The Incidence of Sleep Disordered Breathing One Week After Primary Palatoplasty: Evaluation With Overnight Pulse Oximetry.

BACKGROUND: Sleep disordered breathing (SDB) is defined as a series of disorders including snoring, obstructive sleep apnea, and hypopnea. Few studies investigated the incidence of SDB following primary palatoplasty with objective testing. The aims of this study were to elucidate the prevalence and degree of SDB approximately 1 week following primary palatoplasty with objective testing and to clarify the risk factors. METHOD: A retrospective review was performed on children who underwent primary palatoplasty between April 2013 and July 2017 at National Center for Child Health and Development, Tokyo, Japan. As a national center, the authors accept many syndromic patients. The authors keep all patients after palatoplasty intubated and observe them overnight in intensive care unit to reduce the risks of respiratory events. Patients were evaluated with overnight pulse oximetry on 5 to 7 days postoperatively. RESULTS: Forty-four patients were included, and 30% of the patients were associated with congenital anomaly. Thirteen patients (30%) were diagnosed with SDB. None of the patients required additional treatment after the evaluation. Laryngomalacia and postoperative oxygen requirement significantly correlated with postoperative SDB. CONCLUSION: Approximately one-third of the patients may be at the risk of SDB 1 week after primary palatoplasty. Patients with history of laryngomalacia or those who required oxygen support for prolonged time after primary palatoplasty should be cared for significantly high risk of postoperative SDB.

Perioperative management of living-donor liver transplantation for methylmalonic acidemia.

Methymalonic acidemia (MMA) is a hereditary metabolic disorder characterized by a defect of the methylmalonyl-CoA mutase that breaks down propionate. The efficacy of liver transplantation for MMA was recently reported. However, the anesthetic management of liver transplant for MMA is not clear. The aim of this article is to describe an anesthetic management algorithm of liver transplant for MMA by reviewing our cases of liver transplant for MMA. Fourteen patients received a liver transplant; three cases showed metabolic decompensation during the transplant and two of the patients died. In the two patients who expired, propofol was used for maintenance anesthesia and preoperative continuous hemodiafiltration was used to reduce plasma methylmalonic acid level in one case, and to control severe metabolic decompensation before transplant for the other case. Their renal function was also worse than others and they were already experiencing metabolic decompensation before induction of anesthesia. Based on our experience of these 14 cases, we have established an anesthetic algorithm for patients with MMA undergoing liver transplant or other procedures. There are three important points in our experience: propofol should be avoided, dextrose infusion therapy should be continued to prevent metabolic decompensation, and liver transplant or other procedures should be avoided during metabolic decompensation.

Cerebral hypoperfusion during pediatric cardiac surgery detected by combined bispectral index monitoring and transcranial doppler ultrasonography.

Bispectral index monitoring (BIS) measures depth of anesthesia and sedation. The case of a neonatal patient who underwent surgical repair for a double aortic arch is presented. During surgery, BIS decreased to 0, and cerebral blood flow (CBF), as measured by transcranial doppler ultrasonography, could not be detected immediately after clamping of the arch. BIS returned to baseline, and CBF was detected only after the aortic arch was unclamped. The arch was then carefully reclamped during close BIS and CBF monitoring.

Successful high-dose methotrexate chemotherapy in a patient with acute lymphocytic leukemia who developed acute renal failure during the initial treatment.

Methotrexate (MTX) is a key drug in the chemotherapy for childhood acute lymphocytic leukemia (ALL). It is essential in the treatment of such areas as the central nervous system (CNS) and reproductive organs. High-dose chemotherapy is applied for this purpose to obtain an effective plasma concentration in the target organs. There are three major mechanisms of nephrotoxicity related with MTX. One is that induced by allergic reaction, which usually appears as interstitial nephritis. In this case MTX is contraindicated. Another is direct pharmacological toxicity against renal tubules. The third is precipitation of MTX, which plugs the renal tubules. The latter two are consequently dose dependent, and are usually associated with high-dose chemotherapy. To prevent these nephrotoxicities, hydration and alkalinization of the urine are performed to accelerate the urinary excretion and avoid the precipitation of MTX.

Successful nonmyeloablative cord blood transplantation for an infant with malignant infantile osteopetrosis.

Malignant infantile osteopetrosis (MIOP) is a lethal disorder caused by osteoclast dysfunction. The only curative therapy for MIOP is stem cell transplantation (SCT). Because the number of patients is limited, the conditioning regimen and the use of alternative donors for SCT have been controversial and not established. The authors report a case of successful cord blood transplantation (CBT) with a nonmyeloablative regimen (NMR) for MIOP. The patient was a 9-month-old girl with MIOP. Before this diagnosis, she had received chemotherapy under the tentative diagnosis of juvenile myelomonocytic leukemia. She was on mechanical ventilation with tracheotomy due to the progression of MIOP when CBT with NMR was undergone. The conditioning regimen included fludarabine, melphalan, and antithymocyte globulin. Cyclosporine A and methylprednisolone were used for prophylaxis for graft-versus-host disease. Neutrophil engraftment was achieved on day 26 after SCT and has been fully maintained up to the present. Although grade 3 graft-versus-host disease and hepatic veno-occlusive disease occurred, both were controllable. Although the pretransplant condition of our patient was somewhat unusual, this is the first reported case of successful CBT with NMR for MIOP. Because of the urgent need, CBT can be considered as one of the SCT sources for MIOP, especially in a severe, life-threatening setting.

Allogeneic hematopoietic stem cell transplantation for seven children with X-linked hyper-IgM syndrome: a single center experience.

X-linked hyper-IgM syndrome (XHIM), or hyper-IgM syndrome type 1 (HIGM1), is a rare primary immunodeficiency disorder susceptible to recurrent bacterial infection and opportunistic infection such as Pneumocystis carinii and Cryptosporidium parvum. The long-term outcome is quite poor, and allogeneic hematopoietic stem cell transplantation (HSCT) offers the only cure. Seven patients with XHIM, from age 3 to 19 years (mean 11.3 years), underwent allogeneic HSCT in our institution. Details of pre- and post-transplantation data and transplantation procedure were analyzed retrospectively. The donors were HLA-identical siblings for three patients and HLA-identical unrelated donors for four patients. All but one received conventional conditioning regimen consisting of busulfan and cyclophosphamide and prophylaxis for graft-versus-host disease (GVHD) consisting of cyclosporine and methotrexate. Five out of seven patients are alive and well with normal CD40L expression, and four of these five are free of intravenous immunoglobulin supplementation. The two patients who died had prolonged episodes of severe and recurrent infections and organ damage. We conclude that conventional allogeneic HSCT from HLA matched related or unrelated donors is curative and feasible for XHIM patients, if performed before significant infections and organ damage occur. For the high-risk patients, an alternative approach including nonmyeloablative HSCT may be more feasible.