Splenic Rupture Secondary to Amyloidosis: A Case Report and Review of the Literature.

Amyloidosis is a term describing the extracellular deposit of fibrils composed of subunits of several different normal serum proteins in various tissues. Amyloid light chain (AL) amyloidosis contains fibrils that are composed of fragments of monoclonal light chains. Many different disorders and conditions can lead to spontaneous splenic rupture, including AL amyloidosis. We present a case of a 64-year-old woman with spontaneous splenic rupture and hemorrhage. A final diagnosis of systemic amyloidosis secondary to plasma cell myeloma was made with infiltrative cardiomyopathy and possible diastolic congestive heart failure exacerbation. We also provide a narrative review of all documented cases of splenic rupture associated with amyloidosis from the year 2000 until January 2023, along with the main clinical findings and management strategies.

Improving documentation of blood product administration using a standardized electronic health record-based system: a single-institution experience.

OBJECTIVES: To improve documentation of blood product administration by assessing the completion status of blood transfusions. In this way, we can ensure compliance with the Association for the Advancement of Blood & Biotherapies standards and facilitate investigation of potential blood transfusion reactions. METHODS: This before-and-after study includes the implementation of an electronic health record (EHR)-based, standardized protocol for documenting the completion of blood product administration. Twenty-four months of retrospective data (January-December 2021) and prospective data (January-December 2022) were collected. Meetings were held before the intervention. Ongoing daily, weekly, and monthly reports were prepared, and targeted education to deficient areas as well as spot in-person audits by the blood bank residents were conducted. RESULTS: During 2022, 8,342 blood products were transfused, of which 6,358 blood product administrations were documented. The overall percentage of completed transfusion order documentation improved from 35.54% (units/units) in 2021 to 76.22% (units/units) in 2022. CONCLUSIONS: Interdisciplinary collaborative efforts helped produce quality audits to improve the documentation of blood product transfusion through a standardized and customized EHR-based blood product administration module.

Squamous cell carcinoma arising in a Zenker diverticulum.

Squamous cell carcinoma (SCC) arising in a Zenker diverticulum (ZD) is an extremely rare entity. Approximately 50 cases have been reported worldwide. We report a case of a 74-year-old man who presented to our institution with chronic regurgitation, dysphagia and halitosis. The patient was initially seen in 2015 at which point he reported a 10-year history of these symptoms and was diagnosed with ZD. A barium swallow was done revealing a large posterior esophageal diverticulum with significant residual contrast within the diverticulum lumen. Given these findings, he was taken for open surgical excision where a SCC was identified. Although it is extremely rare for a SCC to occur in a ZD, patients with ZD must undergo regular surveillance endoscopy of the esophagus and the diverticulum itself to identify any suspicious mass or lesion arising in within.

Heterotopic mesenteric ossification: a report of two cases.

Heterotopic mesenteric ossification (HMO) is abnormal bone formation in tissues which usually do not undergo ossification. There are approximately 75 cases reported worldwide. We present two cases of HMO. The first case is that of a 39-year-old man who presented with abdominal pain and a computerized tomography scan of the abdomen and pelvis revealed an apple core lesion resulting in small bowel obstruction. The second case is that of a 36-year-old woman who presented 2 months after undergoing robotic gastric sleeve resection complaining of weakness and emesis. An esophagogram revealed kinking at the distal esophagus. Surgical resection was performed in both, yielding the diagnosis of HMO. There are various theories as to the pathophysiology of HMO, but no clearly defined mechanism has been established. Management should be conservative whenever possible to prevent further ossification with subsequent surgical intervention.

Papillary cystadenoma of the epididymis.

Background: Papillary cystadenoma is a rare benign neoplasm of the epididymis. It may occur sporadically or in association with von Hippel-Lindau disease (VHLD). Papillary cystadenoma of the epididymis (PCE) is a benign mimic of metastatic clear cell renal cell carcinoma (CCRCC) given their histologic similarities. Case presentation: Herein, we present the case of a 40-year-old man with a four-year history of microhematuria and a recently detected right paratesticular mass. A testicular sonogram revealed a hypoechoic, hypervascular solid mass in the right epididymal head treated by surgical excision. Histopathological examination demonstrated a 1.1 cm papillary cystadenoma of the epididymis. Genetic testing performed later showed no signs of VHLD. However, heterozygous mutations in three genes - CASR, POT1, and RAD51D - were found which have never been reported in PCE before. Conclusions: Papillary cystadenoma of the epididymis should always be considered in the differential diagnosis of epididymal lesions, especially those that are cystic. The mainstay of treatment remains surgical excision, which provides an excellent prognosis.

Papillary cystadenoma of the epididymis

ABSTRACT Background Papillary cystadenoma is a rare benign neoplasm of the epididymis. It may occur sporadically or in association with von Hippel-Lindau disease (VHLD). Papillary cystadenoma of the epididymis (PCE) is a benign mimic of metastatic clear cell renal cell carcinoma (CCRCC) given their histologic similarities. Case presentation Herein, we present the case of a 40-year-old man with a four-year history of microhematuria and a recently detected right paratesticular mass. A testicular sonogram revealed a hypoechoic, hypervascular solid mass in the right epididymal head treated by surgical excision. Histopathological examination demonstrated a 1.1 cm papillary cystadenoma of the epididymis. Genetic testing performed later showed no signs of VHLD. However, heterozygous mutations in three genes - CASR, POT1, and RAD51D - were found which have never been reported in PCE before. Conclusions Papillary cystadenoma of the epididymis should always be considered in the differential diagnosis of epididymal lesions, especially those that are cystic. The mainstay of treatment remains surgical excision, which provides an excellent prognosis.

Rectal endometriosis presenting as toxic megacolon.

BACKGROUND: The bowel is the most common site of extragenital endometriosis, with involvement of the locoregional sigmoid colon and anterior rectum seen most often. The clinical presentation varies depending on how soon patients seek medical care, thus requiring changes in management strategies. Endometriosis can cause a life-threatening surgical emergency with progressive obliteration of the bowel lumen leading to obstruction and late complications including toxic megacolon and transmural necrosis. CASE PRESENTATION: We report the case of a 41-year-old woman presenting with an acute abdomen and complete large bowel obstruction complicated by sepsis and toxic megacolon. The patient underwent emergency total colectomy with ileostomy. Medical history was significant for chronic, vague, and episodic lower abdominal pain self-medicated with herbal tea and laxatives. Pathologic examination demonstrated colonic endometriosis within the bowel wall as the cause of obstruction, ischemia, and transmural necrosis. CONCLUSIONS: Although a rare clinical entity, this case highlights two important points. First, it demonstrates the value of performing proper and complete clinical work up to rule out or in all possible causes of colonic obstruction, including intestinal endometriosis. Second, it suggests a potential benefit of a formalized multidisciplinary approach, including surgery, in the management of medically unresponsive endometriosis. In conclusion, this case shows that endometriosis can cause life-threatening colonic obstruction in women of childbearing age. Prompt early intervention is warranted, particularly when obstruction is only partial and ischemia has not supervened, to conceivably prevent the development of a toxic megacolon requiring colectomy and avoid late complications.

The Utility of Elevated Serum Lactate Dehydrogenase in Current Clinical Practice.

OBJECTIVE: Because of its wide tissue distribution, elevation of serum lactate dehydrogenase (LD) is a nonspecific finding. Although serum LD is still included in the prognosis and staging of metastatic melanoma and germ cell tumors, its nonspecificity has led to decreased usefulness. METHODS: In this study, we analyzed the serum LD assays performed in a 726-bed hospital during a 1-year period and reviewed charts of patients with serum LD of >3 standard deviations (SD). RESULTS: Of 312 patients with elevated serum LD, only 9 were patients with melanoma and germ cell tumors. The other 303 patients had other malignancies, chronic conditions, and sepsis. CONCLUSION: Elevated serum LD (even >3 SD) is an extremely nonspecific finding that does not contribute to clinical management in a majority of patients. As such, serum LD testing should be retired from routine clinical order sets and restricted in use.

Rectal endometriosis presenting as toxic megacolon

Background The bowel is the most common site of extragenital endometriosis, with involvement of the locoregional sigmoid colon and anterior rectum seen most often. The clinical presentation varies depending on how soon patients seek medical care, thus requiring changes in management strategies. Endometriosis can cause a life-threatening surgical emergency with progressive obliteration of the bowel lumen leading to obstruction and late complications including toxic megacolon and transmural necrosis. Case presentation We report the case of a 41-year-old woman presenting with an acute abdomen and complete large bowel obstruction complicated by sepsis and toxic megacolon. The patient underwent emergency total colectomy with ileostomy. Medical history was significant for chronic, vague, and episodic lower abdominal pain self-medicated with herbal tea and laxatives. Pathologic examination demonstrated colonic endometriosis within the bowel wall as the cause of obstruction, ischemia, and transmural necrosis. Conclusions Although a rare clinical entity, this case highlights two important points. First, it demonstrates the value of performing proper and complete clinical work up to rule out or in all possible causes of colonic obstruction, including intestinal endometriosis. Second, it suggests a potential benefit of a formalized multidisciplinary approach, including surgery, in the management of medically unresponsive endometriosis. In conclusion, this case shows that endometriosis can cause life-threatening colonic obstruction in women of childbearing age. Prompt early intervention is warranted, particularly when obstruction is only partial and ischemia has not supervened, to conceivably prevent the development of a toxic megacolon requiring colectomy and avoid late complications.

Nail Biting as a Cause of Appendicitis.

Ingestion of a foreign body is commonly encountered in clinical practice, but most cause no complications, passing spontaneously through the gastrointestinal tract. However, they can cause obstructive signs and symptoms, and surgical intervention for extraction of the foreign body may be required after identifying its location. We present here the case of a 49-year-old woman who presented to our emergency room with abdominal pain localizing to the right lower quadrant. Evaluation was most consistent with acute appendicitis, and she underwent uncomplicated appendectomy. A keratin nail with Actinomyces was identified in her appendix. Foreign bodies in the appendix can cause simple appendicitis, perforation, periappendiceal abscess, and peritonitis. Regardless of etiology, an appendectomy often ends up the primary treatment, but unusual and rare causes are worth noting if only for the clinician to be aware of when evaluating the next patient with abdominal pain and considering treatment options or future prevention. Our case is an example of a rare scenario in which an Actinomyces-contaminated human nail lodged in the appendix of a woman eventually resulting in acute appendicitis.

Evaluation of the expression of P16INK4A by immunohistochemistry in post-neoadjuvant chemotherapy hormone receptor negative breast cancer specimens.

BACKGROUND: Hormone-receptor-negative breast carcinoma (HRNBC), including triple-negative and HER-2 amplified tumors, can overexpress P16INK4a with substantial contribution to tumor progression. In nonneoplastic cells, P16INK4a mediates growth arrest and senescence secondary to cytotoxic compounds. OBJECTIVE: We assessed the impact of neoadjuvant chemotherapy (NAC) on P16INK4a expression in breast specimens. METHODS: P16INK4a and CD-44 were evaluated by immunohistochemistry in biopsies and subsequent post-NAC excision in a cohort of 27 women with HRNBC. Positivity was estimated on hotspots of tissue available by calculating cellular densities in nonneoplastic tissues with a low proliferation rate (Ki-67 < 1%) and tumor percentage using ImageJ 1.51t (National Institutes of Health, USA). RESULTS: Pre-NAC P16INK4a and CD-44 tumor expression were similar between the complete (n = 15) and incomplete (n = 12) response groups. Residual HRNBCs exhibited decreased immunoreactivity for P16INK4a, while the expression of CD-44 increased (n = 10, P < 0.05). The magnitude of change correlated with the baseline expression (r = 0.37, P16; r = -0.85, CD-44). Post-NAC nonneoplastic mammary duct and lobular epithelia, perilobular stroma, and adipose tissue, but not peritumoral stroma, accumulated P16INK4a(+) cells. The post-NAC cellular density change was more significant in epithelia of patients with high P16INK4a(+) baseline (r = 0.86, P < 0.0001) and those with a complete pathologic response (n = 14, P < 0.05). All tumors beds with complete treatment effect showed diffuse P16INK4a positivity. CONCLUSION: NAC induced the accumulation of P16INK4a(+)cells in nonneoplastic breast tissues more pronounced in patients with a complete pathologic response. Therapy-induced senescence is a potential marker of bystander damage due to NAC. P16INK4a loss and CD-44 gain may represent a phenotype of chemoresistance in residual HRNBCs.

Fatal Sickling Triggered by Massive Foreign Particle Embolism: A Case Report of Unrecognized Indwelling Venous Catheter Drug Abuse in Sickle Cell Disease.

Sickle cell disease (SCD) manifests itself with vaso-occlusive episodes leading to infarction. Placement of intravascular catheters provides a useful route for management of pain crises as well as other complications. However, catheter misuse is a commonly unrecognized problem, which can have lethal consequences. We present a case of fatal splenic sequestration/hyperhemolysis secondary to foreign body pulmonary and systemic embolization due to intravenous administration of hydromorphone pills in a young woman with SCD. Morphological identification of the embolized particles (crospovidone, microcrystalline cellulose, and hydrophilic polymer) was key in understanding the mechanism of death. Foreign particle deposition induced a granulomatous inflammatory response that resulted in obliteration and distortion of the microvasculature, thus triggering sickling with subsequent organ damage. Despite clinical evidence of asplenia and microscopic white pulp fibrosis with Gamna-Gandy body formation, unexpectedly for the patient's age, the spleen was enlarged by entrapped sickle cells. The findings support the possibility that loss of function can coexist with increased splenic activity under certain circumstances, an overlooked clinical phenotype of SCD. This case illustrates the teaching value of classic autopsy in addition to its crucial role in understanding the natural history of complex heterogeneous diseases.

Is serum lactate necessary in patients with normal anion gap and serum bicarbonate?

INTRODUCTION: There has been an increase in patients having serum lactate drawn in emergency situations. The objective of this study was to determine whether or not it was necessary to obtain a lactate level in patients with a normal serum bicarbonate level and anion gap. METHODS: This is a retrospective chart review evaluation of 304 patients who had serum lactate and electrolytes measured in an emergency setting in one academic medical center. RESULTS: In 66 patients who had elevated serum lactate (>2.2mmol/L), 45 (68%) patients had normal serum bicarbonate (SB) (greater than 21 mmol/L). Normal anion gap (AG) (normal range <16 mEq/l) was found in 51 of the 66 patients (77%). CONCLUSION: We found that among patients with elevated serum lactate, 77% had a normal anion gap and 68% had normal serum bicarbonate. We conclude serum lactate should be drawn based on clinical suspicion of anaerobic tissue metabolism independent of serum bicarbonate or anion gap values.

Reducing blood utilization by implementation of a type-and-screen transfusion policy a single-institution experience.

OBJECTIVES: The Blood Utilization Committee implemented a standardized protocol for the preoperative blood order for cardiac patients. The aim of our study was to assess the improvement in blood utilization using the crossmatch to transfusion ratio (C:T). METHODS: Four months of retrospective data were collected, which included all RBC crossmatch requests and all RBC units transfused. Similar data were gathered for the period of the intervention. The difference in C:T was calculated. RESULTS: The retrospective group had 166 patients for whom blood products were ordered. There were 560 crossmatch requests and 237 transfused RBC units with a C:T of 2.36. The prospective group had 127 patients with 297 crossmatch requests, 190 transfused units, and a C:T of 1.56. There was a statistically significant difference in the C:T. The cost difference was $12,244.00. CONCLUSIONS: Implementing exact guidelines, with the introduction of a type-and-screen concept, allowed more efficient blood usage.

Colouterine fistula secondary to endometriosis with associated chorioamnionitis.

BACKGROUND: Intestinal endometriosis may be complicated by bowel obstruction, colonic rupture, sepsis, and rarely, malignant transformation. Fistula formation is extremely rare. CASE: A 26-year-old woman presented at 16 weeks of gestation with an acute abdomen suggestive of ruptured appendicitis. Blood cultures were positive for Bacteroides fragilis. At laparotomy, she was found to have a colouterine fistula with pelvic sepsis. The resected specimens demonstrated extensive uterine adenomyosis and endometriosis of the cecum, with a fistulous tract lined by endometriosis and suppurative inflammation extending from the cecum to the uterine endometrial cavity associated with severe chorioamnionitis and endomyometritis. CONCLUSION: This case illustrates a rare complication of colouterine fistula secondary to intestinal endometriosis.

Small intestinal angiosarcoma leading to perforation and acute abdomen: a case report and review of the literature.

A 76-year-old man presented to the emergency room with abdominal pain and fatigue. The patient had a history of gastrointestinal bleeding. An abdominal computed tomographic scan showed collections of free air in the abdomen with obstruction of the distal small intestine and multiple masses in the liver. Laparotomy revealed a widespread malignant neoplasm in the abdomen, with multiple tumor nodules in the ileal wall, mesentery, and liver. The ileal wall was perforated within the area of one of the tumors. Pathologic examination of the lesion showed a neoplasm composed of solid sheets of spindle and undifferentiated cells, forming interlacing delicate vascular channels with atypical endothelial cells. The tumor cells were positive for the endothelial marker CD31, whereas reactivity for a broad panel of epithelial and other endothelial markers was negative. This case demonstrates that although angiosarcomas of the gastrointestinal tract are rare, they should be considered in cases of intestinal perforation or severe bleeding, especially in the elderly. A broad panel of immunochemical markers might be necessary to establish the pathologic diagnosis.