Small bowel lymphatic malformation: Clinical presentation and a comprehensive literature review

Table 1 Patient demographics, clinical features, and diagnostic findings

Ref.	Gender	Age (years)	Past history	Presentation	Duration of symptoms	Physical exam	Labs	Imaging modality	Site	Size (cm)	Multiplicity	Appearance on imaging
Akwei et al[46]	M	62	Inguinal hernia repair, hiatus hernia	Acute abdominal pain, nausea, vomiting	36 hours	Soft distended abdomen, palpable tender mass; pyrexial (38.2 °C)	↑Amylase (762 unit/dL), ↑ALT (191 IU/L), ↑GGT (508 unit/L)	CT	Proximal small bowel mesentery	25 × 15 × 10	Multiloculated	Large thin-walled multiloculated mass with chylous fluid
Al-Obeed and Abdulla[45]	M	56	Renal stones surgery, tympanoplasty	Epigastric pain, fullness, heartburn, constipation	3 months	Soft/Lax abdomen; no tenderness/rigidity	↑WBC, ↑bilirubin; ↓RBC, ↓HCT, ↓sodium	CT	Ileocecal valve	Not addressed	Not addressed	Small polypoidal mass
Barghash et al[44]	F	26	Post-abortion vaginal bleeding, smoking	Left-sided abdominal pain, distension	24 hours	Distension; tenderness (left iliac fossa/flank)	Leukocytosis; normal LFTs/electrolytes/amylase/CRP/Lactate	CT, MRI	Jejunal mesentery	Approximately 7	Singular (lobulated)	Lobulated low-density mass; peripheral enhancement
Bucciero et al[43]	M	28	Not addressed	Weakness, melena, anemia (Hb 4 g/dL)	Not addressed	Not addressed	Severe anemia	CT	Jejunum	Not addressed	Not addressed	Inhomogeneous mass with stenosis
Cai et al[42]	F	46	Not addressed	Abdominal pain, melena, fatigue, shortness of breath	3 months	Not addressed	Severe anemia (Hb approximately 90 g/L)	EGD, colonoscopy, CE, SBE	Proximal jejunum	6 × 5	Singular (multicystic)	Circumferential submucosal lesion; yellowish-white folds, bleeding points
Chae et al[40]	F	37	None	Severe abdominal pain, epigastralgia, fullness	Months (fullness); 2 weeks (pain)	Not addressed	Not addressed	CT	Jejunal mesentery	9 × 7	Multilocular	Hypodense well-capsulated cystic mass; whirling mesentery, proximal dilation
Chen et al[41]	F	27	None	Abdominal mass, epigastralgia, fullness after meals	Recent	Soft non-tender mass (upper left abdomen); normal bowel sounds	Normal tumor markers	US, MRI	Jejunal mesentery	15 × 8 × 6	Multilocular	Homogeneous mass with septa (MRI); multilocular cystic mass (US)
Chung et al[7]	M	31	None	Sudden severe abdominal pain, fever	Not addressed	Tenderness/guarding; palpable 10-cm hard mass (LLQ)	Leukocytosis (14600/mm3), ↑CRP (82.86 nmol/L)	CT	Jejunal mesentery	8 × 6 × 6	Not addressed	Low-density homogeneous oval; enhancing septum
Creger et al[39]	M	76	None	Incidental mass (asymptomatic)	Asymptomatic	10-cm irregular soft mass	Aspiration cytology was negative for malignancy	CT	Jejunal mesentery	9 × 6 (CT); 10 (exam)	Multiloculated	Multiloculated fluid-filled non-enhancing lesion
Cupido and Low[5]	F	42	Chronic iron deficiency anemia, menorrhagia	Menorrhagia, complex cystic mass	Several years	Not addressed	Not addressed	MRI	Small bowel mesentery	17.6 × 6.8 × 8.7	Multiloculated	Thin-walled multiloculated cyst; high T2/Low T1; no enhancement/solid components
Du et al[38]	M	54	Inguinal hernia surgery	Abdominal mass	4 days	No positive signs	Normal blood/tumor markers (AFP, CEA, CA12-5, CA199)	US, CT	Jejunum/small bowel mesentery	8 × 10	Not addressed	Not addressed
Hwang et al[37]	F	52	Hysterectomy/salpingectomy	Abdominal mass	Not addressed	No positive signs	Normal blood/tumor markers (AFP, CA199, CA12-5)	US, CT	Small bowel mesentery	5 × 5	Not addressed	Irregular low-density mass
Ignjatovic et al[13]	M	71	Angina (5 years)	Rectal hard mass, hematochezia	Not addressed	Hard rectal mass	Normal labs	CT, PET/CT	Jejunum/mesentery	8 × 6	Nodular/multiloculated	Soft-tissue density; hazy attenuations
Honda et al[14]	M	5-75 (range)	Not addressed	Abdominal pain, discomfort, nausea, vomiting	Approximately 6 months	Non-specific	Normal; tumor markers negative	US, MRI (inconclusive)	Ileal mesentery	2 × 1.5 × 1.3	Multiple locules	Lobulated cystic mass; milky fluid
Iwaya et al[36]	F	31	Not addressed	Profound anemia (Hb 53 g/dL)	Not addressed	Not addressed	Anemia; fecal occult blood+	Barium radiography, CT, enteroscopy	Proximal jejunum	3.5 × 3.0	Multicystic	Filling defect (barium); lobulated mass (CT); yellowish-white submucosal tumor
Jang et al[35]	M	70	Hypertension	Iron deficiency anemia, dark stool	3 years	Not addressed	Anemia	CE, DBE	Proximal small bowel	2.0 × 1.7 × 1.2	Singular	Raised granular lesion; white/thickened villi, oozing blood
Jayasundara et al[34]	M	43	Similar abdominal pain 20 years ago	Epigastric pain, nausea, vomiting	20 days (between visits)	Epigastric tenderness/rebound; hyperperistaltic bowel sounds	↑BUN (42.4 mg/dL), ↑creatinine (2.09 mg/dL)	X-ray, CT	Pelvic cavity (small bowel mesentery)	15 × 10 × 6	Multiple locules	Cystic mass; homogeneous fluid; “beaked” small bowel (CT)
João et al[33]	F	18	None	Acute abdominal pain, vomiting	4 hours	Tender (central/LUQ); early peritonism; temp 37.2 °C	Leukocytosis (13900/mm3), ↑CRP (63 nmol/L); normal amylase	CT	Jejunal mesentery	10 × 10 × 9	Two small polyps	Low-density mass encasing vessels
Khan et al[52]	F	29	None	Melena, severe anemia (Hb 55 g/dL)	Not addressed	Not addressed	Microcytic anemia (Hb 55 g/dL)	CE, enteroscopy	Proximal jejunum	1.2	Singular	Whitish “strawberry-like” mucosa, fresh blood
Konstantinidis et al[32]	F	24	None	Sudden right abdominal pain, nausea	Not addressed	Right abdominal tenderness	↑WBC (15.1 L)	CT, endoscopy	Small bowel (mesentery)	0.8-3.5	Not addressed	Well-demarcated thin-walled oval mass
Kopáčová et al[51]	F	31	None	Acute right abdominal pain, nausea	Not addressed	Guarding, tenderness	Mild leukocytosis	US (initially negative)	Mid-ileum	Not addressed	Not addressed	Well-circumscribed whitish multicystic mass
Li et al[31]	M	69	GERD, peptic ulcer, hemorrhoids	Melena, symptomatic anemia	6 months	Pale skin; melena	Anemia (Hb 60-8.5 g/dL)	CE, DBE	Small bowel	0.4 × 0.6	Singular	Nodular lesion (typical lymphatic malformation)
Lim et al[50]	M	70	Iron deficiency anemia	Anemia, dizziness, melena	3 years	Dizziness, weakness	Hb 52 g/dL (pre-operative); Hb 104 g/dL (post-operative)	CE	Jejunum	< 1	Multiple	Mucosal erosion, blood clot; multifocal erosions
Losanoff et al[29]	M	35	Not addressed	Painful abdominal mass	4 weeks	Baseball-sized hard tender mass (RLQ); hypoactive bowel sounds	Normal	CT, MRI	Terminal ileum mesentery	30 × 12 × 10	Single (multiple cysts possible)	Large cyst; soft tissue mass
Mavrogenis et al[30]	M	59	Not addressed	Melena	Not addressed	Not addressed	Anemia	CE, DBE	Proximal jejunum	3.5 × 7	Singular	Polypoid lesion; whitish/red spots, spontaneous bleeding
Botey et al[49]	M	52	Not addressed	Diffuse abdominal pain, diarrhea, and fever	3 days	Tympanic abdomen; diffuse pain, peritoneal irritation	↑CRP (5.8 mg/dL)	CT	Proximal jejunum	7.2 × 9.5 × 7.5	Multilocular	Multilocular cystic mass; thin walls, higher density
Nakamura et al[28]	M	60	Dialysis	Obscure GI bleeding	Not addressed	Not addressed	Not addressed	EUS, DBE	Jejunum	< 1	Not addressed	Hypoechoic mass with erosion/red sign
Stein et al[15]	M	21	Not addressed	GI bleeding, abdominal pain, weight loss	Months	Large nontender abdominal mass	Hb 70 g/dL (HCT 21%)	CT	Mesentery/duodenum	Not addressed	Not addressed	Large infiltrating spongy mass
Ong et al[27]	M	28	None	Recurrent abdominal pain	18 months	Not addressed	Not addressed	CT, MRI	Distal small bowel mesentery/pelvis	Not addressed	Multicystic	Multicystic pelvic lesion
Rieker et al[4]	M	61	Not addressed	Abdominal pain (LLQ), fever to chills/confusion	Not addressed	Not addressed	Not addressed	US, CT	Ileal mesentery	12 × 9 × 7	Multicystic	Multicystic tumor; clear fluid
Rathod et al[26]	F	18	None	Asymptomatic (incidental)	Asymptomatic	Normal	Normal	CT	Jejunal mesentery	10.5 × 10.0 × 6.0	Two cystic swellings	Large well-defined cystic mass; hyperdense fluid, thin capsule/septa
Rojas and Molina[25]	F	71	Cholecystectomy, appendectomy, hysterectomy	Nausea, lower abdominal pain, palpable mass	1 month	Palpable lower abdominal mass	Normal	CT	Small bowel mesentery	9 × 7 × 4	Singular	Mesenteric mass
Lin et al[24]	F	38	Not addressed	Melena, weakness	3 months (melena); 10 days (weakness)	Anemic appearance	Hb 74 g/L; albumin 20.9 g/L	CT	Fundus, peripancreatic, mesenteric, retroperitoneal, spleen	Largest 6.2	Multiple	Multiple small cystic lesions; no enhancement
Safatle-Ribeiro et al[23]	M	30	Pulmonary tuberculosis	Recurrent melena	14 years	Not addressed	Anemia	SBFT, DBE	Jejunum	15 (specimen)	Diffuse	Irregular mucosa; diffuse thickening/nodularity
Samuelson et al[22]	M	70	ADPKD, CHF, valvular cardiomyopathy	Abdominal pain, nausea, emesis, weight loss, hematemesis	Not addressed	Not addressed	Iron deficiency	CT	Jejunum	2.5	Two lymphatic malformations	“Target sign” (intussusception)
Tang et al[47]	F	38	None	Recurrent melena, anemia	Not addressed	1-cm oozing polypoid lesion	Anemia	CE	Proximal-mid small bowel	1	Singular	White-yellow “strawberry” mucosal pattern
Teng et al[21]	F	55	None	Right upper abdominal discomfort	2 months	Mild RUQ tenderness	Normal	CT	Jejunum	3 × 3; 2 × 2	Two	Space-occupying lesion; calcium deposition, enhancement
Tomizawa et al[20]	F	46	Hypothyroidism	Symptomatic anemia	1 year	Pallor	Hb 6 g/dL; iron deficiency	CT enterography	Small bowel mesentery	8.5 × 6 × 4	Lobulated	Large cystic lobulated mass
Torashima et al[19]	F	31	Appendectomy (age 14)	Upper abdominal pain (pregnancy)	24 weeks (gestation)	Abdominal distention, LUQ tenderness	WBC 7600/mm3; Hb 105 g/dL; CRP 0.19 mg/dL	US, CT, MRI	Ileum	19 × 8 × 5.5	Multiloculated	Thin cyst wall; multiloculated cystic lesion
Wei et al[48]	M	21	Known lymphatic malformation	Vomiting, severe LUQ pain	12 hours	Not addressed	Not addressed	CT	Jejunal mesentery	Not addressed	Not addressed	Low-attenuation mass; “swirl sign” (volvulus)
Yang et al[18]	F	37	None	GI hemorrhage, anemia, melena, dizziness	2 weeks	Not addressed	Anemia	CE, CT angiography	Ileum	6.5 × 4.5 × 0.9	Clustered nodules	Cystic: Low T1/high T2 signal
Yavuz et al[17]	F:14 M:8	40.68 (mean)	Not addressed	Abdominal pain (12), distension (6), mass (4), nausea/vomiting (3)	Not addressed	Palpable mass (4), distension (6)	Not addressed	US, CT	Small intestine (18), colon (4)	10.04 (mean; range 2-27)	Not addressed	Not specified
Yeh et al[16]	M:22 F:12	50 (median)	Varied (e.g., choriocarcinoma, gastric cancer)	OGIB (25), abdominal pain (9)	> 3 years (some)	Not addressed	Anemia (OGIB: Hb 63 g/dL)	CT, DBE	Small bowel	Not addressed	Not addressed	Varied (e.g., mass, angiodysplasia)

F: Female; M: Male; GRAD: Gastroesophageal reflux disease; ADPKD: Autosomal dominant polycystic kidney disease; CHF: Congestive heart failure; Hb: Hemoglobin; GI: Gastrointestinal; LLQ: Left lower quadrant; LUQ: Left upper quadrant; OGIB: Obscure gastrointestinal bleeding; RLQ: Right lower quadrant; RUQ: Right upper quadrant; ALT: Alanine aminotransferase; GGT: Γ-glutamyl transpeptidase; WBC: White blood cell; RBC: Red blood cell; HCT: Hematocrit; LFT: Liver function test; CRP: C-reactive protein; AFP: Alpha-fetoprotein; CEA: Carcinoembryonic antigen; CA: Cancer antigen; CT: Computed tomography; MRI: Magnetic resonance imaging; EGD: Esophagogastroduodenoscopy; CE: Capsule endoscopy; SBE: Small bowel enteroscopy; US: Ultrasound; PET: Positron emission tomography; SBFT: Small bowel follow-through.

Table 2 Management, histopathology, and outcomes

Ref.	Management (surgery/endoscopy)	Histopathology findings	Type	Complications (post-operative)	Follow-up duration	Recurrence
Akwei et al[46]	Surgical resection (incomplete)	Complex multiloculated lesion; flattened cells, vascular channels, lymphoid aggregates	Cystic	Infection, pelvic collection (E. coli), required CT-guided drain	1 year	Intra-abdominal collection (1 month); no cyst recurrence
Al-Obeed and Abdulla[45]	Laparoscopic right hemicolectomy	Dilated lymphatic vessels	Cystic	None	Not addressed	Not addressed
Barghash et al[44]	Segmental bowel resection	Dilated thin-walled channels/cystic spaces; flat endothelial cells, lymphocytes, RBCs	Cystic	None	8 weeks	Not specified (risk noted)
Bucciero et al[43]	Surgical resection (duodenum/jejunum)	Dilated lymphatic vessels; histiocytes, lymphangiectasia	Lymphatic malformation	Not addressed	Not addressed	Not addressed
Cai et al[42]	Surgical resection	Dilated lymphatic channels (mucosa/submucosa); simple lymphatic malformation with bleeding	Simple	None	Not specified	No melena recurrence
Chae et al[40]	Surgical resection	Multi-septate cystic masses; flat lymphatic endothelial cells	Cystic (macrocystic)	None	3 months	None
Chen et al[41]	Laparotomy	Dilated lymphatic channels; factor VIII+, actin+, cytokeratin-	Cystic	None	18 months	None
Chung et al[7]	Surgical excision	Dilated lymphatic spaces; collagenous stroma, flattened endothelial cells	Cystic	None	9 months	None
Creger et al[39]	Laparoscopy to open small bowel resection	Lymphatic malformation; no malignancy	Cystic	None	2 weeks	Not addressed
Cupido and Low[5]	Conservative (follow-up); surgery if symptomatic	Not specified (cystic lymphatic malformation)	Cystic	Not addressed	13 months	Not addressed
Du et al[38]	Not specified	Haemolymphangioma (CD34+, D2-40+)	Cystic	None	4 months	None
Hwang et al[37]	Laparoscopic resection	Haemolymphangioma (CD34+, D2-40+)	Cystic	None	4 months	None
Ignjatovic et al[13]	Laparoscopic abdominal transanal resection + ileostomy	Dark red multiloculated cystic lesion; CD34+ endothelial cells	Cavernous	Not addressed	Not addressed	Not addressed
Honda et al[14]	Surgical resection	Cystic lymphatic malformation	Cystic	None	1 year	None
Iwaya et al[36]	Surgical resection	Dilated lymphatic vessels; eosinophilic fluid/RBCs	Lymphatic malformation	Not addressed	Not addressed	Not addressed
Jang et al[35]	Laparoscopic small bowel resection	Dilated vascular channels; D2-40+ (lymphatic), CD31+ (blood)	Hemangiolymphangioma	Not addressed	Not addressed	Not addressed
Jayasundara et al[34]	Segmental resection + anastomosis	Lymphatic malformation	Cystic	Ischemic stricture	20 days	Complete small bowel volvulus
João et al[33]	Surgical excision	Thin-walled lymphatic spaces; eosinophilic material, neutrophils, lymphocytes	Cystic	None	6 months	Not addressed
Khan et al[52]	Endoscopic mucosal resection	Large dilated lymphatic channels	Cavernous	None	2 months	None
Konstantinidis et al[32]	Diagnostic laparoscopy to laparotomy (intussusception)	Cystic lymphatic malformation (irregular cysts)	Cystic	None	2 weeks	Not addressed
Kopáčová et al[51]	Laparoscopic resection	Multicyclic lymphatic malformation; markedly dilated channels	Cystic	None	Not addressed	Not addressed
Li et al[31]	Endoscopic resection (DBE)	Small-bowel lymphatic malformation	Cystic	None	4 months	Not addressed
Lim et al[50]	Surgical resection	Dilated lymphatics; thrombus/hemorrhage	Lymphatic malformation	Chronic anemia/melena (pre-operative); resolved post-op	3 months	None
Losanoff et al[29]	Exploratory laparotomy	Cystic mass; attenuated endothelium, smooth muscle, lymphocytes	Cystic	Hemorrhage, infection, lymphatic fistula (risk)	Not addressed	0%-100% (risk)
Mavrogenis et al[30]	Single-port laparoscopy	Mixed cavernous hemangioma-lymphatic malformation	Mixed	Not addressed	Not addressed	Not addressed
Botey et al[49]	Emergency laparotomy to bowel resection	Mesenteric lymphatic malformation; D2-40+	Cystic	Not addressed	Not addressed	Not addressed
Nakamura et al[28]	Endoscopic mucosal resection	Lymphatic malformation	None	Not addressed	Not addressed
Stein et al[15]	Open biopsy to alcohol ablation	Microcystic lymphatic malformation (small lymphatic channels)	Microcystic	Mild pain/fever (alcohol ablation)	18 months	None (no bleeding recurrence)
Ong et al[27]	Not addressed	Not addressed	Not addressed	Not addressed	Not addressed	Not addressed
Rieker et al[4]	Complete excision	Dilated lymphatic channels; factor VIII+, Ulex europaeus+	Cystic	None (initial); readmitted at 6 months	6 months (readmission)	Possible (incomplete removal risk)
Rathod et al[26]	Surgical excision	Smooth capsulated cysts; chalky white fluid	Cystic	None	Not addressed	Not addressed
Rojas and Molina[25]	Laparotomy	Encapsulated fat; dilated lymph vessels	Cavernous	None	Not specified	None (incomplete resection risk)
Lin et al[24]	Exploratory laparotomy	Dilated lymphatic channels; D2-40+	Cystic	Not addressed	Not addressed	Not addressed
Safatle-Ribeiro et al[23]	Surgical resection	Diffuse dilated lymphatic vessels	Cystic	Bleeding/wall thickness	42 months	None
Samuelson et al[22]	Not addressed	Not addressed	Not addressed	Cachexia, multiple cancers	Not addressed	Not addressed
Tang et al[47]	Laparoscopic segmental resection	Cavernous lymphatic malformation	Cavernous	Not addressed	Not specified (bleeding/anemia resolved)	Not addressed
Teng et al[21]	Excisional surgery	Chronic inflammation; low-grade neoplasia	Hemolymphangioma	None	6 months	None
Tomizawa et al[20]	Exploratory laparotomy	Cystic spaces with hemorrhage; D2-40+	Lymphatic malformation	Not addressed	Not specified	Not addressed
Torashima et al[19]	Laparotomy to bowel resection	Multicyclic spaces; attenuated endothelium, proteinaceous fluid	Cystic	None	18 months	None
Wei et al[48]	Emergency laparotomy to bowel resection	Dilated thin-walled channels; full-thickness bowel involvement	Lymphatic malformation	None	3 months	None
Yang et al[18]	Laparoscopic-assisted resection	Diffuse proliferative blood/Lymphatic vessels	Hemolymphangioma	Not addressed	1 year	Not addressed
Yavuz et al[17]	Enucleation (10), bowel resection (4), laparoscopic excision (4), hemicolectomy (3)	Simple cyst (17), lymphatic malformation (4), adenocarcinoma (1)	Cystic (simple/Lymphangioma)	SSI (3), anastomosis leak (1)	Not addressed	Not addressed
Yeh et al[16]	LABS (27), converted laparotomy (6)	Lymphatic malformation (1 case)	Lymphatic malformation	Transient fever/abdominal pain (tattoo leak)	14 ± 3 months	Symptomatic recurrence (2)

DBE: Double-balloon enteroscopy; LABS: Laparoscopy; RBC: Red blood cell; E. coli: Escherichia coli; SSI: Surgical site infection.