Bilateral acquired Perirenal Lymphangiectasia: case report

Abstract

Bilateral perirenal lymphangiectasia is a rare condition characterized by the dilation of lymphatic ducts and the formation of fluid-filled cavities. Renal lymphangiectasia is rarely observed, affecting approximately 1% of cases, with only 34 bilateral cases reported in adults by 2025. This case report discusses a 55-year-old male with a history of recurrent pyelonephritis who was eventually diagnosed with bilateral acquired perirenal lymphangiectasia based on CT, MRI, and cytochemical analysis of perirenal fluid. The patient exhibited mild left flank pain without further complications, and given the disease's benign nature, an expectant management approach was adopted. Follow-up assessments confirmed an asymptomatic status. This report highlights the importance of considering lymphangiectasia in differential diagnoses for renal cystic lesions to avoid unnecessary procedures. Our case is the 35th documented instance of bilateral renal lymphangiectasia in the literature, underscoring the need for medical awareness of this condition to improve diagnosis and patient care.

Introduction

Lymphangiectasia is a rare condition characterized by the dilation of lymphatic ducts, which form cavities filled with lymphatic fluid [1]. Renal involvement is extremely rare (1%) and can be found in a perirenal, peripelvic, or intrarenal location. It has a similar incidence in men and women, affects both children and adults, and can be either congenital or acquired [2]. By 2025, 105 cases had been reported, 34 of which were adults with bilateral involvement [3] (See Table 2).

Table 2.

Demographics and treatment options of 35 cases of bilateral renal lymphangiectasia in adults.

#	Author	Year published	Country	Sex	Age (years)	Treatment
1	Riehl et al. [4]	1977	Germany	Male	22	Conservative
2	De Maeyer et al. [5]	1982	Belgium	Male	43	Resection (lumbotomy)
3	Murakumo et al. [6]	1986	Japan	Female	37	Resection
4	Kutcher et al. [7]	1987	USA	Female	74	Nephrectomy
5	Meredith et al. [8]	1988	USA	Female	23	Nephrectomy
6	Meredith et al. [8]	1988	USA	Female	NS	Conservative
7	Murray et al. [9]	1991	USA	Male	30	Resection (Nephrectomy)
8	Schwarz et al. [10]	1993	Germany	Male	42	Conservative resection (Marsupialization)
9	Burton et al. [11]	1994	United Kingdom	Male	35	Percutaneous drainage
10	Leder et al. [12]	1995	USA	Female	NS	NS
11	Varela et al. [13]	1998	Spain	Female	50	NS
12	Ozmen et al. [14]	2001	Turkey	Female	35	Percutaneous drainage
13	Ramseyer [15]	2001	USA	Male	28	NS
14	Llorente et al. [16]	2002	Spain	Male	30	Conservative
15	Shaheen et al. [17]	2003	USA	Male	36	Conservative resection (Marsupialization)
16	Sarikaya et al. [18]	2006	Turkey	Female	53	Percutaneous drainage
17	Ashraf et al. [19]	2007	United Kingdom	Female	23	Conservative
18	Rastogi et al. [20]	2008	India	Male	20	Percutaneous drainage
19	Chen et al. [21]	2009	China	Female	34	Conservative
20	Bagheri et al. [22]	2009	Iran	Male	21	NS
21	Ali Al-Dofri [23]	2009	Yemen	Male	22	Percutaneous drainage
22	P. Antonopoulos [24]	2010	Greece	Female	39	Conservative
23	Rastogi et al. [25]	2010	India	Male	60	Conservative
24	Bazari et al. [26]	2010	USA	Male	49	Conservative resection (Marsupialization)
25	Magu et al. [27]	2010	India	Male	28	Percutaneous drainage
26	Hakeem et al. [28]	2010	India	Female	50	NS
27	Viglietti et al. [29]	2012	France	Male	49	Percutaneous drainage
28	Karkouche et al. [30]	2013	France	Female	22	Conservative resection (Marsupialization)
29	Elbanna et al. [31]	2015	Saudi Arabia	Male	38	Conservative
30	Renacci et al. [32]	2017	USA	Male	30	Conservative
31	Umapathy et al. [33]	2020	India	Female	69	Conservative
32	Umapathy et al. [33]	2020	India	Male	43	Conservative
33	Alzahrani et al. [34]	2021	Saudi Arabia	Female	39	Conservative
34	Ayed et al. [2]	2024	Saudi Arabia	Female	55	Conservative
35	Present case	2025	Ecuador	Male	55	Conservative

NS: Non specified

This case report is particularly relevant because of the condition's exceptionally low frequency, unusual location, and evidenced acquired nature based on imaging findings.

Case report

The patient is a 55-year-old mestizo male with no relevant family or medical history who, in 2014, presented fever, chills, and tenderness on palpation of the left flank. Regarding complementary findings, neutrophilic leukocytosis was observed and creatinine levels were normal (See Table 1). Urinalysis revealed bacteriuria, which led to a diagnosis of pyelonephritis. The patient was treated with oral ciprofloxacin 500 mg twice daily for 10 days, with partial improvement. Due to persistent symptoms, he was hospitalized and treated with IV ceftriaxone 1 g twice daily for 7 days before being discharged with prophylactic antibiotics. Follow-up evaluations showed persistent bacteriuria but normal findings on contrast-enhanced computed tomography (CT), as confirmed by a radiologist's report.

Table 1.

Baseline serum parameters levels.

Serum paramethers	Reference values	Patient’s results
White Blood Cell Count	4000–11 000/μl	13 500/μl
Neutrophils (%)	40%–70%	85%
Lymphocytes (%)	20%–45%	12%
Creatinine	0.5–1.2 mg/dl	0.9 mg/dl
Urea	15–40 mg/dl	27 mg/dl
Blood Urea Nitrogen	7–20 mg/dl	18 mg/dl
Glucose	70–100 mg/dl	89 mg/dl

In 2019, the patient returned with stabbing left flank pain and foul-smelling urine. Urinalysis confirmed bacteriuria, and a urinary tract infection (UTI) was diagnosed. Treatment with ampicillin-sulbactam 375 mg orally twice daily for 10 days resolved symptoms, but the patient did not attend follow-up.

In 2022, the patient presented fever, anorexia, and general malaise. Considering his history of recurrent pyelonephritis, a CT was performed and revealed bilateral perirenal fluid with apparent septations. A subsequent magnetic resonance imaging (MRI) demonstrated subcapsular fluid encasing and displacing the renal parenchyma and adjacent structures, hypointense on T1, and hyperintense on T2. The right kidney contained approximately 332 ml of fluid, and the left 339 ml (Fig. 1). A perirenal fluid aspiration on the left flank showed yellow fluid with sparse lymphocytes, glucose 39.5 mg/dL, cholesterol 19.9 mg/dl, triglycerides 18.3 mg/dl, proteins 2.56 g/dl, albumin 1.85 g/dl, LDH 177 U/l, lipase 3.7 U/l, amylase 26 U/l, cells 0–1 per field, uncountable erythrocytes, and negative for Gram staining and culture, confirming the diagnosis of bilateral perirenal lymphangiectasia. Given the absence of complications, an expectant approach was chosen.

Figure 1.

Magnetic resonance imaging, T1, T2, and STIR sequences. The presence of subcapsular bilateral perirenal fluid envelopes and displaces the renal parenchyma and adjacent structures with apparent septa inside.

At routine follow-up in April 2024, the patient remained asymptomatic. No problems related to lymphangiectasia were observed on CT (Fig. 2), so expectant management continued (See the patient’s timeline in Fig. 3).

Figure 2.

Follow-up computed tomography, coronal and axial planes. No differences or complications were observed.

Figure 3.

Timeline of relevant events.

Patient perspective

I was highly concerned about my health because a doctor, upon reviewing the imaging tests, suggested a total nephrectomy and dialysis. I sought a second opinion at the UROMEDIK center, where I received a more detailed explanation of my condition, learning that I could keep my kidneys.

Discussion

This rare benign malformation of the renal lymphatic vessels has been documented under several terms, such as renal lymphangiectasia, renal lymphangiomatosis, and renal lymphangioma. The condition probably arises from a lack of communication within the renal and retroperitoneal lymphatic vessels, leading to lymphatic vessel dilation and cyst formation in and around the kidneys [2]. It affects both sexes equally and can occur at any age. Most cases are asymptomatic and 16% are identified incidentally [3].

This report shows evidence of an acquired bilateral perirenal lymphangiectasia in an adult, as the patient presented normal renal morphology on previous imaging examinations. The diagnosis was established through characteristic radiological findings supplemented by cytochemical fluid analysis. Nevertheless, the speed and accuracy were limited, probably due to the lack of clinical guidelines and the medical community’s lack of awareness of this condition.

Complications are rare but may include hypertension, ascites, and pleural effusion (the latter two of which should be treated immediately) [1, 3, 35, 36], and the percentage of different renal lymphatic vessels affected is unclear [1–3]. As there is no consensus for treating and supervising this condition, appropriate timing for follow-up should be determined based on the clinician’s criterion (annually in our case) and the potential complications’ usual standard of care (e.g. monthly or bimonthly in hypertension). In our case, the patient experienced mild pain in the left flank with no additional complications. Patient management may be expectant or include percutaneous drainage, medications, or, in severe cases, surgery, depending on the clinical presentation and complications [14, 19, 20]. Asymptomatic patients, like our case, usually require follow-up without invasive interventions. Hypertension, ascites, and pain can be controlled with antihypertensives, diuretics, and analgesics, respectively. Regarding the antihypertensives, the drug choice should follow the regular hypertension guidelines-guided standard of care. Ascites’ management should include loop or potassium sparing diuretics [37], and the analgesia should be personalized based on the patient’s needs.

This case contributes to the medical literature as the 35th documented instance of bilateral renal lymphangiectasia in adults, emphasizing the importance of considering this condition in differential diagnoses for renal cystic lesions.

Conclusion

Renal lymphangiectasia is a rare disease that usually remains asymptomatic. It is diagnosed through imaging studies and should be considered in the differential diagnosis for other renal cystic lesions. Radiologists and physicians must be familiar with this rare condition to ensure accurate diagnosis and treatment and avoid unnecessary invasive diagnostic and therapeutic procedures.

Ethics approval

Not applicable.

Informed consent

Written informed consent was obtained from the patient(s) for their anonymized information to be published in this article.

Conflict of interest

The authors declared no potential conflicts of interest regarding this article's research, authorship, and publication.

Funding

The authors received no financial support for this article's research, authorship, and publication.

Guarantor

Luis Fuenmayor-González, corresponding author.

CRediT statement

P-V. D. contributed to data collection, informed consent acquisition, writing the original draft, and writing, reviewing, and editing the final draft. P.D. contributed to data collection writing the original draft, and writing, reviewing, and editing the final draft. F-G. L. contributed to the conceptualization, methodology, project administration, validation, visualization, and writing, reviewing, and editing of the final draft.