Anesthetic management of a parturient with VACTERL association undergoing Cesarean delivery.

PURPOSE: We present the anesthetic management of a parturient with VACTERL association undergoing combined regional and general anesthesia for Cesarean delivery. Defined as a syndrome, VACTERL association comprises at least three of the following abnormalities: vertebral, anal atresia, cardiac, tracheoesophageal, renal, and limb. CLINICAL FEATURES: The patient's anatomic abnormalities and comorbidities comprised severe cervicothoracic scoliosis, kyphoscoliosis, congenitally fused ribs, and severe restrictive lung disease. She had a Mallampati class 3 airway, a right laterally flexed neck, and reduced mandibular protrusion. We performed a lumbar spine ultrasound for epidural placement which was used to provide peri- and postoperative analgesia. Due to the anticipated difficult tracheal intubation, the patient underwent an awake fibreoptic intubation and subsequently received general anesthesia. The patient's trachea was extubated on the first postoperative day, and she received adequate post-Cesarean epidural analgesia. CONCLUSION: This case highlights the challenges that anesthesiologists face when managing parturients at extremely high risk for perioperative anesthetic morbidity due to the presence of severe pre-existing disease, anticipated difficult airway, and major spinal abnormalities complicating neuraxial anesthesia. We used a combined general and epidural anesthetic approach to control ventilation, provide effective postoperative analgesia, and reduce the risk of anesthetic-related perioperative morbidity. An individualized approach should be considered for the anesthetic management of high-risk pregnant patients with complex and multiple medical and surgical morbidities undergoing labour and delivery.

Pheochromocytoma crisis precipitated by dexamethasone with profound lactic acidosis, but without severe hypertension.

Summary: We describe a case of a 47-year-old patient who presented with severe lactic acidosis, troponinemia, and acute kidney injury after receiving 8 mg of intramuscular dexamethasone for seasonal allergies in the setting of an undiagnosed epinephrine-secreting pheochromocytoma. This case was atypical, however, in that the patient exhibited only mildly elevated noninvasive measured blood pressures. Following a period of alpha-adrenergic blockade, the tumor was resected successfully. Steroid administration can precipitate pheochromocytoma crisis that may present unusually as in our patient with mild hypertension but profound lactic acidosis. Learning points: Steroids administered via any route can precipitate pheochromocytoma crisis, manifested by excessive catecholamine secretion and associated sequelae from vasoconstriction. Lack of moderate/severe hypertension on presentation detracts from consideration of pheochromocytoma as a diagnosis. Lactatemia after steroid administration should prompt work-up for pheochromocytoma, as it can be seen in epinephrine-secreting tumors. Noninvasive blood pressure measurements may be unreliable during pheochromocytoma crisis due to excessive peripheral vasoconstriction.

A case report of an open aortic valve replacement followed by open adrenalectomy in a patient with symptomatic pheochromocytoma and critical aortic stenosis.

BACKGROUND: Pheochromocytoma is a rare medical condition caused by catecholamine-secreting tumor cells. Operative resection can be associated with significant hemodynamic fluctuations due to the nature of the tumor, as well as associated post-resection vasoplegia. To allow for cardiovascular recovery before surgery, patients require pre-operative alpha-adrenergic blockade, which would be limited in the setting of co-existent severe aortic stenosis. In this report, we describe a patient with severe aortic stenosis and symptomatic pheochromocytoma. CASE PRESENTATION: A 51-year-old man with severe aortic stenosis (valve area 0.8 cm2) was found to have a highly active 4 × 4 cm left adrenal pheochromocytoma. Alpha-adrenergic blockade for his pheochromocytoma was limited by syncope in the setting of his aortic stenosis. Open aortic valve replacement (AVR) was performed, followed by adrenalectomy the next day. The perioperative course for each surgical procedure was hemodynamically volatile, exacerbated by severe alcohol withdrawal. During the adrenalectomy, cardiogenic and vasoplegic shock developed immediately after securing the vascular supply to his tumor. This shock was refractory to vasopressin and methylene blue, but responded well to angiotensin II and epinephrine. After both surgeries were completed, his course was further complicated by severe ICU psychosis, ileus, fungal bacteremia, pneumonia/hypoxic respiratory failure and atrial fibrillation. He ultimately recovered and was discharged from the hospital after 38 days. CONCLUSION: To our knowledge, this is the first report of surgical AVR and pheochromocytoma resection in a patient with critical aortic stenosis. The appropriate order and timing of surgeries when both these conditions co-exist remains controversial.

Combined endovascular and open operative approach for mycotic carotid aneurysm.

Mycotic aneurysms of the extracranial carotid artery are rare and warrant surgical intervention. Management involves open and endovascular approaches. We report the case of a 67-year-old woman with an Escherichia coli soft-tissue infection of the right retropharyngeal space and subsequent mycotic carotid aneurysm and thrombosis of the internal jugular vein. The patient presented with a pulsatile mass and right middle cerebral artery stroke. Our surgical management involved coil embolization of the aneurysm to provide for vascular control, with resection of the common carotid artery, internal carotid artery, and extracranial carotid artery branches, along with the internal jugular vein.

Catecholamine-induced cerebral vasospasm and multifocal infarctions in pheochromocytoma.

SUMMARY: We report the case of a 76-year-old male with a remote history of papillary thyroid cancer who developed severe paroxysmal headaches in the setting of episodic hypertension. Brain imaging revealed multiple lesions, initially of inconclusive etiology, but suspicious for metastatic foci. A search for the primary malignancy revealed an adrenal tumor, and biochemical testing confirmed the diagnosis of a norepinephrine-secreting pheochromocytoma. Serial imaging demonstrated multiple cerebral infarctions of varying ages, evidence of vessel narrowing and irregularities in the anterior and posterior circulations, and hypoperfusion in watershed areas. An exhaustive work-up for other etiologies of stroke including thromboembolic causes or vasculitis was unremarkable. There was resolution of symptoms, absence of new infarctions, and improvement in vessel caliber after adequate alpha-adrenergic receptor blockade for the management of pheochromocytoma. This clinicoradiologic constellation of findings suggested that the etiology of the multiple infarctions was reversible cerebral vasoconstriction syndrome (RCVS). Pheochromocytoma remains a poorly recognized cause of RCVS. Unexplained multifocal cerebral infarctions in the setting of severe hypertension should prompt the consideration of a vasoactive tumor as the driver of cerebrovascular dysfunction. A missed or delayed diagnosis has the potential for serious neurologic morbidity for an otherwise treatable condition. LEARNING POINTS: The constellation of multifocal watershed cerebral infarctions of uncertain etiology in a patient with malignant hypertension should trigger the consideration of undiagnosed catecholamine secreting tumors, such as pheochromocytomas and paragangliomas. Reversible cerebral vasoconstriction syndrome is a serious but reversible cerebrovascular manifestation of pheochromocytomas that may lead to strokes (ischemic and hemorrhagic), seizures, and cerebral edema. Alpha-adrenergic receptor blockade can reverse cerebral vasoconstriction and prevent further cerebral ischemia and infarctions. Early diagnosis of catecholamine secreting tumors has the potential for reducing neurologic morbidity and mortality in patients presenting with cerebrovascular complications.

Another Role for Angiotensin II?: Vasopressin-Refractory Shock After Pheochromocytoma Resection: A Case Report.

A patient presented with multiple unrelated tumors and was found to have a small but functional adrenal pheochromocytoma. After pheochromocytoma resection, shock developed unresponsive to vasopressin in recommended doses (0.04 U/min infusion plus repeated 1-U boluses) but responded dramatically to an angiotensin II infusion (20 ng/kg/min) with a mean arterial pressure >100 mm Hg. The patient's blood pressure was maintained for 42 hours postoperatively with an infusion rate that ranged from 2 to 38 ng/kg/min. Because vasopressin may not always be effective for postresection shock in people with pheochromocytomas, angiotensin II may prove to be an effective alternative.

Retrospective Analysis of Peri-Intubation Hypoxemia During the Coronavirus Disease 2019 Epidemic Using a Protocol for Modified Airway Management.

This single-center retrospective study evaluated a protocol for the intubation of patients with confirmed or suspected coronavirus disease 2019 (COVID-19). Twenty-one patients were intubated, 9 of whom were found to have COVID-19. Adherence to the airway management protocol was high. COVID-19 patients had lower peripheral capillary oxygen saturation by pulse oximetry (Spo2) nadirs during intubation (Spo2, 73% [72%-77%] vs 89% [86%-94%], P = .024), and a greater percentage experienced severe hypoxemia defined as Spo2 ≤80% (89% vs 25%, P = .008). The incidence of severe hypoxemia in COVID-19 patients should be considered in the development of guidelines that incorporate high-flow nasal cannula and noninvasive positive pressure ventilation.

A hemodynamic profile for consciousness during cardiopulmonary resuscitation.

We report the quantification of a hemodynamic profile sufficient to support consciousness during cardiopulmonary resuscitation. A 62-yr-old man experienced cardiac arrest while being evaluated for heart failure after heart transplantation. During the emergency, hemodynamic data were obtained from bedside monitors and reviewed at regular intervals. His mean arterial blood pressure and heart rate were correlated with consciousness during cardiopulmonary resuscitation. A mean arterial blood pressure of 50 mm Hg with a heart rate of 100 bpm supported consciousness during cardiac arrest. This case helps to validate the recent emphasis on hard, fast, basic life support.

Needle-guided ultrasound technique for axillary artery catheter placement in critically ill patients: A case series and technique description.

PURPOSE: Axillary arterial cannulation for blood pressure monitoring has been reported in adults since 1973. Reported failure rates using palpation landmarks are high. This report describes a needle-guided ultrasound technique for axillary arterial line placement in critically ill patients. METHODS: A retrospective review of all patients requiring axillary arterial cannulation attempts with ultrasound-assisted needle guidance for hemodynamic monitoring was performed from July 2010 to June 2016 at a single institution. RESULTS: One hundred fifty nine (159) cannulation attempts were performed in 155 patients. The overall success rate was 97%, with a first pass success rate of 84%. Inexperienced operators performed 49% of procedures under direct faculty supervision, and had a 99% success rate, which was not different from experienced operators. Almost 20% of patients had moderate-to-severe coagulopathy (platelets<50k/uL, INR>2.0 or PTT>60s). Complications reported included the following: nonfunctioning of catheter (6%) and hematoma (6%). Ischemia was noted in 2 patients (1%), but only one was attributed to the arterial catheter. CONCLUSIONS: Use of the needle-guided ultrasound assisted approach for axillary arterial line placement is easily teachable and can be used to promote safe and successful placement of axillary arterial lines for novice learners.

Transcription restores DNA repair to heterochromatin, determining regional mutation rates in cancer genomes.

Somatic mutations in cancer are more frequent in heterochromatic and late-replicating regions of the genome. We report that regional disparities in mutation density are virtually abolished within transcriptionally silent genomic regions of cutaneous squamous cell carcinomas (cSCCs) arising in an XPC(-/-) background. XPC(-/-) cells lack global genome nucleotide excision repair (GG-NER), thus establishing differential access of DNA repair machinery within chromatin-rich regions of the genome as the primary cause for the regional disparity. Strikingly, we find that increasing levels of transcription reduce mutation prevalence on both strands of gene bodies embedded within H3K9me3-dense regions, and only to those levels observed in H3K9me3-sparse regions, also in an XPC-dependent manner. Therefore, transcription appears to reduce mutation prevalence specifically by relieving the constraints imposed by chromatin structure on DNA repair. We model this relationship among transcription, chromatin state, and DNA repair, revealing a new, personalized determinant of cancer risk.

Propofol infusion syndrome resuscitation with extracorporeal life support: a case report and review of the literature.

We report a case of propofol infusion syndrome (PRIS) in a young female treated for status epilepticus. In this case, PRIS rapidly evolved to full cardiovascular collapse despite aggressive supportive care in the intensive care unit, as well as prompt discontinuation of the offending agent. She progressed to refractory cardiac arrest requiring emergent initiation of venoarterial extracorporeal membrane oxygenation (ECMO) during cardiopulmonary resuscitation (CPR). She regained a perfusing rhythm after prolonged (>8 hours) asystole, was weaned off ECMO and eventually all life support, and was discharged to home. We also present a review of the available literature on the use of ECMO for PRIS.

The effects of respiratory failure on delivery in pregnant patients with H1N1 2009 influenza.

BACKGROUND: The majority of hospitalizations for H1N1 complications have been in people with high-risk comorbidities, including pregnancy. Here we describe the obstetric and critical care treatment of three patients with confirmed H1N1 influenza virus infection complicated by acute respiratory failure. CASES: We describe the clinical and therapeutic courses of three patients with confirmed H1N1 2009 influenza virus infection complicating singleton, twin, and triplet gestations, each of which were complicated by respiratory failure. CONCLUSION: These three cases illustrate that a high index of suspicion, prompt treatment, timing and mode of delivery considerations, and interdisciplinary treatment are integral to the care of pregnant patients with H1N1 influenza infections complicated by acute respiratory failure.

Catecholamine-secreting paragangliomas: recent progress in diagnosis and perioperative management.

Catecholamine-secreting paragangliomas (CSPs) present challenges for the managing team of surgeons and anesthesiologists. Without proper preoperative management and planning, the patient is at high risk for complications and significant morbidity. A review of the literature looking at all aspects of the care of patients with CSP was performed to provide a consensus on the comprehensive care of these difficult patients. A case study is also provided to illustrate the management algorithm. Specific recommendations are made with regards to preoperative workup, including serum and urine testing, tumor localization, angiography, and embolization. Preoperative and intraoperative management techniques by the surgical and anesthesiology teams are discussed, including pharmaceutical interventions and fluid management. Aspects of postoperative care are also discussed. Management of patients with CSP requires significant attention to detail by a multidisciplinary team of surgeons and anesthesiologists. By following the recommendations included within this article, the morbidity associated with removal of these tumors can be significantly reduced or eliminated.