CA S E R EP O RT
Wunderlich syndrome secondary to ureteropelvic junction
obstruction
Necmi Bayraktar, M.D.,1,2
1
Department of Urology, Burhan Nalbantoglu State Hospital, Nicosia-North Cyprus
2
Department of Urology, Near East University School of Medicine, Nicosia-North Cyprus
ABSTRACT
Wunderlich syndrome (WS) is defined as a rare spontaneous renal hemorrhage. It mostly occurs with concomitant diseases without
trauma. It usually presents with the Lenk triad and is diagnosed in emergency departments with the effective use of advanced imaging
modalities such as ultrasonography, computerized tomography, or magnetic resonance imaging scanning. In the management of WS,
conservative treatment, interventional radiology, or surgical procedures are decided according to the patient’s condition and treated
appropriately. Conservative follow-up and treatment should be considered in patients whose diagnosis is stable. If diagnosed late, the
progression can be life-threatening. As an interesting case of WS, a 19-year-old patient was presented with hydronephrosis due to ureteropelvic junction obstruction. Spontaneous renal hemorrhage without a history of trauma is presented. The patient, who presented
to the emergency department with the sudden onset of flank pain, vomiting, and macroscopic hematuria was imaged by computed
tomography. The patient could be followed and treated conservatively for the first 3 days, and on the 4th day, his general condition
deteriorated, and he underwent selective angioembolization and then laparoscopic nephrectomy.WS is a serious, life-threatening emergency, even in young patients with benign conditions. Early diagnosis is mandatory. Delays in diagnosis and non-energetic approaches
can lead to life-threatening situations. In hemodynamically unstable non-malignant cases, the decision for immediate treatment, such as
angioembolization and surgery, should be taken without hesitation.
Keywords: Laparoscopic simple nephrectomy; selective angioembolization; spontaneous renal hemorrhage; wunderlich syndrome.
INTRODUCTION
Wunderlich syndrome (WS) is an acute, spontaneous, urological emergency characterized by nontraumatic subcapsular
and perirenal hemorrhage. Idiopathic WS is a scarce condition. WS was first described in 1856 by Carl Reinhold August
Wunderlich from Germany.[1] The most common benign and
malignant causes are renal angiomyolipoma and renal cell carcinoma.[2] Other causes are stone-related obstruction, renal
artery rupture, arteriovenous malformations, polyarteritis
nodosa, renal cysts, and infection. Retroperitoneal bleeding
causes life-threatening blood loss and hemodynamic instability.[3] Patients generally apply to hospitals with acute flank pain,
palpable flank mass, and hypovolemia, known as the compo-
nents of Lenk’s triad.[4] Ultrasound, multidetector computed
tomography, and/or magnetic resonance imaging play a significant role in the diagnosis.[5] Conservative follow-up should
be considered in patients with stable conditions. In unstable
patients, nephrectomy should be regarded as if transcatheter
angioembolization fails.
CASE REPORT
A 19-year-old male was applied to the emergency department with complaints of sudden and persistent right flank
pain, vomiting, and macroscopic hematuria. There was no
history or evidence of physical trauma. Physical examination
revealed pale skin, cold sweating, and tenderness at the right
Cite this article as: Bayraktar N, Bayraktar N. Wunderlich syndrome secondary to ureteropelvic junction obstruction. Ulus Travma Acil Cerrahi Derg
2023;29:641-643.
Address for correspondence: Necmi Bayraktar, M.D.
Near East University School of Medicine, Nicosia, Türkiye
E-mail: necmi.bayraktar@neu.edu.tr
Ulus Travma Acil Cerrahi Derg 2023;29(5):641-643 DOI: 10.14744/tjtes.2022.54502 Submitted: 31.05.2022 Revised: 06.11.2022 Accepted: 06.12.2022
OPEN ACCESS This is an open access article under the CC BY-NC license (http://creativecommons.org/licenses/by-nc/4.0/).
Ulus Travma Acil Cerrahi Derg, May 2023, Vol. 29, No. 5
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Bayraktar et al. Wunderlich syndrome secondary to ureteropelvic junction obstruction
(a)
(b)
Figure 1. (a) Axail and (b) Coronal images of hydronephrotic kidney with hemorrhage (arrow)
costovertebral angle. Blood pressure and heart rates were
90/60 mm/Hg and 120/min, respectively. Body temperature
was 36°C. There was no previous surgery or systemic diseases in the past medical history. Blood count indicated deep
anemia secondary to acute blood loss. Hemoglobin was 7.2 g/
dL, hematocrit 23,5%, WBC count was 9700 cells/µL, platelets were 164000, prothrombin time, INR, and aPTT were
normal. Renal and liver function tests were completely normal. The patient had HBsAg positivity.
Emergency abdominal ultrasound and noncontrasted computerized tomography (CT) revealed a huge-sized right kidney with severe hydronephrosis (Fig. 1). CT images were
suggestive of ureteropelvic junction obstruction as the cause
of hydronephrosis. At this stage, because of the patient’s unstable condition, urography and renal scintigraphy were not
considered necessary.
Subsequently, the patient was transferred to the urology
ward, and three units of erythrocyte suspension were transfused. After the patient’s vital signs were stabilized, a percutaneous nephrostomy tube was placed into the right kidney
for pain management and monitoring bleeding. During the 2nd
and 3rd days in the ward, the patient had manageable pain and
completely normal vital signs. The urine from the nephrostomy tube was dark brown but not actively hemorrhagic. On
the 4th day, the situation changed rapidly. Low blood pressure
(65/35 mmHg), tachycardia (over 120/min), and other signs of
hypovolemia were observed. Aggressive liquid replacement
and blood transfusions were made. The patient was immediately transferred to the Interventional Radiology room for
angioembolization. The patient was not stable after renal artery coil placement, and an urgent nephrectomy was planned.
Laparoscopic transperitoneal nephrectomy was performed.
Immediately after vascular (artery and vein) ligation, the patient rapidly stabilized (Fig. 2).
Postoperatively, two more units of erythrocyte suspension
were transfused. Kidney function tests were not changed.
The urethral catheter and abdominal drain were removed on
the 2nd and 4th postoperative days. Finally, he was discharged
from the hospital on the 7th day postoperatively.
Histopathology was reported as hydronephrotic changes, parenchymal atrophy, mixt type inflammatory infiltration with
fresh bleeding and significant hyperemia, chronic interstitial
nephritis, angiomatous changes, and massive interstitial hemorrhage.
Written informed consent was obtained from the patient for
the publication of the case report and the accompanying images.
DISCUSSION
Spontaneous renal bleeding is a rare condition. Although renal tumors are responsible in most cases, this condition may
less frequently be caused by mild infections such as hydronephrosis, pyelonephritis, tuberculosis, abscess, nephrolithiasis,
nephritis, aneurysm, infarction, and autoimmune diseases.[4]
Computed tomography is the most effective imaging method
for diagnosis. CT allows us to see intrarenal bleeding as well
as perinephric and subcapsular hematomas and, in the majority of cases, helps to find the underlying cause.[4]
Bleeding into the renal collecting system is usually self-limited. Unfortunately, the same cannot be assumed for hydronephrotic kidneys. Even though the bleeding is not excessive,
it can turn into a life-threatening situation. For the hydronephrotic kidneys, the area filled with blood is more spacious
and less restrictive. The presence of hydronephrosis and infection weakens the kidney structurally, making it more fragile
and may cause spontaneous ruptures.
Figure 2. Clipped renal artery with endovascular coil (arrow)
642
The majority of patients treated conservatively are those with
benign conditions. It should be noted that the state of patients who are considered to be stable may be unpredictable
and may change dramatically. In hemodynamically unstable
patients, treatment options are selective angioembolization
or emergency nephrectomy.[6,7] Although there are reported
partial excision and repair cases, the surgical procedure usually ends with nephrectomy in most cases.
Ulus Travma Acil Cerrahi Derg, May 2023, Vol. 29, No. 5
Bayraktar et al. Wunderlich syndrome secondary to ureteropelvic junction obstruction
Conclusion
roform and ether narcosis on the kidneys. Ann Surg 1894;19:630–2.
WS is an entity that can be easily diagnosed due to its typical
clinical features together with the help of imaging techniques.
Both selective angioembolization and surgical exploration are
lifesaving treatments. More research and clinical experience
are needed to determine underlying predisposing factors and
pathogenesis, especially in patients with benign conditions.
Informed Consent: Written informed consent was obtained from the patient for the publication of the case report
and the accompanying images.
2.
Wunderlich syndrome secondary to the rupture of an aneurysm of the
renal artery. Review of the literature. Arch Esp Urol 1992;45:417–20.
3.
Alonso AR, Casas TF, Atienza PA, Sanchez MB. Wunderlich disease. First manifestation of a renal adenocarcinoma. Arch Esp Urol
1992;45:73–5.
4.
Bergasa FM, Herrador JV, de la Muela Naverac PS, Reyes JJ. Wunderlich
syndrome caused by the rupture of a simple cyst in a patient treated with
acetyl salicylic acid. Actas Urol Esp 1998;22:362–5.
5.
Katabathina VS, Katre R, Prasad SR, Surabhi VR, Shanbhogue AK,
Sunnapwar A. Wunderlich syndrome: Cross-sectional imaging review. J
Peer-review: Externally peer-reviewed.
Comput Assist Tomogr 2011;35:425–33. [CrossRef ]
Conflict of Interest: None declared.
Financial Disclosure: The authors declared that this study
has received no financial support.
6.
Escudero RM, Castillo OA. Spontaneous retroperitoneal haemorrhage
of renal origin (Wunderlich syndrome): Analysis of 8 cases. Arch Esp
Urol 2013;66:925–9.
7.
REFERENCES
1.
Ruiz CR, Velazquez ET, Creixell CM, Barja JV, Palacio EV, Rosello AS.
Gurbani CM, Khor V, Leow JJ, Tay MH, Chong YL. Wunderlich syndrome secondary to cyst rupture and concurrent anticoagulation. Can J
Warbasse JP. II. Wunderlich on clinical observations on the effect of chlo-
Urol 2020;27:10270–2.
OLGU SUNUMU - ÖZ
Üreteropelvik bileşke darlığına sekonder Wunderlich sendromu
Dr. Necmi Bayraktar,1,2
1
Burhan Nalbantoğlu Devlet Hastanesi, Üroloji Departmanı, Nicosia
2
Yakın Doğu Üniversitesi Tıp Fakültesi, Üroloji Departmanı, Nicosia
Wunderlich sendromu, nadir görülen bir spontan böbrek kanaması olarak tanımlanır. Çoğunlukla travma olmaksızın zemininde eşlik eden hastalıklarla ortaya çıkar. Genellikle Lenk triadı ile prezente olur ve acil servislerde ultrasonografi, BT veya MRG taraması gibi gelişmiş görüntüleme
yöntemlerinin etkin kullanımı ile teşhis edilir. Wunderlich sendromunun tedavisinde konservatif tedavi, girişimsel radyoloji veya cerrahi prosedürler
kullanılarak, hastanın durumuna göre karar verilir ve uygun yöntemle tedavi edilir. Tanı da stabil olan hastalarda konservatif takip ve tedavi düşünülmelidir. Geç teşhis edilirse, progrese olması halinde hayat tehdit edici olabilir. İlginç bir Wunderlich Sendromu olgusu olarak, 19 yaşında üreteropelvik bileşke (UPJ) obstrüksiyonuna bağlı hidronefroz hastasında, travma öyküsü olmayan spontan böbrek kanaması sunulmaktadır. Ani başlayan
yan ağrısı, kusma ve makroskopik hematüri şikayetleri ile acil servise başvuran hastanın bilgisayarlı tomografisi çekildi. İlk üç gün konservatif olarak
takip ve tedavi edilen hasta, 4. günde genel durumu bozulması üzerine hastaya selektif anjiyoembolizasyon ve ardından laparoskopik nefrektomi
uygulandı. Wunderlich sendromu, benign hastalıklar zemininde genç hastalarda dahi ciddi, yaşamı tehdit eden bir acil durumdur. Erken teşhis önem
arzeder. Tanıdaki gecikmeler ve enerjik olmayan yaklaşımlar yaşamı tehdit eden durumlara yol açabilir. Hemodinamik olarak stabil olmayan benign
vakalarda da, anjiyoembolizasyon ve cerrahi gibi acil tedavi kararı tereddüt etmeden alınmalıdır.
Anahtar sözcükler: Laparoskopik basit nefrektomi; selektif anjioembolizasyon; spontan böbrek kanaması; Wunderlich sendromu.
Ulus Travma Acil Cerrahi Derg 2023;29(5):641-643
doi: 10.14744/tjtes.2022.54502
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