Fibrodysplasia Ossificans Progressiva and Pregnancy: A Case Series and Review of the Literature

Objective: To evaluate maternal and fetal outcomes in pregnant patients with fibrodysplasia ossificans progressiva (FOP; OMIM#135100), an ultrarare genetic disorder characterized by progressive heterotopic ossification of soft tissues and cumulative disability. Methods: This is a retrospective case series of three patients with FOP who were admitted to Grady Memorial Hospital in Atlanta, Georgia, from to February 2011 to July 2021. Results: Three women delivered preterm infants at our institution. These cases posed unique anesthetic and obstetric technical challenges, particularly when securing the airway and performing cesarean delivery. Importantly, each patient received perioperative glucocorticoids for prevention of further heterotopic ossification. Conclusion: FOP is a unique clinical diagnosis encountered by obstetricians and requires multidisciplinary management for optimal outcomes

Meeting the Critical Need for Ventilators in Treatment of COVID-19 Patients

We have designed and built a microprocessor-controlled valve manifold having a single air input supplied from a standard ventilator, three air outlets (one per patient), and a digital control panel for setting the pressure supplied to each patient as well as the desired respiration rate. The manifold features multiple pressure sensors for system monitoring. Each inspiration limb of the manifold will be fitted with a viral filter. Each expiration limb will have a passive HME in line with a viral filter to prevent patient cross-contamination and spread of virions. Each patient will receive one epoch of inspiration pressure followed by expiration as set by the operator. The electronic system ensures that the respiratory cycles are repeated for each patient at a set respiratory rate. In a later version of the device, the pressure waveform may be variable and different for each patient. In operation, the device will display the realtime pressure and respiration rate for each outlet. Because the device will merely multiplex an existing FDAapproved critical care ventilator now in use at Jefferson, it will not exceed the safety and therapy parameters set for the patients, merely delivering those parameters to three patients instead of one, thereby multiplying surge capacity

When Limb Surgery Has Become the Only Life-Saving Therapy in FOP: A Case Report and Systematic Review of the Literature

Fibrodysplasia ossificans progressiva (FOP) is a rare disease in which heterotopic ossification (HO) is formed in muscles, tendons and ligaments. Traumatic events, including surgery, are discouraged as this is known to trigger a flare-up with risk of subsequent HO. Anesthetic management for patients with FOP is challenging. Cervical spine fusion, ankylosis of the temporomandibular joints, thoracic insufficiency syndrome, restrictive chest wall disease, and sensitivity to oral trauma complicate airway management and anesthesia and pose life-threatening risks. We report a patient with FOP suffering from life-threatening antibiotic resistant bacterial infected ulcers of the right lower leg and foot. The anesthetic, surgical and postoperative challenges and considerations are discussed. In addition, the literature on limb surgeries of FOP patients is systemically reviewed. The 44 year-old female patient was scheduled for a through-knee amputation. Airway and pulmonary evaluation elicited severe abnormalities, rendering standard general anesthesia a rather complication-prone approach in this patient. Thus, regional anesthesia, supplemented with intravenous analgosedation and N2O-inhalation were performed in this case. The surgery itself was securely planned to avoid any unnecessary tissue damage. Postoperatively the patient was closely monitored for FOP activity by ultrasound and [18F]PET/CT-scan. One year after surgery, a non-significant amount of HO had formed at the operated site. The systematic review revealed seventeen articles in which thirty-two limb surgeries in FOP patients were described. HO reoccurrence was described in 90% of the cases. Clinical improvement due to improved mobility of the operated joint was noted in 16% of the cases. It should be noted, though, that follow-up time was limited and no or inadequate imaging modalities were used to follow-up in the majority of these cases. To conclude, if medically urgent, limb surgery in FOP is possible even when general anesthesia is not preferred. The procedure should be well-planned, alternative techniques or procedures should be tested prior to surgery and special attention should be paid to the correct positioning of the patient. According to the literature recurrent HO should be expected after surgery of a limb, even though it was limited in the case described

Special considerations for clinical trials in fibrodysplasia ossificans progressiva (FOP).

Clinical trials for orphan diseases are critical for developing effective therapies. One such condition, fibrodysplasia ossificans progressiva (FOP; MIM#135100), is characterized by progressive heterotopic ossification (HO) that leads to severe disability. Individuals with FOP are extremely sensitive to even minor traumatic events. There has been substantial recent interest in clinical trials for novel and urgently-needed treatments for FOP. The International Clinical Council on FOP (ICC) was established in 2016 to provide consolidated and coordinated advice on the best practices for clinical care and clinical research for individuals who suffer from FOP. The Clinical Trials Committee of the ICC developed a focused list of key considerations that encompass the specific and unique needs of the FOP community - considerations that are endorsed by the entire ICC. These considerations complement established protocols for developing and executing robust clinical trials by providing a foundation for helping to ensure the safety of subjects with FOP in clinical research trials

Gene Therapy for Fibrodysplasia Ossificans Progressiva: Feasibility and Obstacles

Fibrodysplasia ossificans progressiva (FOP) is a rare and devastating genetic disease, in which soft connective tissue is converted into heterotopic bone through an endochondral ossification process. Patients succumb early as they gradually become trapped in a second skeleton of heterotopic bone. Although the underlying genetic defect is long known, the inherent complexity of the disease has hindered the discovery of effective preventions and treatments. New developments in the gene therapy field have motivated its consideration as an attractive therapeutic option for FOP. However, the immune system\u27s role in FOP activation and the as-yet unknown primary causative cell, are crucial issues which must be taken into account in the therapy design. While gene therapy offers a potential therapeutic solution, more knowledge about FOP is needed to enable its optimal and safe application

Electroconvulsive Therapy for a Patient with a Penetrating Ulcer in the Aortic Arch

Case: An 85 year-old male presented with major depressive disorder refractory to medical therapy. A series of eight electroconvulsive therapy (ECT) was planned. Past medical history includes a small penetrating ulcer in the aortic arch, an ascending aortic aneurysm measuring 4.3 x 4.4 cm, atrial fibrillation on anticoagulation and hypertension. Medications include metoprolol, coumadin, simvastatin, alprazolam and venlafaxine

Development of a Staff Recall System for Mass Casualty Incidents Using Cell Phone Text Messaging

BACKGROUND: After a mass casualty incident (MCI), rapid mobilization of hospital personnel is required because of an expected surge of victims. Risk assessment of our department's manual phone tree recall system revealed multiple weaknesses that would limit an effective response. Because cell phone use is widespread within the department, we developed and tested a staff recall system, based in our anesthesia information management system (AIMS), using Short Message Service (SMS) text messaging. METHODS: We sent test text messages to anesthesia staff members' cell phone numbers, determined the distance from their home to the hospital, and stored this information in our AIMS. Latency testing for the time from transmission of SMS test messages from the server to return of an e-mail reply was determined at 2 different times on 2 different dates, 1 of which was a busy holiday weekend, using volunteers within the department. Two unannounced simulated disaster recall drills were conducted, with text messages sent asking for the anticipated time to return to the hospital. A timeline of available staff on site was determined. Reasons for failure to respond to the disaster notification message were tabulated. RESULTS: Latency data were fit by a log-normal distribution with an average of 82 seconds from message transmission to e-mail reply. Replies to the simulated disaster alert were received from approximately 50% of staff, with 16 projecting that they would have been able to be back at the hospital within 30 minutes on both dates. There would have been 21 and 23 staff in-house at 30 minutes, and 32 and 37 staff in-house at 60 minutes on the first and second test date, respectively, including in-house staff. Of the nonresponders to the alert, 48% indicated that their cell phone was not with them or was turned off, whereas 22% missed the message. CONCLUSIONS: Our SMS staff recall system is likely to be able to rapidly mobilize sufficient numbers of anesthesia personnel in response to an MCI, but actual performance cannot be predicted with confidence. Using our AIMS as the source for contact information and from which to send messages was simple, inexpensive, and easy to implement. Updating contact information, periodic testing, and analysis of responses to simulated disaster alerts are essential for the effective functioning of such a system. However, maintenance of alternative methods of communication is recommended, because there may be more significant message transmission delays and failures during an actual MCI, and not all staff will receive the text message in a timely fashion. (Anesth Analg 2010;110:871-8