RESUMO
Localized cystic kidney disease (LCKD) is a distinct renal disorder characterized by the presence of cysts within specific regions of the kidneys. We present a rare case of a 41-year-old African American man, who presented to our medical center with lower urinary tract symptoms and gross hematuria. The initial assessment culminated in the identification of an uncomplicated urinary tract infection, prompting the prescription of appropriate oral antibiotic therapy. On follow-up after 5 months, the patient presented with gross hematuria. Imaging studies revealed a mixed-density cystic lesion of 2.6 cm situated within the interpolar region of the right kidney. This cystic lesion exhibited intricate septations at the superior pole of the kidney. Robotic-assisted right partial nephrectomy was performed, and pathologic examination was diagnostic for LCKD. This report not only underscores the uniqueness of LCKD but also presents a comprehensive review of the existing literature that pertains to this condition. Particular emphasis is placed upon its inherent benign behavior and its marked divergence from the progressive trajectory commonly associated with other renal diseases. We also explored the incidental findings of the disease, its diverse clinical symptomatology, conceivable etiological underpinnings, and the array of diagnostic modalities used. Finally, similarities in histopathologic findings with polycystic kidney disease and other entities are discussed, underscoring the importance of accurate diagnosis and management.
RESUMO
ABSTRACT Localized cystic kidney disease (LCKD) is a distinct renal disorder characterized by the presence of cysts within specific regions of the kidneys. We present a rare case of a 41-year-old African American man, who presented to our medical center with lower urinary tract symptoms and gross hematuria. The initial assessment culminated in the identification of an uncomplicated urinary tract infection, prompting the prescription of appropriate oral antibiotic therapy. On follow-up after 5 months, the patient presented with gross hematuria. Imaging studies revealed a mixed-density cystic lesion of 2.6 cm situated within the interpolar region of the right kidney. This cystic lesion exhibited intricate septations at the superior pole of the kidney. Robotic-assisted right partial nephrectomy was performed, and pathologic examination was diagnostic for LCKD. This report not only underscores the uniqueness of LCKD but also presents a comprehensive review of the existing literature that pertains to this condition. Particular emphasis is placed upon its inherent benign behavior and its marked divergence from the progressive trajectory commonly associated with other renal diseases. We also explored the incidental findings of the disease, its diverse clinical symptomatology, conceivable etiological underpinnings, and the array of diagnostic modalities used. Finally, similarities in histopathologic findings with polycystic kidney disease and other entities are discussed, underscoring the importance of accurate diagnosis and management.
RESUMO
Small intestinal volvulus (SBV) is the abnormal twisting of bowel around the axis of its mesentery, leading to obstruction and vascular compromise, resulting in bowel ischemia and necrosis which are life-threatening. Risk factors include malformation, malrotation, and adhesions. Its rare incidence and vague clinical presentation make it a difficult diagnosis, more so in a nonverbal patient who cannot express his pain, which is the first and most prominent symptom. Studies suggest an increased frequency of intestinal obstruction in cerebral palsy patients. There are no reported cases of small intestinal volvulus in association with cerebral palsy. We present a case of a 21-year-old man with severe cerebral palsy and kyphoscoliosis. The patient presented to the emergency room with respiratory distress and abdominal distension. An acute abdomen was noted. Abdominal X-rays revealed gas patterns suggestive of small intestinal obstruction. The patient rapidly deteriorated, and resuscitation attempts were unsuccessful. Autopsy revealed peritoneal cavity filled with extensively dilated and thin-walled loops of small intestine. Twisting of the small intestine, showing 360° rotation around the mesenteric root in a clockwise manner at two separate sites, was noted. On bowel dissection, mucosal folds were absent, and mucosa was green with patchy areas of hemorrhage consistent with ischemic necrosis. There was no evidence of any malformation, malrotation or adhesions. Small intestinal volvulus is a rare entity with a nonspecific clinical presentation that poses a diagnostic challenge. This autopsy highlights the need to maintain a high index of suspicion for small intestinal volvulus in cases of bowel obstruction in cerebral palsy patients to expedite surgery and prevent mortality. The primary caregivers of non-verbal cerebral palsy patients living outside of healthcare facilities need to be trained in recognition of life-threatening medical emergencies such as gastrointestinal obstruction and seek emergent attention at the earliest to prevent treatment delays.