Anesthesia in children with osteogenesis imperfecta: Retrospective chart review of 83 patients and 205 anesthetics over 7 years.

BACKGROUND: Osteogenesis imperfecta is the collective term for a heterogeneous group of connective tissue syndromes characterized by bone fragility with multisystem involvement and perioperative implications. AIMS: Literature review of anesthetic management of patients with osteogenesis imperfecta revealed a paucity of data on the incidence of perioperative challenges. We sought to determine the rates of these challenges in our study cohort. METHODS: Data were collected in a specialty orthopedic hospital from 2008 to 2015 for 83 osteogenesis imperfecta patients undergoing 205 surgeries: 203 orthopedic surgeries and 2 mid-face reconstructive surgeries. Airway management, intravenous access, surgical blood loss, use of peripheral nerve blockade and/or neuraxial techniques, presence of perioperative fracture, and peak intraoperative temperature were evaluated and analyzed. RESULTS: Difficult airway was encountered in 3/205 (1.5%) cases and perioperative fracture in 2/205 (1%) cases. Neuraxial anesthesia was attempted in 64/205 cases with an 87.5% success rate. All peripheral nerve block attempts (33/205 cases) were successful. Difficult intravenous catheter placement was noted in 8/205 (4%) cases. Estimated blood loss >10% of estimated blood volume was considered significant, and occurred in 35/205 (17%) cases. Significant blood loss occurred more often in severe osteogenesis imperfecta types: 18/76 (23.7%) in Type III and 11/65 (16.9%) in Type IV, whereas only 4/47 (8.5%) occurred in mild Type I. In our 205 case cohort, osteogenesis imperfecta Type III had 5.6 times the odds [(95% CI = 1.8-17.2) P = 0.003] of having an anesthetic complication as compared to osteogenesis imperfecta Type I. CONCLUSION: Patients with osteogenesis imperfecta undergo frequent anesthetic exposures, but anesthetic challenges in our series were uncommon. Odds of challenges are greater in severe osteogenesis imperfecta Type III, with significant blood loss and difficulty placing intravenous catheters more likely encountered in the more severe types.

The Biomechanical Effects of Resuscitation Colloids on the Compromised Lung Endothelial Glycocalyx.

BACKGROUND: The endothelial glycocalyx is an important component of the vascular permeability barrier, forming a scaffold that allows serum proteins to create a gel-like layer on the endothelial surface and transmitting mechanosensing and mechanotransduction information that influences permeability. During acute inflammation, the glycocalyx is degraded, changing how it interacts with serum proteins and colloids used during resuscitation and altering its barrier properties and biomechanical characteristics. We quantified changes in the biomechanical properties of lung endothelial glycocalyx during control conditions and after degradation by hyaluronidase using biophysical techniques that can probe mechanics at (1) the aqueous/glycocalyx interface and (2) inside the glycocalyx. Our goal was to discern the location-specific effects of albumin and hydroxyethyl starch (HES) on glycocalyx function. METHODS: The effects of albumin and HES on the mechanical properties of bovine lung endothelial glycocalyx were studied using a combination of atomic force microscopy and reflectance interference contrast microscopy. Logistic regression was used to determine the odds ratios for comparing the effects of varying concentrations of albumin and HES on the glycocalyx with and without hyaluronidase. RESULTS: Atomic force microscopy measurements demonstrated that both 0.1% and 4% albumin increased the thickness and reduced the stiffness of glycocalyx when compared with 1% albumin. The effect of HES on glycocalyx thickness was similar to albumin, with thickness increasing significantly between 0.1% and 1% HES and a trend toward a softer glycocalyx at 4% HES. Reflectance interference contrast microscopy revealed a concentration-dependent softening of the glycocalyx in the presence of albumin, but a concentration-dependent increase in stiffness with HES. After glycocalyx degradation with hyaluronidase, stiffness was increased only at 4% albumin and 1% HES. CONCLUSIONS: Albumin and HES induced markedly different effects on glycocalyx mechanics and had notably different effects after glycocalyx degradation by hyaluronidase. We conclude that HES is not comparable with albumin for studies of vascular permeability and glycocalyx-dependent signaling. Characterizing the molecular and biomechanical effects of resuscitation colloids on the glycocalyx should clarify their indicated uses and permit a better understanding of how HES and albumin affect vascular function.

Pharmacologic blockade of vascular adhesion protein-1 lessens neurologic dysfunction in rats subjected to subarachnoid hemorrhage.

Aneurysmal subarachnoid hemorrhage (SAH) is a potentially devastating clinical problem. Despite advances in the diagnosis and treatment of SAH, outcome remains unfavorable. An increased inflammatory state, one that is characterized by enhanced leukocyte trafficking has been reported to contribute to neuronal injury in association with multiple brain insults, including hemorrhagic and ischemic stroke. This study was designed to investigate, in rats, the neuropathologic consequences of heightened leukocyte trafficking following SAH, induced via endovascular perforation of the anterior cerebral artery. Experiments focused on the initial 48 h post-SAH and sought to establish whether blockade of vascular adhesion protein-1 (VAP-1), with LJP-1586, was able to provide dose-dependent neuroprotection. Treatment with LJP-1586 was initiated at 6h post-SAH. An intravital microscopy and closed cranial window system, that permitted examination of temporal patterns of rhodamine-6G-labeled leukocyte adhesion/extravasation, was used. Effects of LJP-1586 on neurologic outcomes and leukocyte trafficking at 24 h and 48 h post-SAH were examined. In VAP-1-inhibited vs control rats, results revealed a significant attenuation in leukocyte trafficking at both 24 h and 48 h after SAH, along with an improvement in neurologic outcome. In conclusion, our findings support the involvement of an amplified inflammatory state, characterized by enhanced leukocyte trafficking, during the first 48 h after SAH. VAP-1 blockade yielded neuroprotection that was associated with an attenuation of leukocyte trafficking and improved neurologic outcome.