Cornelia de Lange syndrome (CdLS) is a rare genetic disorder with a wide variation of presentations affecting several organ systems. Most patients with CdLS have a mutation of the NIPBL (Nipped-B-like protein) gene responsible for making delangin, a protein important in human development. Patients with CdLS typically present with intellectual disability, growth delay, cleft palate, and extremely limited mouth opening. These findings can complicate induction of general anesthesia, airway management, and intubation. Treatment of CdLS is based on clinical presentation and includes pharmacologic, therapeutic, and surgical intervention. Cleft palate repair is common in these patients which may impact the anesthetic plan. Additionally, the anesthesiologist should be aware of associated cardiovascular anomalies, ranging from innocent murmurs to persistent structural defects and leading to increased morbidity and mortality in these patients. This case report discusses CdLS and its implications on the general anesthetic management of a 37-year-old woman undergoing treatment for dental caries in an ambulatory care setting. Although CdLS is a rare genetic disorder, anesthesiologists treating such patients with special needs should be familiar with its presentation and anesthetic considerations.

Malignant hyperthermia (MH) is a rare but life-threatening pharmacogenetic disorder triggered by volatile anesthetics and/or depolarizing muscle relaxants, resulting in a hypermetabolic crisis. We report a case of postoperative fulminant MH in a 26-year-old man who underwent general anesthesia with desflurane and rocuronium for mandibular fracture repair. Despite a history of multiple uneventful anesthetics, the patient developed acute hypercapnia, tachycardia, muscle rigidity, and rapid hyperthermia during emergence, following sugammadex administration. Initial treatment for presumed bronchospasm and postoperative shivering-including sevoflurane sedation, hyperventilation, and inhaled bronchodilators-proved ineffective. A clinical diagnosis of MH was made based on the progression of clinical findings, and treatment with intravenous dantrolene 100 mg, active cooling, and antipyretics was initiated. The patient responded well to intervention and recovered without sequelae. This case highlights the importance of considering MH even in the postoperative phase, particularly with the use of desflurane and potentially contributory agents such as sugammadex and ondansetron. Early clinical suspicion, rapid administration of dantrolene, and supportive measures are critical for favorable outcomes. Anesthesia providers must remain vigilant for MH at all perioperative stages and ensure that emergency protocols, appropriate drug stock, and staff readiness are in place.

Temporomandibular disorders (TMDs) comprise a group of multifactorial conditions involving the masticatory muscles, temporomandibular joint, and associated structures influenced by biomechanical, neuromuscular, and biopsychosocial factors. Myogenous TMDs may involve muscle hyperactivity, parafunction, and central sensitization, contributing to chronic pain and functional limitation. Oral skeletal muscle relaxants (SMRs) are commonly used as adjunctive pharmacologic therapy; however, these agents vary considerably in mechanism, efficacy, and safety. This review summarizes the pharmacology, clinical evidence, and risk profiles of commonly prescribed SMRs-including cyclobenzaprine, baclofen, tizanidine, and others-within the context of chronic TMD management. Evidence supports short-term benefit for select agents, though data for chronic daily use remain limited. Given concerns regarding sedation, dependence, hepatotoxicity, and interactions with polypharmacy, clinicians should employ these medications judiciously and primarily for acute exacerbations rather than long-term therapy. An evidence-based, multimodal approach remains essential for optimizing care in patients with chronic TMDs.

Mixed connective tissue disease (MCTD) is an autoimmune disorder characterized by overlapping features of multiple connective tissue diseases in addition to anti-U1-ribonucleoprotein (RNP) antibodies and Raynaud's phenomenon. A 53-year-old female patient with MCTD, interstitial pneumonia, and idiopathic thrombocytopenic purpura underwent partial tongue resection under general anesthesia. Perioperative management included steroid coverage and careful titration of the oxygen concentration. Although venous access was difficult, the anesthetic course was uneventful. MCTD can present perioperative challenges including pulmonary and cardiac complications, effects of long-term steroid use, and airway and cardiovascular disturbances. Safe anesthesia for patients with MCTD requires assessment of disease activity and severity across multiple conditions and individualized management accordingly.

The trigeminocardiac reflex (TCR) is clinically defined as the sudden onset of increased parasympathetic activity resulting from stimulation of the trigeminal nerve. This reflex increases parasympathetic tone and can lead to significant bradycardia and hypotension. We report the case of severe bradycardia and hypotension that occurred under general anesthesia during insertion of a cotton swab into the nasal cavity. A 57-year-old woman was scheduled for nasotracheal intubation for oral surgery, and following loss of consciousness, intubation was initiated using cotton swabs soaked in a solution of 2% lidocaine and 1:100,000 epinephrine that were inserted through the left nostril. The patient immediately developed severe bradycardia and hypotension which were managed with intravenous ephedrine after removing the cotton swabs rather than atropine due to concerns related to closed-angle glaucoma. The patient responded promptly to the ephedrine bolus, and no further complications occurred. It was assumed that the ophthalmic (V1) and maxillary (V2) branches of the trigeminal nerve were stimulated by the cotton swabs being inserted into the nasal cavity, triggering the TCR. Anesthesiologists need to be aware of the risks associated with administering mydriatic drugs such as ephedrine and epinephrine, which are relatively contraindicated, as well as atropine to patients with closed-angle glaucoma.

Cleft lips and palate are orofacial deformities that require surgical repair in childhood to promote speech development, facial growth, and improved aesthetics. In these cases, preformed oral Ring-Adair-Elwyn (RAE) endotracheal tubes (ETTs) are frequently used for airway management during general anesthesia. Due to its acute U-shaped curve, the oral RAE ETT was our first choice. However, the ability to adjust the intubation depth can be limited because of the preformed curve, and insertion length is fixed based on the internal tube diameter. During neck extension after intubation, the ETT moves away from the carina, which can cause the ETT cuff to compress the vocal cords. Therefore, oral RAE ETTs are associated with a risk of inadvertent ETT malpositioning. Our study assessed ETT positioning in 186 patients in whom oral RAE ETTs were initially used, and we found an 8% incidence rate of inadvertent ETT malpositioning after head and neck extension. In such cases, we replaced the malpositioned oral RAE ETT with a wire-reinforced ETT, which we feel was ideal to serve as a secondary option. Wire-reinforced ETTs tolerate extreme flexion without kinking and can withstand high pressure without collapsing. Although oral RAE ETTs can be advantageous for cleft lip and palate surgery, they can become malpositioned after neck extension and should be assessed accordingly. Consideration for using a wire-reinforced ETT as a replacement is warranted in such instances.

This case report describes use of a new gastric tube tip localization system, the Tumguide (Otsuka Pharmaceutical Factory), in a 50-year-old male patient undergoing general anesthesia for treatment of a mandibular fracture. After induction of general anesthesia and intubation, a gastric tube was inserted using the Tumguide system. After the gastric tube was advanced approximately 50 cm, light from the Tumguide was confirmed emanating below the left rib. The gastric tube was ultimately advanced another 20 cm and fixed in place. A postoperative chest radiograph confirmed the gastric tube was positioned inside the stomach; however, it was withdrawn approximately 10 cm and resecured. Although there are various methods, like radiography, for confirming appropriate positioning of a gastric tube, the insertion process is blind. The Tumguide uses light with high biopermeability for illumination, which enables the gastric tube tip to be indirectly viewed as it is moved into position. Although this system has not gained wide usage, we feel this is a useful device that merits further exploration.

In this report, we aimed to describe an appropriate setting for a patient-controlled analgesia (PCA) pump for postoperative pain control using local anesthetic after bimaxillary osteotomy, which is considered a highly invasive procedure. We conducted a cadaver study to determine the appropriate flow rate setting and then applied our findings to a clinical case. We examined the distribution of local anesthetic administered as ultrasound-guided inferior alveolar nerve blocks (UGIANBs) at different volumes. Using the same UGIANB approach, we placed an indwelling catheter in a healthy male who had undergone bimaxillary osteotomy and set the PCA pump loaded with 0.2% ropivacaine to continuously administer local anesthetic at a flow rate of 7 mL/hour with an additional 5-mL bolus per activation and 30-minute lockout. The patient reported no postoperative pain, did not require other analgesics or antiemetics postoperatively, and had no complications. This approach may have a major impact on clinical practice as it enables opioid-free postoperative analgesia, which may accelerate patient recovery by reducing perioperative complications and shortening hospital stays.