CASE 11024 Published on 09.06.2013

Small bowel volvulus induced by mesenteric lymphangioma in an infant

Section

Abdominal imaging

Case Type

Clinical Cases

Authors

Linda Metaxa1, Feidias Metaxas2, Maria Michailidou3, Ioannis Tsifountoudis4, Panagiota Metaxa5, Afroditi Haritanti6

(1) corresponding author: Linda Metaxa,
AHEPA General University Hostpital of Thessaloniki, Email: lindamet25@gmail.com
(2) AHEPA Hospital Thessaloniki, Email: Sonografer2008@yahoo.gr
(3) AHEPA Hospital Thessaloniki, Email: Docmairi@yahoo.gr
(4) 424 General Military Hospital, Thessaloniki, Email: tsifjo@gmail.com
(5) Limassol General Hospital of Cyprus limassolrad@yahoo.com
(6) AHEPA Hospital Thessaloniki, Email: aheparadiology@gmail.com
Patient

14 years, male

Categories
Area of Interest Abdomen, Gastrointestinal tract ; Imaging Technique CT
Clinical History
A fourteen-year-old male child presented at the Emergency Department of our hospital with severe abdominal pain and vomiting. During the clinical examination there was abdominal distension and a palpable abdominal mass was determined.
Imaging Findings
On radiographic imaging there were atypical hydroaeric levels.
Ultrasonography revealed a large anechoic cystic mass in the lower abdomen with septations.
On CT examination a dilated small bowel was seen tapering with a beaked appearance at the centre and this continued to a collapsed small bowel that abutted a cystic mass in the pelvic cavity. A thin fatty layer between the mass and the small bowel suggested that the mass probably originated from the mesentery rather than the bowel loop. The findings were suggestive of a small bowel volvulus and we presumed that the cystic mesenteric mass was the cause of the volvulus.
A surgical operation revealed a mass consisted of well-defined locules filled with clear fluid. There was no communication between the cystic mass and the small bowel. A segmental resection of the small bowel was performed and end-to-end anastomosis followed.
The mass was pathologically confirmed as being a mesenteric lymphangioma.
Discussion
Lymphangioma is a congenital malformation of the lymphatic vessels found usually in the head and neck of young patients. Lymphangioma of the small-bowel mesentery is an extremely rare entity having been reported for less than 1% of all lymphangiomas of the body [1]. In general they are commoner in children, 40% usually present by age 1 and 80% by age 5 (In our case the child was 14 years old) [2]. Volvulus usually is the result of bands, adhesions, Meckel's diverticulum, internal hernia, Ascariasis, ileal atresia, meconium ileus, enteroenterostomy, leiomyoma of the mesentery and following operations while mesenteric lymphangioma is described rarely.
It is believed that there is a congenital developmental defect of the lymphatics so that there are blind-ended lymphatic sacs which lack proper connections with the venous system that lead to dilatation and the creation of cystic masses [3]. There are also some other theories suggesting that it can be caused by abdominal trauma, inflammation, abdominal surgery, radiation lymphatic obstruction or lymphatic degeneration [4].
The most common symptom is a palpable abdominal mass and abdominal distension because of bowel obstruction and volvulus induced by the compression or rotation of the mass [5]. There are cases in the literature of asymptomatic patients [6] or cases of organ dysfunction because of infiltration into surrounding viscera [4, 7].
Ultrasound or computed tomography (CT) usually confirms the presence of a cystic multi-loculated mass with thin septations and clear fluid. Multi-planar CT can show the small bowel obstruction, the whirl sign of the mesenteric vessels and small bowel around the mesenteric vessels and the relation with the cystic masses of the mesenterium. Calcification may occur but is uncommon. Also it helps to exclude other causes of intra-abdominal masses such as enteric duplication cyst, enteric cyst, non-pancreatic-pseudocyst, cystic teratoma.
The signal pattern of lymphangiomas on MRI resembles that of fluid: low signal intensity on T1-weighted images and high signal intensity on T2-weighted images.
Because there is overlap in imaging features between pathological entities, CT and MRI might be insufficient to provide a definite diagnosis and a surgeon has to make the final diagnosis.
According to Kurtz et al in 1986, correct preoperative diagnosis is made in only 25% of cases [8]. The improvement of the imaging modalities and the multi-slice CT with 3D reconstructions improve the accuracy of the diagnosis [9], but still in many cases the differential diagnosis is only made after laparotomy [10].
Differential Diagnosis List
Small bowel volvulus in an infant induced by mesenteric lymphangioma
Mesenteric lymphangioma
Enteric duplication cyst
Non-pancreatic pseudocyst
Cystic teratoma
Reactive ascites
Duplication cysts arising from the bladder
Final Diagnosis
Small bowel volvulus in an infant induced by mesenteric lymphangioma
Case information
URL: https://www.eurorad.org/case/11024
DOI: 10.1594/EURORAD/CASE.11024
ISSN: 1563-4086