Arterial Stiffness in a Toddler with Neurofibromatosis Type 1 and Refractory Hypertension

Arterial hypertension is a common finding in patients with neurofibromatosis (NF) type 1. Renovascular hypertension due to renal artery stenosis or midaortic syndrome could be the underlying cause. We report the case of a 4-year-old girl with NF type 1 and midaortic syndrome whose changes in blood pressure and pulse wave velocity suggested the evolution of vasculopathy, diagnosis of renovascular hypertension, and provided insights of response to treatment. Hypertension persisted after percutaneous transluminal angioplasty in the abdominal aorta, requiring escalation of antihypertensive treatment, while arterial stiffness demonstrated a mild decrease. Regular assessment of blood pressure using ambulatory blood pressure monitoring and noninvasive assessment of arterial stiffness may enhance the medical care of patients with NF type 1.

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Non-operative management of isolated spontaneous superior mesenteric artery dissection

Abstract

Isolated spontaneous superior mesenteric artery (SMA) dissection is a rare differential for patients presenting with abdominal pain. Due to limited cases reported, management strategies have been poorly defined. We present the case of a 49-year-old male with history of hypertension and ischemic colitis, presenting with abdominal pain. CT imaging demonstrated a thrombosed dissection of the SMA extending into second and third order braches. He was managed conservatively with therapeutic anticoagulation. His symptoms improved and upon discharge he was transitioned to aspirin and warfarin. Repeat CT imaging continued to show the dissection with resolution of the SMA thrombus. Spontaneous SMA dissection is exceedingly rare with no universally agreed upon standard of care for treatment. Operative intervention should be reserved for failed conservative management or vascular compromise. Understanding the current treatment options helps ensure a favorable patient outcome.

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Malignant proliferating trichilemmal tumor of the forearm: a case report of an unusual location of a rare cutaneous adnexal tumor

Abstract

Proliferating trichilemmal tumor (PTT) is a rare cutaneous adnexal neoplasm of the hair follicle that undergoes outer root sheath differentiation in the isthmus. Histological hallmarks include trichilemmal keratinization, lack of granular layer and lobular proliferation of squamous epithelium with glycogenated clear cells. It affects predominantly elderly women, especially over the scalp. In some cases, malignant transformation can occur. However, only a few cases are reported in the literature. We hereby describe the case of a patient diagnosed with malignant PTT (MPTT) in the dorsal forearm, 2 years after undergoing surgical excision of a squamous cell carcinoma in the same topography. Thus, providing a thorough description of the clinical presentation of MPTT will assist surgeons in diagnosing and treating this rare tumor.

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Resection of a mature cystic teratoma of the liver harboring a carcinoid tumor

Abstract

Cystic teratomas are rare pluripotent embryonic tumors which most commonly originate in gonadal organs. Extra-gonadal cystic teratomas are exceedingly uncommon, accounting for only 1% of all cystic teratomas, and have been reported in unusual locations including the kidney, mediastinum and liver. These extra-ovarian cystic teratomas have also been known to harbor other neoplasms including carcinoid tumors. In this report, we describe a unique case of a hepatic cystic teratoma occurring as a combined tumor with a carcinoid in a young female. The patient underwent elective laparoscopic resection of her tumor after extensive radiographic and endoscopic work-up for chronic, non-localizable abdominal pain. We believe the carcinoid tumor arose de novofrom committed differentiation of a cell line within the teratoma, and not metastatic spread.

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Decompressive laparotomy for a patient on VA-ECMO for massive pulmonary embolism that suffered traumatic liver laceration after mechanical CPR

Abstract

Massive pulmonary embolism (PE) is an embolus sufficiently obstructing pulmonary blood flow to cause right ventricular (RV) failure and hemodynamic instability. We have utilized veno-arterial extracorporeal membrane oxygenation (VA-ECMO) for early and aggressive intervention for massive PE patients. We present a case of a 61-year-old female placed on VA-ECMO for a massive PE while presenting in cardiac arrest and receiving mechanical cardiopulmonary resuscitation (CPR) via the LUCAS 2.0 device (Physio-Control Inc., Lund, Sweden). The patient suffered a severe liver laceration secondary to mechanical CPR and required a decompressive laparotomy. This case highlights that mechanical CPR during other interventions can lead to malposition of the device and could result in solid organ injury.

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Rhabdomyolysis-Induced Acute Kidney Injury in Diabetic Emergency: Underdiagnosed and an Important Association to Be Aware of

Rhabdomyolysis is a potentially life-threatening clinical syndrome associated with muscle injury which can cause a leakage of intracellular contents, manifested from the range of being asymptomatic to a life-threatening condition causing acute kidney injury and severe electrolyte abnormalities. Rhabdomyolysis has been associated with both diabetic ketoacidosis (DKA) and hyperosmolar hyperglycemic nonketotic syndrome, though there is an increased association with rhabdomyolysis and acute kidney injury with hyperosmolar nonketonic state compared with patients with diabetic ketoacidosis. Common clinical manifestations are muscle pain, dark urine, and generalized weakness. The causes of rhabdomyolysis are broadly categorized into three groups: traumatic, nontraumatic exertional, and nontraumatic nonexertional. Here, we present a case of rhabdomyolysis-induced acute kidney injury in a patient with hyperosmolar hyperglycemic state. The patient was discharged on insulin and needed intermittent dialysis for two months. Our case highlights the importance of the rare association of rhabdomyolysis causing acute kidney injury in a diabetic emergency.

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Multiple myeloma presenting as cutaneous leukocytoclastic vasculitis and eosinophilia disclosing a T helper type 1/T helper type 2 imbalance: a case report

Multiple myeloma is a very heterogeneous disease comprising a number of genetic entities that differ from each other in their evolution, mode of presentation, response to therapy, and prognosis. Due to its mor...

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