Abstract
Background:
Small intestinal venous malformation is a rare vascular anomaly that most commonly presents with gastrointestinal bleeding. Its manifestation as acute bowel obstruction without hemorrhage is exceedingly uncommon, especially in children, posing a diagnostic dilemma.
Case description:
A previously healthy 9-year-old boy presented with acute abdominal pain and vomiting. Contrast-enhanced computed tomography revealed a homogeneously enhancing lesion with persistent enhancement on portal venous phase in the left pelvic small bowel, raising suspicion of a vascular malformation. Diagnostic laparoscopy was subsequently performed, identifying a 5 cm segment of small intestine with its serosal surface densely covered by tortuous vessels. Laparoscopic segmental resection with primary hand-sewn end-to-end anastomosis was performed. Immunohistochemistry revealed CD31 and CD34 positivity in lesional endothelial cells and GLUT-1 negativity, excluding infantile hemangioma. This immunoprofile, together with the characteristic morphology, supported the diagnosis of a venous malformation according to the International Society for the Study of Vascular Anomalies (ISSVA) classification. The patient recovered uneventfully and remained asymptomatic with no evidence of residual lesion or symptom relapse during a 6-month follow-up period.
Conclusions:
This case suggests that a small intestinal venous malformation can rarely present as acute obstruction in the absence of bleeding. Contrast-enhanced CT with persistent enhancement pattern is a useful preoperative indicator, and laparoscopy appears to be a feasible approach for both diagnosis and minimally invasive resection. This integrated diagnostic and therapeutic strategy should be considered in select children with small bowel obstruction of uncertain etiology.
Introduction
Small intestinal venous malformations are rare vascular anomalies. The term “hemangioma” was historically used to describe many vascular lesions of the gastrointestinal tract, but according to the current ISSVA classification, vascular anomalies are divided into two main categories: vascular tumors (e.g., infantile hemangioma) and vascular malformations (e.g., venous malformation) (, ). Venous malformations are non-neoplastic lesions composed of dilated, thin-walled vascular channels, distinct from true vascular tumors. Most reported cases in the literature—often under the older term “cavernous hemangioma”—present with gastrointestinal bleeding or anemia (, ). Acute bowel obstruction as the sole manifestation, particularly without hemorrhage, is uncommon in children. This article reports a case of acute intestinal obstruction caused by a small intestinal venous malformation in a child, confirmed by immunohistochemistry according to ISSVA criteria.
Case presentation
A 9-year-old boy presented with a one-day history of abdominal pain and vomiting. He denied dizziness, fatigue, or pallor. On admission, vital signs were as follows: temperature 36.9 °C, heart rate 88 beats/min, respiratory rate 20 breaths/min, and blood pressure 96/56 mmHg. Physical examination revealed a flat abdomen with mild guarding and periumbilical tenderness. Skin and mucous membrane examination showed no hemangiomas, telangiectasias, or abnormal pigmentation. Laboratory findings showed: hemoglobin 125 g/L, white blood cell count 8.91 × 10⁹/L, neutrophil ratio 75.5%, lymphocyte ratio 18.7%, and high-sensitivity C-reactive protein 0.99 mg/L. A fecal occult blood test was negative.
The patient was initially evaluated at an external hospital, where non-contrast CT suggested intestinal obstruction, and empiric antibiotic therapy was initiated. However, his abdominal pain did not improve significantly, prompting transfer to our institution. Upon arrival, abdominal radiography revealed incomplete small bowel obstruction, as evidenced by scattered gas within the bowel loops, dilatation of several small bowel loops, and multiple air-fluid levels of varying heights (Figure 1). Concurrently, contrast-enhanced abdominal CT demonstrated a well-circumscribed lesion in the left pelvic small bowel suggestive of a hypervascular lesion (Figures 2A,B). No phleboliths were identified. Given these CT findings and the persistent obstructive symptoms, diagnostic laparoscopy was performed.
Figure 1
Figure 2
The entire small bowel was examined from the ligament of Treitz to the ileocecal valve, and no other lesions were identified. A segment of small intestine measuring approximately 5 cm was found, its serosal surface densely covered with tortuous and engorged vessels (Figure 3A). The affected segment appeared viable with no signs of ischemia or perforation. Laparoscopic segmental resection with 2 cm macroscopic margins, (Figure 3B) followed by primary hand-sewn end-to-end anastomosis, was successfully completed without conversion to open surgery. The operative time was 165 min, and estimated blood loss was 10 mL. The nasogastric tube was removed on postoperative day 3, and oral feeding was resumed on postoperative day 4. The patient had an uneventful recovery and was discharged on postoperative day 9, with a total hospital stay of 13 days.
Figure 3
Histopathological examination of the resected specimen was performed with hematoxylin and eosin staining (Figure 4A). Immunohistochemical staining demonstrated that lesional endothelial cells were strongly positive for CD31 (Figure 4B) and CD34 (Figure 4C), and negative for GLUT-1 (Figure 4D). Both surgical margins were negative for lesion involvement. At 6-month follow-up period, the patient remained asymptomatic with no evidence of residual lesion or symptom relapse.
Figure 4
Discussion
In the older literature, many lesions reported as “cavernous hemangioma” or simply “hemangioma” of the small bowel would currently be classified as venous malformations under ISSVA criteria, because they consist of dilated, thin-walled vascular channels lined by flattened endothelium without endothelial hyperplasia (). We have therefore included these historical cases for clinical comparison, while recognizing that their nomenclature predates the current ISSVA classification.
We report a case of a solitary venous malformation of the small intestine in a 9-year-old boy who presented with acute bowel obstruction without any evidence of hemorrhage. The diagnosis was suspected preoperatively based on imaging features and definitively established postoperatively by immunohistochemical staining (CD31+, CD34+, GLUT-1–). This case is notable for three reasons: (1) the lesion caused acute obstruction rather than the more typical gastrointestinal bleeding; (2) preoperative contrast-enhanced CT demonstrated a hypervascular lesion, raising suspicion of a vascular anomaly; and (3) laparoscopic segmental resection was completed successfully without conversion to open surgery.
According to the ISSVA classification, vascular anomalies are broadly categorized into vascular tumors and vascular malformations (, ). Venous malformations are non-neoplastic structural anomalies of the vasculature, whereas infantile hemangiomas are true vascular tumors with a distinct proliferative phase, potential for spontaneous involution, and characteristic GLUT-1 expression (, ).
In the present case, the lesional endothelial cells were strongly positive for CD31 and CD34, confirming vascular origin, but GLUT-1 was negative, and no endothelial hyperplasia was observed. The diagnosis of venous malformation rests on the combined findings of characteristic morphology—dilated channels with flattened endothelium and absent hyperplasia—and the CD31+/CD34+/GLUT-1− immunoprofile (). The original pathology report used the term “hemangioma” based on H&E morphology alone; the immunohistochemical findings now permit a more precise classification.
GLUT-1 negativity excludes infantile hemangioma but does not by itself distinguish venous malformation from congenital hemangioma. Congenital hemangioma of the small intestine is exceptionally rare, typically presents at birth, and undergoes rapid involution or non-progression. Our patient had no such history, and the lesion was identified at age 9 years. Thus, the combination of GLUT-1 negativity, lack of endothelial hyperplasia, and the clinical context supports the diagnosis of venous malformation.
We also considered whether this venous malformation was part of a systemic syndrome. Blue rubber bleb nevus syndrome (BRBNS) is characterized by multiple cutaneous and gastrointestinal venous malformations (), while hereditary hemorrhagic telangiectasia (HHT) may involve gastrointestinal arteriovenous malformations (). Our patient had no cutaneous vascular lesions, no family history of bleeding or telangiectasias, and no evidence of visceral involvement beyond the small intestine, effectively ruling out these syndromic associations.
Most gastrointestinal venous malformations present with bleeding, leading to chronic anemia (). In our patient, however, the fecal occult blood test was negative, and the obstruction was not caused by intussusception or intraluminal blockage. Histologically, the lesion involved the muscularis propria and subserosa, with the overlying mucosa remaining intact. Although the precise mechanism cannot be determined from histology alone, we hypothesize that the extensive mural involvement resulted in segmental rigidity and impaired peristalsis, thereby precipitating obstruction. A similar mechanism has been proposed in other reports (, ).
Contrast-enhanced CT played a useful role in the preoperative evaluation. The lesion appeared as a homogeneously enhancing mass with persistent enhancement on delayed phases, favoring a slow-flow vascular malformation over a hypervascular tumor (, ).
Phleboliths represent calcified thrombi within the slow-flow channels of venous malformations. When present in a young patient, they are virtually pathognomonic for the diagnosis (). However, phleboliths are not invariably present: Levy et al. () noted that these lesions are soft and compressible unless they contain areas of thrombosis or phleboliths, indicating that phlebolith formation is a secondary phenomenon. Yoo () reported that phleboliths are radiographically evident in only approximately 50% of gastrointestinal venous malformations.
The rarity of phleboliths in children further supports this view. Yeoman and Shaw () identified phleboliths in only 4 of 12,000 pelvic radiographs in children. Azouz (), citing the series of Marquis and Dovey, emphasized that pelvic phleboliths are virtually absent in children. Consistent with these observations, Han et al. () found histologically documented calcification in only one of three pediatric cases, and Sugito et al. () described a 2-year-old child with a small intestinal venous malformation in whom phleboliths were not observed on preoperative imaging.
Therefore, while the presence of phleboliths in a young patient strongly supports the diagnosis of a venous malformation, their absence—particularly in a child—should not diminish diagnostic confidence when other clinical, radiologic, operative, and histopathologic findings are consistent.
In our case, CT was the key imaging modality because the patient presented with acute obstruction. In a child with acute obstruction and no bleeding, the presence of a hypervascular lesion should raise suspicion of a vascular anomaly. This preoperative clue helped narrow the differential diagnosis and guided the decision to proceed with laparoscopic exploration ().
A review of the literature confirms that isolated obstruction without hemorrhage is uncommon in pediatric small bowel venous malformations. Fu et al. () found that among 25 pediatric cases, melena was the most common presentation (44%), followed by anemia (28%), while obstruction was observed in only one case (4%). Sugito et al. () reported a 2-year-old with an ileal venous malformation presenting with melena and anemia. Abdul Aziz et al. () reported a 6-year-old with a venous malformation causing subacute obstruction after trauma, but their case had active bleeding within the lesion. Our case differs in that the lesion caused acute obstruction without any bleeding, and the definitive diagnosis of venous malformation was established postoperatively by histopathology and immunohistochemistry. This unusual presentation adds to the clinical spectrum of small bowel venous malformations and underscores the importance of including vascular anomalies in the differential diagnosis of pediatric bowel obstruction of unclear etiology.
Laparoscopic segmental resection was performed successfully, with minimal blood loss (10 mL), clear histological margins, and an uneventful recovery. This approach offers advantages over open surgery in children, including reduced postoperative pain, shorter hospital stay, and better cosmetic outcomes (). A 2 cm macroscopic margin was selected empirically to ensure complete excision of the serpiginous vessels visible on the serosal surface. In selected cases, laparoscopic resection appears safe and feasible, provided the lesion is localized and the surgeon is experienced in pediatric minimally invasive techniques. If the lesion is not readily identifiable intraoperatively, intraoperative enteroscopy or preoperative tattooing may aid in localization (, ). Kong et al. () reported successful transumbilical single-site laparoscopic resection of a small intestinal cavernous hemangioma (venous malformation) in a 21-month-old child. Abdul Aziz et al. () performed segmental resection in their 6-year-old patient with good outcomes.
Several limitations should be acknowledged. First, immunohistochemistry for D2-40 (podoplanin) was not performed; therefore, a lymphatic component cannot be definitively excluded based on immunophenotype alone. While the morphological features are most consistent with a venous malformation, D2-40 negativity is required for definitive exclusion of a lymphatic or venolymphatic malformation. This represents a limitation and should be addressed in future cases. Second, genetic testing was not performed to definitively exclude RASA1-related disorders or other hereditary vascular anomaly syndromes, although clinical examination and family history made syndromic associations unlikely. Third, this is a single case report, and conclusions cannot be generalized. Fourth, the follow-up period is limited to six months; longer observation is needed to assess for late complications such as bleeding or stricture. Despite these limitations, the completeness of clinical, imaging, and immunohistochemical data provides useful guidance for clinicians managing similar rare cases.
Conclusion
This case suggests that a solitary venous malformation of the small intestine can present as acute bowel obstruction without hemorrhage in a child. Clinicians should consider vascular anomalies in the differential diagnosis of pediatric obstruction that does not respond to conservative management. Contrast-enhanced CT is a useful preoperative tool, and laparoscopic segmental resection appears a safe and effective treatment in selected cases. Future studies with longer follow-up and genetic testing are needed to better define the optimal management of these rare lesions.
Statements
Data availability statement
The original contributions presented in the study are included in the article/Supplementary Material, further inquiries can be directed to the corresponding author.
Ethics statement
The studies involving humans were approved by The Ethics Committee of the First Affiliated Hospital of Gannan Medical University. The studies were conducted in accordance with the local legislation and institutional requirements. Written informed consent for participation in this study was provided by the participants’ legal guardians/next of kin. Written informed consent was obtained from the minor(s)' legal guardian/next of kin for the publication of any potentially identifiable images or data included in this article. Written informed consent was obtained from the participant/patient(s) for the publication of this case report.
Author contributions
WP: Formal analysis, Investigation, Supervision, Writing – original draft, Writing – review & editing. YZ: Investigation, Writing – original draft. ZL: Formal analysis, Resources, Supervision, Writing – review & editing. HL: Investigation, Supervision, Writing – review & editing. ZZ: Formal analysis, Investigation, Validation, Writing – review & editing. FC: Writing – review & editing, Writing – original draft.
Funding
The author(s) declared that financial support was received for this work and/or its publication. This work was supported by the National Natural Science Foundation of China (Grant No. 82460560), the Natural Science Foundation of Jiangxi Province (Grant No. 20242BAB25523), the Science and Technology Project of Jiangxi Provincial Health Commission (Grant No. 202410057), and the Science and Technology Bureau of Ganzhou City (Grant No. GZ2024ZSF183).
Conflict of interest
The author(s) declared that this work was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
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Summary
Keywords
children, laparoscopy, small intestine, surgery, venous malformation
Citation
Peng W, Zheng Y, Li Z, Zhao Z, Liu H and Chen F (2026) Laparoscopic resection of a small intestinal venous malformation presenting as acute bowel obstruction without hemorrhage in a child: a case report. Front. Pediatr. 14:1866822. doi: 10.3389/fped.2026.1866822
Received
27 April 2026
Revised
10 July 2026
Accepted
13 July 2026
Published
29 July 2026
Volume
14 - 2026
Edited by
Meng Kong, Children’s Hospital Affiliated to Shandong University, China
Updates
Copyright
© 2026 Peng, Zheng, Li, Zhao, Liu and Chen.
This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
*Correspondence: Feng Chen a02416@gmu.edu.cn
† These authors have contributed equally to this work
Disclaimer
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.