Anesthetic Management of an Adult Patient With Hyaline Fibromatosis Syndrome Undergoing Laparoscopic Colectomy: A Case Report.

Hyaline fibromatosis syndrome (HFS) is a rare autosomal recessive disorder characterized by hyaline fibrous depositions in the skin and internal organs. Contractured joints and gingival hypertrophy make airway management difficult in patients with HFS, while trunk deformities complicate surgical positioning. A 56-year-old woman with HFS underwent laparoscopic colectomy for sigmoid colon cancer. Her airway was secured by awake fiberoptic intubation, and general anesthesia was maintained uneventfully. This report discusses the oldest reported patient with HFS and is the first to describe the management of epidural anesthesia in a patient with HFS.

Anesthesia management for emergency laparotomy in a pediatric patient with suspected hereditary angioedema.

Hereditary angioedema (HAE) is caused by complement factor 1 inhibitor (C1-INH) deficiency, and its mode of inheritance is autosomal dominant. We present a case of an 8-year-old patient who required emergency laparotomy after a traffic accident. General anesthesia with tracheal intubation was necessary. The patient's mother and maternal grandmother had been diagnosed with HAE. HAE is associated with high mortality when airway edema is caused by tracheal intubation. It was impossible to rule out HAE preoperatively in the patient. Therefore, we presumed that he had HAE and treated him with pasteurized C1-INH concentrate. The patient underwent laparotomy uneventfully. Several days after the operation, the laboratory data revealed that the perioperative plasma complement 1 q subunit (C1q) protein level and C1-INH function were not lowered. The diagnosis of HAE was not confirmed, but it was not possible to rule out the diagnosis either. The prophylactic use of a C1-INH in this case may be justified, because the procedure was an emergency and because of the high mortality associated with tracheal intubation in patients with HAE.

Unexpectedly difficult intubation caused by subglottic stenosis in Wegener's granulomatosis.

A 76-year-old woman was scheduled to undergo abdominal aortic repair for progressive abdominal aortic aneurysm. After inducing general anesthesia, the 7.5-mm internal diameter (ID) tracheal tube could not be advanced below the level of the vocal cords because of resistance, and intubation was re-attempted several times using smaller tubes. An otolaryngologist was consulted and subglottic stenosis of unknown origin was suggested. The aortic repair was cancelled and tracheostomy was performed instead. She was diagnosed with Wegener's granulomatosis 46 days after the operation because she developed symptoms of renal dysfunction, hemoptysis, gastrointestinal bleeding, and presence of anti-neutrophil cytoplasmic autoantibodies (c-ANCA). The patient was treated with steroids but died 89 days after the operation because of pulmonary bleeding and renal dysfunction. Tracheal stenosis is a rare presenting feature of Wegener's granulomatosis that usually occurs late in the disease; however, anesthesiologists around the world need to bear in mind that the disease can present airway symptoms and can be the cause of airway obstruction.

[Postextubation laryngeal edema seven years after undergoing neck dissection].

We report a case of upper airway obstruction after extubation in a 69-year-old female patient who underwent transurethral ureterolithotripsy (TUL). She had underwent bilateral modified radical neck dissection 7 years previously. TUL went smoothly in Trenderenburg position, and the extubation was performed after antagonism of neuromuscular block. The patient was closely observed in the operating theater, but about 10 minutes after extubation, she was noted to have dyspnea and tracheal tug. Dexamathasone 2 mg IV was given but was unsuccessful. Although we could support the airway with bag-mask ventilation, continuous stridor required re-intubation. Direct laryngoscopy revealed severe obstruction caused by laryngeal edema. An otolaryngologist was consulted and he performed tracheostomy. We transferred the patient to the intensive care unit for observation. Flexible fiberoptic scope examination performed on postoperative day (POD) 1 showed the decrease of the laryngeal edema. Tacheal tube was removed on POD 7 and she was discharged from the hospital POD 10 without further complications. Patients after a neck dissection may be at elevated risk for postoperative laryngeal edema caused by lymphatic destruction or venous congestion of the neck.

Postoperative neuroleptic malignant syndrome that occurred repeatedly in a patient with cerebral palsy.

A wide variety of neuroleptic agents are associated with neuroleptic malignant syndrome (NMS). However, the association between general anesthesia and NMS is uncertain. We report a case of a patient with cerebral palsy, who showed signs of NMS only after repeated general anesthesia. The patient received general anesthesia three times in a period of 9 months. The first anesthetic passed uneventfully. NMS symptoms were observed only after the second and third anesthetics. The NMS was effectively treated with IV dantrolene and the patient recovered on both occasions. Inhalational anesthetics, muscle relaxants and fentanyl were suspected as possible triggering factors for NMS. After examining the three anesthesia records and previous publications, we surmized that a nondepolarizing muscle relaxant was associated with NMS in this patient.

A case report of anesthetic management of the minimally invasive Nuss operation for pectus excavatum.

We describe the anesthetic management of a patient with pectus excavatum, receiving the minimally invasive Nuss operation, which corrects chest wall deformity by insertion of a convex steel bar (pectus bar). An 8-year-old female patient was scheduled for the Nuss operation. The manipulation of the bar in the thorax was guided by thoracic endoscopy. Anesthesia was maintained with a combination of general and epidural anesthesia. The intraarterial catheter and epidural catheter were inserted after the induction of general anesthesia. During the manipulation of the pectus bar in the anterior mediastinum, a sudden decrease in arterial pressure might occur due to the compression of the heart. Therefore, the intraarterial line was essential for continuous monitoring of arterial pressure. The pectus bar causes severe postoperative pain, and the patient was required to remain at bed rest for several days. Continuous epidural infusion of ropivacaine and morphine eliminated the postoperative pain and enabled the patient to maintain bed rest. For the anesthetic managements of patients undergoing the Nuss operation, close monitoring of arterial pressure intraoperatively and postoperative analgesia are important.

Comparison of ropivacaine with bupivacaine and lidocaine for ilioinguinal block after ambulatory inguinal hernia repair in children.

BACKGROUND: We have compared ropivacaine with bupivacaine and lidocaine for ilioinguinal block in thirty children undergoing ambulatory inguinal hernia repair. METHODS: Patients were assigned randomly to receive 0.5 ml.kg(-1) of 0.2% ropivacaine (Group R, n = 10), 0.25% bupivacaine (Group B, n = 10) or 1% lidocaine (Group L, n = 10). The patients' parents, who were not informed of the type of local anaesthetic employed, evaluated the postoperative pain at 2 h and 6 h after operation using the Wong-Baker FACES Pain Rating Scale. RESULTS: There was a significant difference in the face scale score between Group R and Group L, and Group B and Group L. There was no difference in the face scale score between Group R and Group B. There were no complications or clinical evidence of local anaesthetic toxicity. CONCLUSIONS: We have confirmed that bupivacaine and ropivacaine are more effective than lidocaine in the prevention of postoperative pain after children's inguinal hernia repair. We suggest that ropivacaine 0.2% is an alternative to bupivacaine 0.25% for ilioinguinal block in ambulatory paediatric surgery.