Repeated testicular infarction in a patient with sickle cell disease: a possible mechanism for testicular failure

We report a case of repeated testicular infarction in a 39-year-old man with sickle cell disease. The patient presented with a 2-week history of testicular pain and was found clinically and sonographically to have a testicular mass, suspicious for a testicular tumor. The pathologic examination of the orchiectomy specimen revealed multiple infarcts, showing temporal variation ranging from acute (several days old) to recent (2 to 3 weeks old) to remote. This is the fifth case of segmental testicular infarction reported in patients with sickle cell disease/trait. We propose repeated testicular infarction as a probable mechanism of testicular failure and impaired fertility in patients with sickle cell disease.     

Intra‐flap thrombosis secondary to acute sickle crisis: A case report

We present the case of a 40‐year‐old patient with sickle cell trait who underwent bilateral breast reconstruction with microvascular TRAM flap transfer. Intraoperatively, the patient developed arterial anastomotic thrombosis of the right breast flap. The left breast flap had already been harvested and was placed on ice. Both anastomoses were then successfully completed. Postoperatively, the patient developed a pulmonary embolism and heparin‐induced thrombocytopenia. On postoperative day 12, the left cutaneous Doppler signals were lost, and exploration revealed a thrombosed pedicle and nonviable left breast flap. Pathologic specimen evaluation revealed sickled cells within the flap microvasculature. We believe that prolonged ischemia time and hypothermia precipitated erythrocyte sickling within the flap, causing intra‐flap thrombosis that propagated to the pedicle. While sickle cell diseases are not a contraindication to free tissue transfer, we believe that flap cooling should be utilized with caution in this circumstance. © 2012 Wiley Periodicals, Inc.     

Metachronous bilateral testicular tumors in a case of male infertility

A 42-year-old man referred to our hospital with the chief complaint of a solid mass of right scrotal contents. As ultrasonography revealed a right testicular tumor, right high orchiectomy was performed and the pathlogical diagnosis was testicular seminoma. At the age of 35-year-old, he was admitted to another hospital for male infertility due to azoospermia. Because left testicular tumor was found, left high orchiectomy was performed, with right testicular biopsy and testicular sperm extraction of the right testis at the same time. Pathological diagnosis revealed left testicular seminoma and no malignancy of the right testicular biopsy specimen. After the second operation, he has been receiving androgen replacement therapy, with no evidence of tumor recurrence.     

Don't forget sickled cells in the urine when investigating a patient for haematuria

Haematuria is a well-known complication of sickle cell disease.A South African coloured patient with repeated episodes of grosshaematuria is described in whom the diagnosis of sickle celldisease was suggested after the finding of sickled erythrocytesin the urine sediment. The diagnosis was then confirmed by haemoglobin electrophoresis, which revealed sickle cell trait (Hb-AS).It is concluded that sickled erythrocytes must be looked forwhen urine is microscopically scrutinized to determine the sourceof a haematuria.     

The effect of bicarbonate and distilled water on sickle cell trait hematuria and in vitro studies on the interaction of osmolality and pH on erythrocyte sickling in sickle cell trait.

The effect of intravenously administered distilled water was examined alone and during alkalization in a patient with gross hematuria associated with the sickle cell trait. On each of 4 occasions hematuria ceased promptly after the infusion of distilled water. Bicarbonate therapy also consistently decreased hematuria. In vitro studies on erythrocytes from another patient with sickle cell trait and hematuria demonstrated that slight increases in urinary pH similar to those that occur in the urine during alkalization can reverse or prevent erythrocyte sickling in the sicle cell trait. If patients with the sickle cell trait are hydrated adequately and have a good rate of urine flow distilled water can be given intravenously with virtually no danger of acute tubular necrosis secondary to erythrocyte hemolysis.     

Splenic syndrome in patients at high altitude with unrecognized sickle cell trait: splenectomy is often unnecessary.

BACKGROUND: The health risks associated with sickle cell trait are minimal in this sizable sector of the world's population, and many of these patients have no information about their sickle cell status. Splenic syndrome at high altitude is well known to be associated with sickle cell trait, and unless this complication is kept in mind these patients may be subjected to unnecessary surgery when they present with altitude-induced acute abdomen. METHODS: Four patients were admitted to the surgical ward with a similar complaint of acute severe left upper abdominal pain after arrival to the mountainous resort city of Abha, Saudi Arabia. All were subjected to splenectomy because of lack of suspicion regarding sickle cell status. RESULTS: Histologic examination of the spleen showed all patients had sickle cells in the red pulp. On further assessment all were found to have sickle cell trait with splenic infarction. In a similar study of 6 patients with known sickle cell disease who had comparable problems when they travelled to the Colorado mountains, all made an uncomplicated recovery with conservative management. CONCLUSIONS: In ethnically vulnerable patients with splenic syndrome, sickle cell trait should be ruled out before considering splenectomy. These patients could respond well to supportive management, and splenectomy would be avoided.     

Segmentaler Hodeninfarkt bei Sichelzellenanämie

Vascular occlusions are the clinical indicators of sickle cell disease and in urology they can lead to papillary necrosis, renal infarction or priapism. Segmental testicular infarction in patients with sickle cell disease is a rare event and only a few cases have been reported. We present a 25-year-old man with right testicular pain increasing over 3 days and sickle cell disease. Ultrasound of the right scrotum presented an inhomogeneous, mainly hypoechegenic mass with a hyperechogenic margin and no sign of blood flow. A partial orchiectomy was performed with total enucleation of the lesion, which was histologically diagnosed as benign hemorrhagic necrotic testicular tissue.     

Two cases of hematuria with hemoglobin C trait

Patients with sickle cell disease commonly experience painless hematuria. Hematuria may be found in patients with sickle cell trait, sickle cell anemia, and sickle cell hemoglobin C disease, but it is believed to be uncommon in patients with other hemoglobinopathies, such as hemoglobin C disease and hemoglobin C trait. We report two cases of children with hemoglobin C trait who presented with persistent painless hematuria. Because it is possible that hematuria in a patient with hemoglobin C trait is purely coincidental, all patients with a hemoglobinopathy and hematuria should undergo a complete evaluation so as not to overlook other causes of hematuria.     

Renal medullary carcinoma. Case report and review of the literature

Young patients with sickle cell trait or disease present a higher risk for renal medullary carcinoma, an aggressive renal tumor, with dismal prognosis, with a median survival of 4 months from the time of diagnosis. We report a 26-year-old patient with a 2-month history of bone pain at the right iliac crest, loss of weight, recurrent macroscopic hematuria and abdominal mass. Imaging studies demonstrated a 10-cm mass in the right kidney. Pathological evaluation revealed a medullary carcinoma and the patient died after 4 months, despite adjuvant treatment. Whenever a patient with sickle cell trait or disease presents with hematuria and/or flank pain, the possibility of a renal medullary carcinoma should always be kept in mind, with thorough investigation and expeditious treatment.     

Acute scrotal pain with a twist

Intratesticular lesions identified on ultrasound are usually malignant. It presents a particular dilemma to the surgeon when conservative approach is considered. A 55-year-old smoker with peripheral vascular disease had attended the accident and emergency with acute left hemiscrotal pain of 24-h duration. Clinical examination revealed a swollen, tender hemiscrotum. Ultrasound had demonstrated a hypo-echoic lesion in the testis measuring 2 × 1.8 cm. This was reported as possible infarct and managed conservatively. On review after 4 weeks, the patient was still symptomatic with persistent dull pain. A repeat scan in 4 weeks showed persistence of the lesion raising the possibility of tumour. Tumour marker profile was not elevated. The patient had a radical orchidectomy and the histology showed segmental infarction of the testis with thrombosis of the segmental testicular vessels. Peripheral vascular disease can cause segmental infarction of the testis due to the end arterial blood supply; in these cases, magnetic resonance scan can be diagnostic. Once confirmed, segmental infarcts can be safely managed conservatively.     

Segmental testicular infarction

We report a case of segmental testicular infarction occurring in a 24-year-old African Malian man who presented with a complaint of sudden and severe left testicular pain for 4 days. Scrotal ultrasound showed a hypoechoic mass in the left testicle. The hypoechoic area demonstrated no blood flow in colour Doppler mode. The patient underwent a left testicular exploration. A partial orchiectomy was performed with complete excision of the lesion. Pathological evaluation revealed a segmental testicular haemorrhagic infarction.     

Case of testicular venous hemangioma

A case of a testicular venous hemangioma is presented. A 65-year-old man complained of left testicular swelling. Physical examination and ultrasonography revealed a 2.0 x 1.6 x 1.5 cm roundish, well demarcated isoechoic elastic hard tumor in the left testis. No other abnormal findings including tumor markers were observed. Since preoperative examination did not rule out malignancy, we performed left high orchiectomy. Pathological diagnosis was a venous hemangioma of the left testis. Venous hemangioma is a rare entity among the testicular solid lesions.     

Unilateral Renal Venous Thrombosis Secondary to the Use of Vibration Belt in a Young Woman

We report on the isolated unilateral renal venous thrombosis (RVT) detected in a young patient who used vibration belt to stay thin. Apart from her sickle cell trait, the patient presented no other clinical situations associated with RVT.     

Testicular seminoma metastasis to duodenum. Misdiagnosed as primary duodenal tumor

IntroductionTesticular cancer is the most common malignancy in Males aged 15–35 years. Its incidence comprises 0.8% of all Male cancers worldwide, with a mortality rate of 0.1%. Rarely it metastasizes to the retroperitoneum and invades upper gastrointestinal tract (GIT). Complications like intestinal obstruction, hemorrhage, and perforation are usually present.Presentation of caseWe report a 30 year-old male, presented to GIT unit with severe anemia due to upper GIT bleeding. Esophagogastroduodenoscopy (OGD) documented duodenal growth. Histopathology findings of biopsy taken from the growth revealed, moderately differentiated adenocarcinoma of duodenum. Abdominal computed tomogram (CT) scan showed retroperitoneal mass which could be primary duodenal tumor or para − aortic lymph node. The patient was referred to surgery unit for pancreatico − duodenectomy with the diagnosis of primary duodenal malignancy. In Surgery unit, a left testicular mass was discovered. Ultrasound revealed suspicious mass in left testis. Review of duodenal biopsy (by another pathologist) was asked for. Duodenal metastatic seminoma was the diagnosis which was confirmed by immunohistochemical Stains (that was not done before). Left testicular biopsy showed testicular seminoma .Patient was treated by high inguinal orchiectomy followed by chemotherapy. One year, later the patient had no GIT symptoms, was not anemic and started to put on weight. Follow up endoscopy showed no evidence of tumor in duodenum. There was no evidence of retroperitoneal growth by follow up CT scan.DiscussionTestis lymphatic drainage is through para aortic lymph nodes .These are in contact with GIT. When testicular malignancy metastasizes to retroperitoneum it may invade GIT causing confusion whether symptoms are primarily from GIT, or they are primarily extra intestinal.ConclusionHigh index of suspicion for testicular seminoma must be raised when treating young males with GIT complications like hemorrhage. Testicular seminoma is the most common solid tumor at this age. Sometimes it is the cause behind this complication.     

Fungal splenic abscess in a sickle cell trait

Aim-Background

With an incidence of 0.07–0.14%, splenic abscess is a rare entity that occurs mainly in immunocompromised patients and carries a dismal outcome. With the advancement of chemotherapy and the widespread emergence of immunodeficiency disorders, splenic abscess is becoming more prevalent. This paper highlights the case of a unilocular fungal abscess in an immunocompetent patient with no known underlying immunodeficiency disorder, and with sickle cell trait being found at subsequent workup as the only predisposing factor.

Clinical Profile

A 39-year-old lady presented with low grade fever, pain in the left upper abdomen and a one-month history of shortness of breath. At admission, the patient appeared well but displayed high grade fever, tachycardia and tachypnoea. Abdominal examination revealed diffuse tenderness all over the abdomen with localized guarding and rigidity in the left hypochondrium.

Investigations

Laboratory investigations showed Hb 12.0gm/dl, leukocytosis ? 24,000 /mm³, differential polymorphonuclear count 56%, lymphocytes 30%, eosinophils 8%, monoctyes 4%, basophils 2%, and a platelet count of 650,000/cumm. Biochemical parameters indicated a mildly deranged renal function test. Abdominal CECT showed a large unilocular splenic abscess that had ruptured in the peritoneum. The patient was taken for exploratory surgery, which disclosed moderate splenomegaly with 1.5 litres of non-purulent fluid and a large abscess in the lower pole. A splenectomy was performed, followed by a thorough peritoneal lavage. A pus culture revealed candida species which was confirmed on Lacto phenol cotton blue mount. The patient was treated with fluconazole therapy for 21 days. An aetiological workup was done to rule out infective endocarditis, tuberculosis, diabetes, and other immunocompromised conditions. Haemoglobulin electrophoresis showed sickle cell trait. Twenty months later, the patient is currently completely asymptomatic.

Conclusion

Although a rare entity, fungal splenic abscess should be kept in mind as a possibility, particularly in patients with a long relevant history and imaging showing a single abscess. Such patients need to receive appropriate antifungal treatment to achieve good results.     

Sickle cell trait is not independently associated with susceptibility to end-stage renal disease in African Americans

Conflicting reports exist as to whether sickle cell trait is a risk factor for the progression of nephropathy. In order to determine whether African Americans with sickle cell trait are at increased risk for kidney disease, we assessed the genetic association between sickle cell trait and end-stage renal disease (ESRD). Hemoglobin S, non-muscle myosin heavy chain 9 (MYH9), and apolipoprotein L1 (APOL1) risk variants were genotyped in 3258 unrelated African Americans: 1085 with non-diabetic ESRD, 996 with type 2 diabetes-associated ESRD, and 1177 controls. Since APOL1 is strongly associated with ESRD in African Americans, interactions between APOL1 and MYH9 risk variants and hemoglobin S were assessed using case-only and case-control centered two-way logistic regression interaction analyses. The sickle cell trait genotype frequencies were 8.7% in non-diabetic ESRD, 7.1% in type 2 diabetes-ESRD, and 7.2% in controls. There was no age-, gender-, and admixture-adjusted significance for sickle cell trait association with non-diabetic ESRD (odds ratio 1.16); type 2 diabetes-ESRD (odds ratio 1.01); or all-cause ESRD (combined non-diabetic and type 2 diabetic-ESRD patients compared to the controls; odds ratio 1.05) in dominant models. In addition, no evidence of APOL1 or MYH9 interactions with sickle cell trait was detected. Hence, sickle cell trait is not associated with diabetic or non-diabetic ESRD in a large sample of African Americans.     

Aorta-coronary bypass in a patient with sickle cell trait.

A triple aorto-coronary bypass was performed in a patient with sickle cell trait. Partial exchange transfusion with normal packed erythrocytes was used in preparation of the patient for extracorporeal circulation, hypothermia and cardioplegic arrest.     

Embryonal carcinoma of the testis associated with prostate cancer in a 72-year-old man

A 72-year-old Japanese man presented with a painless swollen left scrotal mass with elevated levels of serum alpha-fetoprotein and prostate specific antigen. The patient underwent high orchiectomy under diagnosis and a final pathological examination revealed embryonal carcinoma of the left testis. A systematic needle prostate biopsy under guidance of transrectal ultrasound revealed prostate cancer (Gleason score, 8) on the left lobe (T2aN0M0). Systemic chemotherapy was given for retroperitoneal lymph node metastasis of testicular cancer and hormonal therapy (LH-RH analog) was given for prostate cancer. The patient was well with no evidence of metastasis from the testicular cancer or prostate cancer and with no elevation of serum alpha-fetoprotein or prostate specific antigen 26 months after the orchiectomy.     

Hypothermic cardiopulmonary bypass in a patient with sickle-cell trait

A male patient, aged 7.5 years, with known sickle cell trait, presented for correction of Fallot's tetralogy. The successful management under hypothermic cardiopulmonary bypass is described and the behaviour of sickle haemoglobin under hypothermic conditions is discussed.     

Concurrent Renal Cell Carcinoma and Transitional Cell Carcinoma in a Chronic Hemodialysis Patient

A 60-year-old female patient had been on maintenance hemodialysis for 12 years was suffering from gross hematuria. Subsequent image studies revealed left renal and ureteral tumors. She then received left radical nephroureterectomy. Histological examination revealed the renal tumor was renal cell carcinoma and ureteral tumor was transitional cell carcinoma respectively. To our knowledge, this is the first reported case of simultaneous occurrence of these two urological cancers in a chronic hemodialysis patient. Our case may imply the increased susceptibility of urological malignancy in dialysis patients. Physicians should always raise the possibility of urological malignancy when a dialysis patient with gross hematuria is encountered. A thorough and careful screening for the malignancy should be performed on a regular basis in these patients with high risk.