspondence Address:
Aditya P Singh
MCh, Near the Mali Hostel, Main Bali Road, Falna, Dist-Pali, Rajasthan
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/mmj.mmj_272_18

Abstract

Duodenal web presents usually during the neonatal period with frank features of upper intestinal obstruction. Duodenal webs are rare congenital anomalies that are among the causes of duodenal obstructions. However, on rare occasions, duodenal web might present during late infancy or early childhood in an unusual pattern. The association of duodenal web with malrotation is also unusual. We are presenting here a case of duodenal web in fourth part with malrotation in a 14-month-old male child. The baby had been experiencing intermittent episodes of subacute intestinal obstruction for the past 8 months.

Keywords: delayed presentation, duodenal obstruction, duodenal web, malrotation, subacute intestinal obstruction

How to cite this article:
Singh AP, Barolia DK, Chaturvedi V, Raipuria G, Sharma C. Delayed presentation of the duodenal web with malrotation. Menoufia Med J 2020;33:1418-20

How to cite this URL:
Singh AP, Barolia DK, Chaturvedi V, Raipuria G, Sharma C. Delayed presentation of the duodenal web with malrotation. Menoufia Med J [serial online] 2020 [cited 2021 Apr 19];33:1418-20. Available from: http://www.mmj.eg.net/text.asp?2020/33/4/1418/304501

Introduction

Duodenal atresia (including type 1 atresia or duodenal web) presents typically during the neonatal period with frank features of upper intestinal obstruction and typical radiological Findings. However, on rare occasions, duodenal webs can have a distinct pattern of presentation with a delayed onset during the late infancy or early childhood with various symptomology^[1],[2]. Duodenal web most commonly presents in the second part of the duodenum. We are presenting here a case of delayed presentation of fourth part duodenal web with malrotation of gut in a 14-month-old male child.

Case report

The patient gave an informed consent and the ethical Committee accepted the case report. Parents of a 14-month-old male child presented to us with complaints of intermittent episodes of the bilious and nonbilious vomiting with upper abdominal distension for the past 8 months. The child was admitted frequently to a rural hospital, where no facilities were available to investigate his disease, and he received only intravenous fluids and antibiotics. They reported that he had longstanding symptoms of intermittent vomiting and abdominal distension. Since the age of 6 months, the mother started to notice recurrent abdominal distension with on and off vomiting (approximately every 10–12 days), more notably after solid and semisolid feeds. Physical examination revealed an unwell, emaciated, and pale child. His vital signs were stable. There were no dysmorphic features. His weight was 8.5 kg. There was mild abdominal distension with no visible peristalsis. Abdomen was soft, nontender with no guarding or rigidity. There were neither palpable masses nor organomegaly. Routine blood investigations were within normal limits except low hemoglobin (8 g/dl). Erect plain radiographies showed dilated upper abdominal bowel loops. An upper gastrointestinal contrast series was performed and revealed a dilated duodenum with a fair passage of contrast into the distal duodenum, jejunum, and ileum after a 30 min duodenal hold-up. A provisional diagnosis of a duodenal obstruction was suggested. We planned for exploratory laparotomy. During laparotomy, there was malrotation of the gut with normal pancreas. The stomach and proximal duodenum (especially the second and third parts) were grossly dilated, with an apparent thickening palpated in the wall in the fourth part of the duodenum during straightening, suggesting a luminal obstruction. We performed Ladd's procedure and appendicectomy was done. A duodenotomy was made just proximal to the obstruction site, and the incision was extended beyond it vertically. Luminal obliteration by a mucosal web with a small central perforation was found. There were also food residues present. The web was completely excised. Luminal patency of the distal duodenum was checked. The duodenotomy was closed transversely using an absorbable suture (Vicryl 4/0) in a single layer. Postoperative course was uneventful. The patient was allowed oral intake on the fifth postoperative day, starting with clear fluids, which were tolerated well. He was able to take a full mixed diet by the seventh postoperative day. He was discharged in good condition on the ninth postoperative day. On regular follow-up over a 6-month period, he was tolerating a normal mixed diet with no recurrence of his symptoms. He had a reasonable weight gain of ~2.5 kg over a 6-month period [Figure 1] and [Figure 2].

Figure 1: (a and b) Upper gastrointestinal contrast series.

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Figure 2: (a) Dilated stomach (red arrow) with dilated duodenum (green arrow), malrotation (black arrow-cecum located in left upper quadrant). (b) Web in the fourth part of the duodenum.

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Discussion

Duodenal atresia and duodenal web are reported causes of intestinal obstruction in children, with an incidence that ranges from 1 in 10 000 to up to 40 000 live births^[3]. The fundamentally proposed pathological basis of duodenal atresia (including type 1 or duodenal web) is a partial or complete failure of recanalization of endodermal proliferation during the solid cord stage of intestinal development^[4]. The second part of the duodenum is the most common site of occurrence of webs (85%) as it is the last part to recanalize^[4]. Involvement of other parts of the duodenum is typically uncommon^[4]. In this reported case, the web was located in the fourth part, which is an unusual site of web's occurrence, and this finding represents a rarity. This abnormal process leaves behind a web made out of only the mucosa and the submucosa layers. The muscularis layer is absent^[3]. A duodenal web is a thin, round, partially permeable, web-like structure. The resulting barrier may be complete or less frequently may have a small orifice that forms a duodenal membrane with fenestration. The continuous peristaltic activity causes the membrane to prolapse distally, forming the windsock sign. This windsock sign is typical for the duodenal web in contrasted imaging. The symptoms and the age of onset may vary depending on the size and location of the duodenal web, and if the obstruction is total or partial. In cases where the web completely closes the passage, severe vomiting is observed and the disease is diagnosed during the neonatal phase. If, as in our case, the passage is not completely but only partially obstructed, the diagnosis may be delayed until late childhood or even young adulthood. Interestingly enough, a duodenal web has been reported to present for the first time during adulthood^[5]. The late presentation of duodenal web is rare but well-known and has been reported in many cases^{[1],[2],[6],[7]}.

Besides duodenal webs, conditions like midgut volvulus, annular pancreas, duplication cysts, superior mesenteric artery syndrome, and periduodenal portal vein are among the reasons for partial obstructions in the duodenum.

Duodenal web was associated with malrotation in our case. It was coincidental per operative finding, but while operating an isolated case of malrotation, it is mandatory to see associated duodenal web. There are some reported cases where both were seen in the same patient^[8],[9].

The clinical presentation of this disease entity includes upper abdominal distension, postprandial pain, and intermittent emesis secondary to partial or complete duodenal obstruction. However, nonspecific abdominal symptoms may also be observed. Moreover, this fenestrated membrane could be a site of impaction of food residues or foreign bodies^[4]. In fact, inability to pass an ingested foreign body in a child after a reasonable period of observation should trigger further investigations to rule out a mechanical bowel obstruction that is not previously detected^[2].

The diagnosis of a partial or total duodenal obstruction can be made based on the clinical and radiological findings. In most cases of duodenal webs, diagnosis can be established by plain radiographies. However, the classical 'double-bubble' appearance may not be seen in patients with fenestrated duodenal web^[10]. The common findings in these patients are distension in the stomach and duodenum observed in upright abdominal radiograph, air-fluid levels, and normal gas distribution in the more distal intestinal loops.

Upper gastrointestinal contrast study is the investigation of choice to reach a diagnosis, especially in older children with partial obstruction, as in our case^[2]. Furthermore, flexible endoscopy can reveal the dilatation of the duodenum and the protrusion of the web in the lumen^[10]. It is claimed that the condition may be viewed using ultrasonography when the duodenum is filled with water; however, the clinical experience of the operator plays a key role in this method.

Surgical treatment with excision of the web is the mainstay of management. Excision should be complete as it may regrow in case of incomplete excision and can lead to recurrent obstruction^[4]. Checking of distal patency during operation is mandatory to avoid missing a double duodenal web, although occurrence of the latter is exceedingly rare^[4].

Furthermore, endoscopic interventions using electrocauterization with an insulated tip have also been shown to be successful^[10]. Use? of Trans-Anastomotic Tube (TAT tube), with or without an Nasogastric Tube (NGT) tube, is also associated with reduction in duration of total parentral nutrition (TPN) use and in total hospital stay^[11]. We did not use the TAT.

Conclusion

A high index of suspicion is needed to diagnose a duodenal web when it has a delayed presentation and should be considered as a differential diagnosis of subacute intestinal obstruction during late infancy and early childhood. Especially when dealing with a case of malrotation, always see for any associated duodenal web.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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