A call for innovation in fetal monitoring during fetal surgery.

PURPOSE: A growing number of fetal procedures are performed at specialized fetal care centers for congenital problems that classically would have poor outcomes despite advanced postnatal management. Consistent fetal monitoring is integral to the safety of these challenging and innovative surgeries. However, standardization of fetal monitoring during various forms of fetal surgery has yet to be established. MATERIALS AND METHODS: We searched all articles on literature platforms until August 2019 using the terms "fetal surgery," "fetal monitoring," and "fetal interventions." Titles and abstracts were screened by our coauthors to determine the type of fetal monitoring used in these cases. RESULTS: The search identified 1,625 citations, of which the 50 citations considered most pertinent were included in this review. CONCLUSIONS: Fetal monitoring during in utero fetal surgeries continues to be challenging because of limited physical fetal access and technological aspects. Innovations in fetal cardiac monitoring during fetal surgeries have the potential for continuous and high-fidelity hemodynamic and physiologic monitoring, with the goal of early detection and treatment of fetal compromise.

Toward Eliminating Perinatal Comfort Care for Prenatally Diagnosed Severe Congenital Heart Defects: A Vision.

Over the past 40 years, the medical and surgical management of congenital heart disease has advanced considerably. However, substantial room for improvement remains for certain lesions that have high rates of morbidity and mortality. Although most congenital cardiac conditions are well tolerated during fetal development, certain abnormalities progress in severity over the course of gestation and impair the development of other organs, such as the lungs or airways. It follows that intervention during gestation could potentially slow or reverse elements of disease progression and improve prognosis for certain congenital heart defects. In this review, we detail specific congenital cardiac lesions that may benefit from fetal intervention, some of which already have documented improved outcomes with fetal interventions, and the state-of-the-science in each of these areas. This review includes the most relevant studies from a PubMed database search from 1970 to the present using key words such as fetal cardiac, fetal intervention, fetal surgery, and EXIT procedure. Fetal intervention in congenital cardiac surgery is an exciting frontier that promises further improvement in congenital heart disease outcomes. When fetuses who can benefit from fetal intervention are identified and appropriately referred to centers of excellence in this area, patient care will improve.

Fetal Surgery in the Era of SARS-CoV-2 Pandemic: A Single-Institution Review.

OBJECTIVE: To cope with the changing health care services in the era of SARS-CoV-2 pandemic. We share the institutional framework for the management of anomalous fetuses requiring fetal intervention at Mayo Clinic, Rochester, Minnesota. To assess the success of our program during this time, we compare intraoperative outcomes of fetal interventions performed during the pandemic with the previous year. PATIENTS: We implemented our testing protocol on patients undergoing fetal intervention at our institution between March 1, and May 15, 2020, and we compared it with same period a year before. A total of 17 pregnant patients with anomalous fetuses who met criteria for fetal intervention were included: 8 from 2019 and 9 from 2020. METHODS: Our testing protocol was designed based on our institutional perinatal guidelines, surgical requirements from the infection prevention and control (IPAC) committee, and input from our fetal surgery team, with focus on urgency of procedure and maternal SARS-CoV-2 screening status. We compared the indications, types of procedures, maternal age, gestational age at procedure, type of anesthesia used, and duration of procedure for cases performed at our institution between March 1, 2020, and May 15, 2020, and for the same period in 2019. RESULTS: There were no statistically significant differences among the number of cases, indications, types of procedures, maternal age, gestational age, types of anesthesia, and duration of procedures (P values were all >.05) between the pre-SARS-CoV-2 pandemic in 2019 and the SARS-CoV-2 pandemic in 2020. CONCLUSIONS: Adoption of new institutional protocols during SARS-CoV-2 pandemic, with appropriate screening and case selection, allows provision of necessary fetal intervention with maximal benefit to mother, fetus, and health care provider.

Innovative 2-Step Management Strategy Utilizing EXIT Procedure for a Fetus With Hypoplastic Left Heart Syndrome and Intact Atrial Septum.

Hypoplastic left heart syndrome (HLHS) with intact atrial septum (HLHS-IAS) carries a high risk of mortality and affects about 6% of all patients with HLHS. Fetal interventions, postnatal transcatheter interventions, and postnatal surgical resection have all been used, but the mortality risk continues to be high in this subgroup of patients. We describe a novel, sequential approach to manage HLHS-IAS and progressive fetal hydrops. A 28-year-old, gravida 4 para 2 mother was referred to Mayo Clinic for fetal HLHS. Fetal echocardiography at 28 weeks of gestation demonstrated HLHS-IAS with progressive fetal hydrops. The atrial septum was thick and muscular with no interatrial communication. Ultrasound-guided fetal atrial septostomy was performed with successful creation of a small atrial communication. However, fetal echocardiogram at 33 weeks of gestation showed recurrence of a pleural effusion and restriction of the atrial septum. We proceeded with an Ex uteroIntrapartum Treatment (EXIT) delivery and open atrial septectomy. This was performed successfully, and the infant was stabilized in the intensive care unit. The infant required venoarterial extracorporeal membrane oxygenator support on day of life 1. The patient later developed hemorrhagic complications, leading to his demise on day of life 9. This is the first reported case of an EXIT procedure and open atrial septectomy performed without cardiopulmonary bypass for an open-heart operation and provides a promising alternative strategy for the management of HLHS-IAS in select cases.

En bloc resection of a C-1 lateral mass osteosarcoma: technical note.

Osteosarcoma is an aggressive primary bone tumor. It is currently treated with multimodality therapy including en bloc resection, which has been demonstrated to confer a survival benefit over intralesional resection. The authors present the case of an 8-year-old girl with a C-1 lateral mass osteosarcoma, which was treated with a 4-stage en bloc resection and spinal reconstruction. While technically complex, the feasibility of en bloc resection for spinal osteosarcoma should be explored in the pediatric population.

Anesthesia and Duchenne or Becker muscular dystrophy: review of 117 anesthetic exposures.

BACKGROUND: Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are associated with life-threatening perioperative complications, including rhabdomyolysis, hyperkalemia, and hyperthermia. Current recommendations contraindicate use of succinylcholine and volatile anesthetics; however, the latter recommendation remains controversial. OBJECTIVE: To review the perioperative outcomes of patients with DMD and BMD. METHODS: We reviewed records of patients with DMD or BMD who underwent anesthetic management at our institution from January 1990 through December 2011. RESULTS: We identified 47 patients (DMD, 37; BMD, 10) who underwent 117 anesthetic exposures (DMD, 101; BMD, 16). Volatile anesthetic agents were used 66 times (DMD, 59; BMD, 7). One patient with undiagnosed BMD received succinylcholine and developed acute rhabdomyolysis and hyperkalemic cardiac arrest. All other major complications were attributed to the procedure (i.e., large bleeding), to preexisting comorbidities (i.e., respiratory failure, cardiac disease), or to both. CONCLUSIONS: Use of succinylcholine in children with dystrophinopathy is contraindicated. These patients have significant comorbidities and are frequently undergoing extensive operations; complications related to these factors can develop, as evidenced by our series. These complications may occur with use of volatile and nonvolatile anesthetics. However, because most of our patients were older than 8 years at the time of surgery, our observation cannot be generalized to younger dystrophin-deficient children.

Retrograde intubation during laryngeal cleft repair on cardiopulmonary bypass.

Retrograde intubation is part of the difficult airway algorithm in the American Society of Anesthesiologists, but its usage is rare in small pediatric patients with the advent of new intubation techniques. We present our experience of retrograde intubation for a 4-month-old patient who presented for laryngeal cleft repair on cardiopulmonary bypass. This case highlights the unique place for retrograde intubation in small patients in the current era.

Simulation education in anesthesia training: a case report of successful resuscitation of bupivacaine-induced cardiac arrest linked to recent simulation training.

Simulation training is rapidly becoming an integral element of the education curriculum of anesthesia residency programs. We report a case of successful resuscitation of bupivacaine-induced cardiac arrest treated with i.v. lipid emulsion by providers who had recently participated in simulation training involving a scenario nearly identical to this case. Upon debriefing, it was determined that the previous training influenced execution of the following steps: rapid problem recognition, prompt initiation of specific therapy in the setting of supportive advanced cardiac life support measures, and coordinated team efforts. Although the true cause of efficient resuscitation and ultimate recovery cannot be proven, the efficiency of the resuscitation process, including timely administration of lipid emulsion, is evidence that simulation may be useful for training providers to manage rare emergencies.

Plexiform fibrohistiocytic tumor: a rare low-grade malignancy of children and young adults.

Plexiform fibrohistiocytic tumor (PFT) is a low-grade, superficial, soft tissue neoplasm with a limited but significant ability to metastasize. This type of tumor only rarely presents in the skin of the head and neck. Clinicians first encountering young patients with facial neoplasia, such as a PFT, might be unaware of its exact oncologic potential and instead be primarily concerned with the cosmetic outcome. We treated a 17-year-old boy with a PFT on his cheek who was initially treated only by shave biopsy. The tumor subsequently recurred and metastasized to the cervical lymph nodes 3 years after the initial biopsy. Therefore, appropriate initial therapy for PFT requires complete excision with negative resection margins.