<abstract xmlns="http://www.w3.org/1999/xhtml">

<p>Type I intestinal atresias (webs) are rare causes of gastrointestinal obstruction in infants, the most common site being the second portion of the duodenum. According to the Louw and Barnard classification, type 1 atresia has been defined as an intra-luminal web which results in either complete (web with no perforation) or incomplete (web with central perforation) intestinal obstruction. The jejunum is a rare site of such webs. Diagnosis of an incompletely obstructing web due to central perforation is usually difficult and challenging. We present two cases of jejunal web with a central perforation in which the presentation was delayed. Both were managed by excision of the web.</p></abstract>

Type I intestinal atresias (webs) are rare causes of gastrointestinal obstruction in infants, the most common site being the second portion of the duodenum. According to the Louw and Barnard classification, type 1 atresia has been defined as an intra-luminal web which results in either complete (web with no perforation) or incomplete (web with central perforation) intestinal obstruction. The jejunum is a rare site of such webs. Diagnosis of an incompletely obstructing web due to central perforation is usually difficult and challenging. We present two cases of jejunal web with a central perforation in which the presentation was delayed. Both were managed by excision of the web.


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<sec id="j_devperiodmed.20172102.9597_s_001"><div>Introduction</div>
<p>Type I intestinal atresias (webs) are rare causes of gastrointestinal (GI) obstruction in infants [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_001">1</a>]. The most common site is the second portion of the duodenum where it is often associated with trisomy 21, cardiac or renal anomalies [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_001">1</a>]. The jejunum is a rare site of such webs. A large study from Taiwan has reported only 8% of the webs arising from the jejunum [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_001">1</a>, <a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_002">2</a>]. The presenting symptoms of the fenestrated mucosal web are sub-acute intestinal obstruction with poor weight gain in children [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_001">1</a>]. Diagnosis is difficult [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_001">1</a>, <a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_002">2</a>]. We present two cases of jejunal web with central perforation and delayed presentation.</p>
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<sec id="j_devperiodmed.20172102.9597_s_002"><div>Case summary</div>
<p>Informed consent was taken from the parents of both the patients.</p>
<sec id="j_devperiodmed.20172102.9597_s_002_s_001"><div>Case 1</div>
<p>An 8-month-old male child presented with intermittent episodes of bilious vomiting after starting a semisolid diet at 5 months. The abdomen was soft with no distention or visible peristalsis. Plain erect x-ray findings were unremarkable. An upper gastrointestinal contrast study revealed a distended stomach and duodenum with persistent dilatation of the duodenum in delayed plates (<a ref-type="fig" href="#j_devperiodmed.20172102.9597_fig_001">Fig. 1</a>). Abdominal ultrasound (USG) and Contrast Enhanced Computed Tomography (CECT) revealed a distended stomach and duodenum with no evidence of malrotation.</p>
<figure id="j_devperiodmed.20172102.9597_fig_001" position="float" fig-type="figure">
<h2>Fig. 1</h2>
<figCaption><p>Delayed plate of an upper gastrointestinal contrast study showing a distended stomach and persistently dilated duodenum.</p></figCaption>
<img xmlns:xlink="http://www.w3.org/1999/xlink" xlink:href="graphic/j_devperiodmed.20172102.9597_fig_001.jpg" src="https://sciendo-parsed.s3.eu-central-1.amazonaws.com/64721c65215d2f6c89dbc591/j_devperiodmed.20172102.9597_fig_001.jpg?X-Amz-Algorithm=AWS4-HMAC-SHA256&amp;X-Amz-Date=20240626T010925Z&amp;X-Amz-SignedHeaders=host&amp;X-Amz-Expires=18000&amp;X-Amz-Credential=AKIA6AP2G7AKP25APDM2%2F20240626%2Feu-central-1%2Fs3%2Faws4_request&amp;X-Amz-Signature=a2f0143050fdafc5b2a7ce94b08cd955d133df9abcc7ecceb098e2d9cd37e701" class="mw-100"/>
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<p>after taking informed consent, the patient was taken up for surgery. At diagnostic laparoscopy, the stomach and proximal jejunum were distended. At laparotomy, a jejunal web located 12 cm from the duodeno-jejunal junction was found (<a ref-type="fig" href="#j_devperiodmed.20172102.9597_fig_002">Fig. 2</a>). The web was excised and distal patency was confirmed. Histopathology was consistent with jejunal mucosa.</p>
<figure id="j_devperiodmed.20172102.9597_fig_002" position="float" fig-type="figure">
<h2>Fig. 2</h2>
<figCaption><p>Intra-operative image of the first patient showing the jejunal web.</p></figCaption>
<img xmlns:xlink="http://www.w3.org/1999/xlink" xlink:href="graphic/j_devperiodmed.20172102.9597_fig_002.jpg" src="https://sciendo-parsed.s3.eu-central-1.amazonaws.com/64721c65215d2f6c89dbc591/j_devperiodmed.20172102.9597_fig_002.jpg?X-Amz-Algorithm=AWS4-HMAC-SHA256&amp;X-Amz-Date=20240626T010925Z&amp;X-Amz-SignedHeaders=host&amp;X-Amz-Expires=18000&amp;X-Amz-Credential=AKIA6AP2G7AKP25APDM2%2F20240626%2Feu-central-1%2Fs3%2Faws4_request&amp;X-Amz-Signature=ad8653c1039c6573cb550a4136da372544b2c1d56320c301755743d682cbb007" class="mw-100"/>
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<sec id="j_devperiodmed.20172102.9597_s_002_s_002"><div>Case 2</div>
<p>A 2-year-old emaciated male child presented with intermittent episodes of bilious vomiting of one year’s duration. He had also presented with failure to thrive and poor weight gain.</p>
<p>A plain erect x-ray of the abdomen showed a grossly dilated stomach and upper duodenum. An upper gastrointestinal contrast study revealed a distended stomach and duodenum with persistent dilatation of the duodenum in delayed plates. Abdominal USG and CECT revealed a dilated first and second part of the duodenum. There was no evidence of malrotation. after taking informed consent, the patient was taken up for surgery. At laparotomy, a jejunal web with central perforation was found approximately 15 cm from the duodeno-jejunal flexure (<a ref-type="fig" href="#j_devperiodmed.20172102.9597_fig_003">Fig. 3</a>). The web was excised.</p>
<figure id="j_devperiodmed.20172102.9597_fig_003" position="float" fig-type="figure">
<h2>Fig. 3</h2>
<figCaption><p>Intra-operative image of the second patient showing the jejunal web.</p></figCaption>
<img xmlns:xlink="http://www.w3.org/1999/xlink" xlink:href="graphic/j_devperiodmed.20172102.9597_fig_003.jpg" src="https://sciendo-parsed.s3.eu-central-1.amazonaws.com/64721c65215d2f6c89dbc591/j_devperiodmed.20172102.9597_fig_003.jpg?X-Amz-Algorithm=AWS4-HMAC-SHA256&amp;X-Amz-Date=20240626T010925Z&amp;X-Amz-SignedHeaders=host&amp;X-Amz-Expires=18000&amp;X-Amz-Credential=AKIA6AP2G7AKP25APDM2%2F20240626%2Feu-central-1%2Fs3%2Faws4_request&amp;X-Amz-Signature=aff710173ef9bce8a094e393947094d2b8b976936a89bc97e2b50a76f0c19830" class="mw-100"/>
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<sec id="j_devperiodmed.20172102.9597_s_003"><div>Discussion</div>
<p>Jejuno-ileal atresias have been classified as type I (mucosal web), type II (atretic fibrous cord), type IIIa (V-shaped mesenteric defect), type IIIb (apple peel atresia), and type IV (multiple atresias) [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_003">3</a>]. Type 1 atresia, according to the Louw and Barnard classification [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_001">1</a>, <a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_004">4</a>] has been defined as an intra-luminal web which results in either complete (web with no perforation) or incomplete (web with central perforation) intestinal obstruction [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_001">1</a>]. The jejunum is a rare site of such a web. In a jejunal web with central perforation, the clinical presentation is variable and the diagnosis often difficult and delayed [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_005">5</a>].</p>
<p>The exact etiology of intestinal atresias is unknown. Louw and Barnard proposed vascular accidents as a cause, especially ones associated with gastroschisis [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_004">4</a>, <a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_006">6</a>]. However, this theory fails to explain vascular events in otherwise normal children [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_006">6</a>].</p>
<p>Other factors like vasoconstricting medications, toxins and cystic fibrosis have also been related to jejuno-ileal atresias [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_006">6</a>]. Recently, it has been suggested that disruption in endodermal development results in intestinal atresias [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_006">6</a>]. Mutations in the endodermal Fgfr2IIIb gene or its encoding ligand Fgf10 have been found to result in both colonic and duodenal atresias [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_006">6</a>]. Disruption of Hedgehog signaling proteins produced in the fetal endoderm or mutations of a gene encoding Cdx-2, an endodermal transcription factor, may also result in intestinal atresia [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_006">6</a>].</p>
<p>The extent of clinical symptoms is proportionate to the diameter of the perforation in the web [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_005">5</a>]. Presentation is usually delayed with features of intermittent vomiting, failure to thrive, poor weight gain and emaciation [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_001">1</a>, <a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_005">5</a>, <a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_006">6</a>]. A plain erect X-ray shows a hugely dilated stomach and proximal bowel. Contrast studies reveal a persistently dilated stomach and proximal bowel with delayed transit of the contrast in delayed films.</p>
<p>Review of the literature suggests few reports and studies on jejunal atresias. Andrews and Stem, De Backer et al, Kothari et al, Seltz and Baba et al [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_005">5</a>] have reported jejunal webs in their studies which were managed by laparotomy and excision [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_005">5</a>]. Rudolph et al [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_006">6</a>] have reported jejunal web to be associated with intestinal hyperproliferation [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_006">6</a>]. Recently, Tang [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_001">1</a>] has reported a jejunal web causing diagnostic confusion as intestinal malrotation in a neonate [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_001">1</a>]. The clinical presentation of sub-acute obstruction coupled with radiology often falls short of the correct diagnosis in atresias with central perforation.</p>
<p>The management of jejunal web is surgical excision, even though variable approaches may be used [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_005">5</a>]. Endoscopic laser therapy, as well as simultaneous laparotomy with endoscopy has also been reported [<a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_005">5</a>, <a ref-type="bibr" href="#j_devperiodmed.20172102.9597_ref_007">7</a>].</p>
<p>Though rare, jejunal web with perforation should be considered as a differential in patients presenting with longstanding sub-acute intestinal obstruction in childhood.</p>
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</div>

Delayed presentation of jejunal atresia

Journal of Mother and Child

This work is licensed under the Creative Commons Attribution 4.0 International License.

{"article-title":"Delayed presentation of jejunal atresia"}

Type I intestinal atresias (webs) are rare causes of gastrointestinal obstruction in infants, the most common site being the second portion of the duodenum....

Delayed presentation of jejunal atresia

Data publikacji: 11 sie 2017

Zakres stron: 95 - 97

Otrzymano: 21 mar 2017

Przyjęty: 05 kwi 2017

DOI: https://doi.org/10.34763/devperiodmed.20172102.9597

© 2017 Charu Sharma, Hemanshi Shah, Mukta Waghmare, Jayesh Desale, Pankaj Dwivedi, published by Sciendo

This work is licensed under the Creative Commons Attribution 4.0 International License.

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