8 research outputs found

    PRKAR positive angiomyxoma

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    Carney complex is a rare multiple neoplasia syndrome characterized by spotty pigmented lesions of the skin, cardiac and non-cardiac myxomas, as well as multiple endocrine tumors. The disease is typically associated with inactivating mutations or deletions of the PRKAR1A gene on chromosome. We report the case of a 15-year-old male who presented with a right sided thigh mass found to be myxomatous in origin. Whole genome chromosome SNP microarray analysis of this mass revealed a deletion of the PRKAR1A gene characteristic of the Carney complex. However, a microarray analysis of the patient's blood did not demonstrate any deletion of the PRKAR1A gene. Furthermore, physical examination and echocardiogram did not locate any other myxomatous lesions. Patients with unusual presentations of Carney complex should undergo close monitoring to identify the development of other lesions characteristic of this syndrome, despite the lack of any gene mutation in the somatic cells. Keywords: Angiomyxoma, Carney complex, PRKAR1A gen

    Pediatric dog bite with peritoneal violation

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    While dog bites are common in pediatric trauma centers, peritoneal violation from a dog bite is an uncommon finding given the significant bite force required to traverse the abdominal wall musculature. A very high incidence of clinical suspicion is necessary in order to avoid missing this rare, potentially life-threatening injury. In this instance, we report a case of peritoneal violation, retroperitoneal hematoma, colonic contusion, and mesenteric injury in a four year old male suffering from a dog attack in which computed axial tomography (CT) scanning was key in identifying the injuries. We also review the natural history (and injury patterns) of canine attacks in the United States. Keywords: Pediatric, Trauma, Bites and sting

    Solid pseudopapillary neoplasm of the pancreas (Frantz tumor) in a 14-year-old girl

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    We report the case of a 14-year old female who presented with a two day history of abdominal pain. A contrast enhanced computed tomography (CT) of the abdomen and pelvis demonstrated a characteristic solid-cystic mass in the pancreatic tail which was concerning for a solid pseudopapillary neoplasm (SPN) of the pancreas. The patient underwent an uneventful laparoscopic subtotal pancreatectomy with splenic preservation. We have shown that laparoscopic pancreatic resection with splenic preservation can be performed safely for SPN in a child. We also used two techniques that are useful to accomplish this safely- [1] lateral to medial dissection which assists in the identification of the splenic vein and artery, and [2] slow staple compression of the pancreas which may decrease pancreatic leak rates. Keywords: Solid pseudopapillary neoplasm of the pancreas, Frantz tumor, Pancreatic tumor, Laparoscopy pancreatectomy, Splenic preservatio

    Pain Control in a Pediatric Patient With Sickle Cell Disease Using Regional Nerve Blocks for Laparoscopic Cholecystectomy: A Case Report.

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    The sickle cell patient population continues to provide challenges in pain control. Current therapies include narcotic usage with adjuvant therapies such as anti-inflammatories and nonpharmacological interventions. Poor pain management in the sickle cell patient population, especially postoperatively, can lead to hypoventilation, escalating opioid requirements, poor recovery, and longer hospital stays. This case report addresses a novel addition of ultrasound-guided paravertebral and rectus sheath blocks postinduction of general anesthesia and before surgical incision to assist with the intravenous postoperative pain management regimen after laparoscopic cholecystectomy in a 10-year-old boy with sickle cell disease
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