scholarly journals Case Report: Prune perineum syndrome: a rare case with an unfavourable outcome

F1000Research ◽  
2016 ◽  
Vol 5 ◽  
pp. 363
Author(s):  
Roberto I. Lopes ◽  
Francisco T. Dénes ◽  
Gustavo B. Messi ◽  
Marcos G. Machado
Keyword(s):  
Urinary Diversion ◽  
Social Acceptance ◽  
Case Reports ◽  
Vena Cava ◽  
Perinatal Period ◽  
Anterior Wall ◽  
Initial Result ◽  
First Case ◽  
Bilateral Cryptorchidism ◽  
Staged Reconstruction

Prune perineum syndrome (PPS) is a rare anomaly, with only two previous case reports, both dying in the perinatal period. We report the first case of PPS that reached childhood. The patient presented with a hypoplastic genitalia and bilateral cryptorchidism. There was no evidence of an anal orifice. A significant prune-like mass was observed, extending from the perineum to both gluteal regions and to a cephalic mid-line bony prominence, with a 1cm central orifice that discharged urine. MRI confirmed the previous findings and revealed a right crossed ectopic kidney, intestinal malrotation, a hypoplastic infrarenal inferior vena cava and a hypoplastic right iliac artery. Endoscopic evaluation through the orifice revealed a cavity lined by urothelial mucosa, with a small communication to the anterior urethra in its anterior wall. A staged reconstruction was planned, with a first-step urinary diversion through a continent abdominal reservoir associated to bilateral orchiopexy. He was discharged from the hospital three weeks later under intermittent catheterization. The next surgical step would be the resection of the perineal mass and its cavity associated to the removal of the prominent sacrococcygeal bones. Unfortunately, four months after the first surgery the patient developed an acute abdomen and was submitted to a laparotomy that revealed a necrotic ileal segment secondary to obstructive adherences. He developed severe malabsorption followed by septic shock, dying five weeks after the procedure. Due to the lack of literature, there is no consensus for the management of these cases. The wish of the family for a better quality of life and social acceptance, compelled us to perform a urinary diversion, to be followed by a plastic and orthopedic reconstruction. Despite the successful initial result, the patient developed a late abdominal obstruction that was misdiagnosed, precipitating his untimely death five months after the first procedure.

scholarly journals Case Report: Prune perineum syndrome: A rare case with an unfavourable outcome

F1000Research ◽  
2016 ◽  
Vol 5 ◽  
pp. 363
Author(s):  
Roberto I. Lopes ◽  
Francisco T. Dénes ◽  
Gustavo B. Messi ◽  
Marcos G. Machado
Keyword(s):  
Urinary Diversion ◽  
Social Acceptance ◽  
Case Reports ◽  
Vena Cava ◽  
Perinatal Period ◽  
Anterior Wall ◽  
Initial Result ◽  
First Case ◽  
Bilateral Cryptorchidism ◽  
Staged Reconstruction

Prune perineum syndrome (PPS) is a rare anomaly, with only two previous case reports, both dying in the perinatal period. We report the first case of PPS that reached childhood.The patient presented with hypoplastic genitalia and bilateral cryptorchidism. There was no evidence of an anal orifice. A significant prune-like mass was observed, extending from the perineum to both gluteal regions and to a cephalic mid-line bony prominence, with a 1cm central orifice that discharged urine. MRI confirmed the previous findings and revealed a right crossed ectopic kidney, intestinal malrotation, a hypoplastic infrarenal inferior vena cava and a hypoplastic right iliac artery. Endoscopic evaluation through the orifice revealed a cavity lined by urothelial mucosa, with a small communication to the anterior urethra in its anterior wall.            A staged reconstruction was planned, with a first-step urinary diversion through a continent abdominal reservoir associated to bilateral orchiopexy. The patient was discharged from the hospital three weeks later under intermittent catheterization. The next planned surgical step was the resection of the perineal mass and its cavity associated to the removal of the prominent sacrococcygeal bones. Unfortunately, four months after the first surgery the patient developed an acute abdomen and underwent a laparotomy that revealed a necrotic ileal segment secondary to obstructive adherences. He developed severe malabsorption followed by septic shock, dying five weeks after the procedure.            Due to the lack of literature, there is no consensus for the management of these cases. The wish of the family for a better quality of life and social acceptance, compelled us to perform a urinary diversion, to be followed by a plastic and orthopedic reconstruction. Despite the successful initial result, the patient developed a late abdominal obstruction that was misdiagnosed, precipitating his untimely death five months after the first procedure.

scholarly journals Mediastinal and pulmonary entomophthoromycosis with superior vena cava syndrome: case report

1989 ◽  
Vol 31 (6) ◽  
pp. 430-433 ◽  
Author(s):  
João Carlos Coelho Filiio ◽  
Jorge Pereira ◽  
Álvaro Rabello Júnior
Keyword(s):  
Superior Vena Cava ◽  
Superior Vena Cava Syndrome ◽  
Superior Vena ◽  
Amorphous Material ◽  
Vena Cava ◽  
Clinical Manifestations ◽  
Anterior Wall ◽  
Tropical Regions ◽  
First Case ◽  
Vena Cava Syndrome

The first case of mediastinal and pulmonary entomophthoromycosis with supe rior vena cava syndrome is reported. The patient presented with a history of edema of the face, neck and upper limbs as well as collateral circulation in the anterior wall of the chest. Histological examination of tissue from mediastinum revealed a granulomatous reaction with microabscesses surrounded by eosinophilic amorphous material and with broad hyphae in the center. Culture was not performed because a preliminary diagnosis of nonHodgkin's malignant lymphoma was made. Surgical correction of the obstructed area was performed and the patient was sucessfully treated with potassium iodide. The authors propose that mediastinal entomoph thoromycosis must be considered in the differential diagnosis of diseases causing superior vena cava syndrome in tropical and sub-tropical regions. This case enlarges the spectrum of clinical manifestations of the zigomycosis caused by Entomoph-thoraceae.

A Giant Coronary Sinus Aneurysm with Secondary Vena Cava Aneurysms

2016 ◽  
Vol 19 (1) ◽  
pp. 028
Author(s):  
Shengjun Wu ◽  
Peng Teng ◽  
Yiming Ni ◽  
Renyuan Li
Keyword(s):  
Computed Tomography ◽  
Heart Transplantation ◽  
Coronary Sinus ◽  
Unusual Case ◽  
Superior Vena ◽  
Vena Cava ◽  
Final Diagnosis ◽  
Rare Entity ◽  
First Case ◽  
Enhanced Computed Tomography

Coronary sinus aneurysm (CSA) is an extremely rare entity. Herein, we present an unusual case of an 18-year-old symptomatic female patient with a giant CSA. Secondary vena cava aneurysms were also manifested. The final diagnosis was confirmed by enhanced computed tomography (CT) and cardiac catheterization. As far as we know, it is the first case that such a giant CSA coexists with secondary vena cava aneurysms. Considering the complexity of postoperative reconstruction, we believe that heart transplantation may be the optimal way for treatment. The patient received anticoagulant due to the superior vena cava (SVC) thrombosis while waiting for a donor.

scholarly journals Emphysematous pyelonephritis with air bubble in the inferior vena cava

2020 ◽  
Vol 26 (1) ◽  
Author(s):  
Tiffany A. Perkins ◽  
Alberic Rogman ◽  
Murali K. Ankem
Keyword(s):  
Septic Shock ◽  
Inferior Vena Cava ◽  
Inferior Vena ◽  
Vena Cava ◽  
Rare Presentation ◽  
Emphysematous Pyelonephritis ◽  
Case Presentation ◽  
Air Bubble ◽  
First Case ◽  
The Right

Abstract Background Emphysematous pyelonephritis (EPN) with gas in the inferior vena cava (IVC) is a rare presentation and to our knowledge, this is the first case report in the urologic literature. Case presentation A 35-Year-old obese diabetic Hispanic female presented to the emergency room with a clinical picture of septic shock. Prompt computerized tomography scan revealed EPN with gas throughout the right renal parenchyma and extending to the right renal vein, IVC, and pulmonary artery. She died before surgical intervention Conclusion This case demonstrates that patients presenting with severe EPN have a high mortality risk and providers should acknowledge that septic shock, endogenous air emboli, or a combination of both could result in cardiovascular collapse and sudden death.

scholarly journals Fetus in fetu: two case reports from North African country

2021 ◽  
Vol 69 (1) ◽  
Author(s):  
Moutaz Ragab ◽  
Omar Nagy Abdelhakeem ◽  
Omar Mansour ◽  
Mai Gad ◽  
Hesham Anwar Hussein
Keyword(s):  
Histopathological Examination ◽  
Case Reports ◽  
Abdominal Mass ◽  
Mature Teratoma ◽  
Surgical Techniques ◽  
Rare Condition ◽  
Mass Effect ◽  
Fetus In Fetu ◽  
Vascular Connection ◽  
First Case

Abstract Background Fetus in fetu is a rare congenital anomaly. The exact etiology is unclear; one of the mostly accepted theories is the occurrence of an embryological insult occurring in a diamniotic monochorionic twin leading to asymmetrical division of the blastocyst mass. Commonly, they present in the infancy with clinical picture related to their mass effect. About 80% of cases are in the abdomen retroperitoneally. Case presentation We present two cases of this rare condition. The first case was for a 10-year-old girl that presented with anemia and abdominal mass, while the second case was for a 4-month-old boy that was diagnosed antenatally by ultrasound. Both cases had vertebrae, recognizable fetal organs, and skin coverage. Both had a distinct sac. The second case had a vascular connection with the host arising from the superior mesenteric artery. Both cases were intra-abdominal and showed normal levels of alpha-fetoprotein. Histopathological examination revealed elements from the three germ layers without any evidence of immature cells ruling out teratoma as a differential diagnosis. Conclusions Owing to its rarity, fetus in fetu requires a high degree of suspicion and meticulous surgical techniques to avoid either injury of the adjacent vital structures or bleeding from the main blood supply connection to the host. It should be differentiated from mature teratoma.

scholarly journals Bittersweet: infective complications of drug-induced glycosuria in patients with diabetes mellitus on SGLT2-inhibitors: two case reports

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Caroline Bartolo ◽  
Victoria Hall ◽  
N. Deborah Friedman ◽  
Chloe Lanyon ◽  
Andrew Fuller ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Fungal Infections ◽  
Case Reports ◽  
Sglt2 Inhibitor ◽  
Sglt2 Inhibitors ◽  
Urogenital Tract ◽  
Hypoglycemic Agents ◽  
Drug Induced ◽  
First Case ◽  
Increased Risk

Abstract Background Sodium-glucose co-transporter 2 (SGLT2) inhibitors are novel hypoglycemic agents which reduce reabsorption of glucose at the renal proximal tubule, resulting in significant glycosuria and increased risk of genital mycotic infections (GMI). These infections are typically not severe as reported in large systematic reviews and meta-analyses of the medications. These reviews have also demonstrated significant cardiovascular benefits through other mechanisms of action, making them attractive options for the management of Type 2 diabetes mellitus (T2DM). We present two cases with underlying abnormalities of the urogenital tract in which the GMI were complicated and necessitated cessation of the SGLT2 inhibitor. Case presentations Both cases are patients with T2DM on empagliflozin, an SGLT2 inhibitor. The first case is a 64 year old man with Candida albicans balanitis and candidemia who was found to have an obstructing renal calculus and prostatic abscess requiring operative management. The second case describes a 72 year old man with Candida glabrata candidemia who was found to have prostatomegaly, balanitis xerotica obliterans with significant urethral stricture and bladder diverticulae. His treatment was more complex due to fluconazole resistance and concerns about urinary tract penetration of other antifungals. Both patients recovered following prolonged courses of antifungal therapy and in both cases the SGLT2 inhibitor was ceased. Conclusions Despite their cardiovascular benefits, SGLT2 inhibitors can be associated with complicated fungal infections including candidemia and patients with anatomical abnormalities of the urogenital tract may be more susceptible to these infections as demonstrated in these cases. Clinicians should be aware of their mechanism of action and associated risk of infection and prior to prescription, assessment of urogenital anatomical abnormalities should be performed to identify patients who may be at risk of complicated infection.

scholarly journals A Rare Case Report of Unusual Path of Left Internal Jugular Central Line

2021 ◽  
pp. 1-3
Author(s):  
Rajashekar Rangappa Mudaraddi ◽  
Hany Fawzi Greiss ◽  
Navin Kumar Manickam
Keyword(s):  
Superior Vena Cava ◽  
Case Reports ◽  
Superior Vena ◽  
Vena Cava ◽  
Central Line ◽  
Left Superior Vena Cava ◽  
Common Procedure ◽  
Rare Case Report ◽  
The Right ◽  
Line Path

Central venous cannulation is the most common procedure performed in perioperative setting and intensive care unit. Many case reports reported unusual positioning of central line catheters. Here, we would like to report a case of central line path in persistent left superior vena cava, a rare entity with a course similar to the right internal jugular central line. Preoperative computed tomography chest showed duplex superior vena cava which was not reported.

scholarly journals Granulomatous interstitial nephritis in a patient with SARS-CoV-2 infection

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Katarzyna Szajek ◽  
Marie-Elisabeth Kajdi ◽  
Valerie A. Luyckx ◽  
Thomas Hans Fehr ◽  
Ariana Gaspert ◽  
...  
Keyword(s):  
Acute Kidney Injury ◽  
Interstitial Nephritis ◽  
Kidney Biopsy ◽  
Kidney Diseases ◽  
Case Reports ◽  
Kidney Injury ◽  
Maculopapular Rash ◽  
Tubular Injury ◽  
Granulomatous Interstitial Nephritis ◽  
First Case

Abstract Background Acute kidney injury (AKI) associated with severe coronavirus disease 19 (COVID-19) is common and is a significant predictor of morbidity and mortality, especially when dialysis is required. Case reports and autopsy series have revealed that most patients with COVID-19 – associated acute kidney injury have evidence of acute tubular injury and necrosis - not unexpected in critically ill patients. Others have been found to have collapsing glomerulopathy, thrombotic microangiopathy and diverse underlying kidney diseases. A primary kidney pathology related to COVID-19 has not yet emerged. Thus far direct infection of the kidney, or its impact on clinical disease remains controversial. The management of AKI is currently supportive. Case Presentation The patient presented here was positive for SARS-CoV-2, had severe acute respiratory distress syndrome and multi-organ failure. Within days of admission to the intensive care unit he developed oliguric acute kidney failure requiring dialysis. Acute kidney injury developed in the setting of hemodynamic instability, sepsis and a maculopapular rash. Over the ensuing days the patient also developed transfusion-requiring severe hemolysis which was Coombs negative. Schistocytes were present on the peripheral smear. Given the broad differential diagnoses for acute kidney injury, a kidney biopsy was performed and revealed granulomatous tubulo-interstitial nephritis with some acute tubular injury. Based on the biopsy findings, a decision was taken to adjust medications and initiate corticosteroids for presumed medication-induced interstitial nephritis, hemolysis and maculo-papular rash. The kidney function and hemolysis improved over the subsequent days and the patient was discharged to a rehabilitation facility, no-longer required dialysis. Conclusions Acute kidney injury in patients with severe COVID-19 may have multiple causes. We present the first case of granulomatous interstitial nephritis in a patient with COVID-19. Drug-reactions may be more frequent than currently recognized in COVID-19 and are potentially reversible. The kidney biopsy findings in this case led to a change in therapy, which was associated with subsequent patient improvement. Kidney biopsy may therefore have significant value in pulling together a clinical diagnosis, and may impact outcome if a treatable cause is identified.

Proteus syndrome in pregnancy: A case report

2020 ◽  
pp. 1753495X2097079
Author(s):  
Niccole Ranaei-Zamani ◽  
Mandeep K Kaler ◽  
Rehan Khan
Keyword(s):  
Case Report ◽  
Case Reports ◽  
Proteus Syndrome ◽  
Cutaneous Lesions ◽  
Genetic Syndrome ◽  
Skeletal Tissue ◽  
First Case ◽  
Large Teaching Hospital ◽  
Clinical Presentations ◽  
In Pregnancy

Proteus syndrome is a rare, multi-system, genetic syndrome characterised by atypical and excessive growth of skeletal tissue. Clinical presentations include abnormal musculoskeletal growth and cutaneous lesions. Due to its rarity, there have been a limited number of published case reports of Proteus syndrome. This is the first case report on the management of Proteus syndrome in pregnancy. We present the case of a pregnant woman with Proteus syndrome in her first pregnancy in a large teaching hospital and discuss the considerations and challenges faced in her antenatal, intrapartum and postnatal care.

Sign in / Sign up

Export Citation Format

Share Document