We performed aortic valve reconstruction (AVrC) using autologous pericardium for a patient with severe aortic stenosis and chronic renal failure, prior to kidney transplantation. The patient received kidney transplantation in the early phase after cardiac surgery. The case was a 61-year-old man with severe aortic valve stenosis who received dialysis due to chronic renal failure. We performed AVrC using autologous pericardium for the following reasons. Anticoagulant therapy is not desirable because of the need to perform kidney transplantation in the early phase after cardiac surgery. Implantation of prosthesis was not desirable because the patient requires oral immunosuppression therapy after kidney transplantation. There was no significant postoperative pressure gradient of the aortic valve orifice or aortic valve regurgitation (AR). The patient received kidney transplantation 113 days after surgery. AVrC using autologous pericardium was feasible for aortic stenosis patients in a patient waiting to receive kidney transplantation because anticoagulation therapy is not necessary after AVrC.

Syndromic craniosynostosis has severe cranial stenosis and deformity, combined with hypoplastic maxillary bone and other developmental skeletal lesions. Among these various lesions, upper air way obstruction by hypoplastic maxillary bone could be the first life-threatening condition after birth. Aggressive cranial vault expansion for severely deformed cranial vaults due to multiple synostoses is necessary even in infancy, to normalize the intracranial pressure. Fronto-orbital advancement (FOA) is recommended for patients with hypoplastic anterior part of cranium induced by bicoronal and/or metopic synostoses, and posterior cranial vault expansion is recommended for those with flattening of the posterior part of the cranium by lambdoid synostosis. Although sufficient spontaneous reshaping of the cranium can be expected by expansive cranioplasty, keeping the cranial bone flap expanded sufficiently is often difficult when the initial expansion is performed during infancy. So far distraction osteogenesis (DO) is the only method to make it possible and to provide low rates of re-expansion of the cranial vault. DO is quite beneficial for both FOA and posterior cranial vault expansion, compared with the conventional methods. Associated hydrocephalus and chronic tonsillar herniation due to lambdoid synostosis can be surgically treatable. Abnormal venous drainages from the intracranial space and air way obstruction should be always considered at any surgical procedures. Neurosurgeons have to know well about the managements not only of the deformed cranial vault and the associated brain lesions but also of other multiple skeletal lesions associated with syndromic craniosynostosis, to improve treatment outcome.
Brain ; Congenital Abnormalities ; Constriction, Pathologic ; Craniosynostoses* ; Encephalocele ; Humans ; Hydrocephalus ; Intracranial Pressure ; Maxilla ; Osteogenesis, Distraction ; Parturition ; Skull ; Synostosis ; Treatment Outcome

We report a case of aortic arch replacement and extraanatomic bypass from a branched graft to both bifemoral arteries in a patient with aortic dissection complicated by ischemia in the lower extremities. A 61-year-old woman was found to have thrombosed type II aortic dissection by enhanced computed tomography (CT). Because she had no clinical symptoms, we chose conservative pharmacotherapy. A year later, she suddenly felt severe back pain and dyspnea. CT demonstrated type IIIb aortic dissection. She developed lower extremity ischemia because the true lumen in the abdominal aorta was severely compressed by the false lumen. Two weeks after onset, we planned a bilateral axillo-femoral bypass because the right lower limb ischemia had worsened, with severe pain. However, CT showed ascending aortic dissection. Hence, emergency graft replacement of aortic arch was required. A T-shaped graft was anastomosed to the bilateral femoral arteries, and was used as a delivery line during cardiopulmonary bypass. Although distal anastomosis of the arch was constructed only to the true lumen, leg ischemia persisted. Therefore, the T-shaped graft was connected to the branched graft used for antegrade systemic perfusion. We used INVOS as an indicator of intraoperative lower limb ischemia, which was useful for judging whether or not revascularization of lower extremity was achieved. After the operation, the bypass graft was patent, and ischemia in the lower extremities disappeared.

Neoadjuvant chemotherapyeoadjuvant chemoradiotherapy (NAC/NACRT) can be performed in patients with pancreatic cancer to improve survival. We aimed to clarify the clinical outcomes of biliary drainage with a metal stent (MS) or a plastic stent (PS) during NAC/NACRT. Between October 2013 and April 2016, 96 patients with pancreatic cancer were registered for NAC/NACRT. Of these, 29 patients who underwent biliary drainage with MS or PS before NAC/NACRT and a subsequent pancreatoduodenectomy were retrospectively analyzed with regard to patient characteristics, preoperative recurrent biliary obstruction rate, NAC/NACRT delay or discontinuation rate, and operative characteristics. The median age of the patients was 67 years. NAC and NACRT were performed in 14 and 15 patients, respectively, and MS and PS were used in 17 and 12 patients, respectively. Recurrent biliary obstruction occurred in 6% and 83% of the patients in the MS and PS groups, respectively (p<0.001). NAC/NACRT delay was observed in 35% and 50% of the patients in the MS and PS groups, respectively (p=0.680). NAC/NACRT discontinuation was observed in 12% and 17% of the patients in the MS and PS groups, respectively (p=1.000). The operative time in the MS group tended to be longer than that in the PS group (625 minutes vs 497 minutes, p=0.051), and the operative blood loss volumes and postoperative adverse event rates were not different between the two groups. MS was better than PS from the viewpoint of preventing recurrent biliary obstruction, although MS was similar to PS with regards to perioperative outcomes.

Autoimmune enteropathy (AIE) is a rare disease, characterized by intractable diarrhea, villous atrophy of the small intestine, and the presence of circulating anti-enterocyte autoantibodies. Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome, and mutations in FOXP3, which is a master gene of regulatory T cells (Tregs), are major causes of AIE. Recent studies have demonstrated that mutations in other Treg-associated genes, such as CD25 and CTLA4, show an IPEX-like phenotype. We present the case of a 13-year-old girl with CTLA4 haploinsufficiency, suffering from recurrent immune thrombocytopenic purpura and intractable diarrhea. We detected an autoantibody to the AIE-related 75 kDa antigen (AIE-75), a hallmark of the IPEX syndrome, in her serum. She responded well to a medium dose of prednisolone and a controlled dose of 6-mercaptopurine (6-MP), even after the cessation of prednisolone administration. Serum levels of the soluble interleukin-2 receptor and immunoglobulin G (IgG) were useful in monitoring disease activity during 6-MP therapy. In conclusion, autoimmune-mediated mechanisms, similar to the IPEX syndrome, may be involved in the development of enteropathy in CTLA4 haploinsufficiency. Treatment with 6-MP and monitoring of disease activity using serum levels of soluble interleukin-2 receptor and IgG is suggested for such cases.