Surgical airway management in Pierre Robin sequence: is there a role for tongue-lip adhesion?

OBJECTIVE: The purpose of this study was to examine the efficacy of tongue-lip adhesion (TLA) in the management of clinically significant airway obstruction associated with Pierre Robin sequence. DESIGN: The records of all children admitted to The Children's Hospital of Philadelphia with a diagnosis of Pierre Robin sequence were reviewed. Charts were reviewed for birth data, diagnosis, preoperative airway management methods, and surgical intervention. Records of infants undergoing TLA were analyzed for timing of surgery, operative technique, postoperative complications, length of hospital stay, and treatment outcome. RESULTS: Over the 28-year period 1971 to 1999, 107 patients (47 boys, 60 girls) meeting the criteria for Pierre Robin sequence were admitted for treatment. Of these, 74 (69.2%) were successfully managed by positioning alone. Surgical management of the airway was performed in the remaining 33 (30.8%) patients, 29 of whom underwent TLA and 4 of whom underwent tracheostomy. Dehiscence of the adhesion occurred in five patients (17.2%), two of whom subsequently required tracheostomy. Within the group of patients who underwent mucosal adhesion alone, the dehiscence rate was 41.6%. When the adhesion included muscular sutures, however, dehiscence was not observed in any patient. Of the 24 patients in whom primary TLA healed uneventfully, airway obstruction was successfully relieved in 20 (83.3%). Failure of a healed TLA to relieve the airway obstruction resulted in conversion to a tracheostomy in four patients. Six patients who underwent TLA (20.7%) ultimately required a tracheostomy; five of these patients (83.3%) were syndromic. Of patients requiring preoperative intubation, 42.9% ultimately required tracheostomy. CONCLUSION: TLA successfully relieves airway obstruction that is unresponsive to positioning alone in the majority of patients with Pierre Robin sequence and should therefore play an important role in the management of these infants.     

Split rib cranioplasty for aplasia cutis congenita and traumatic skull defects: more than 30 years of follow-up

Aplasia cutis is a rare skin defect usually presenting over the vertex of the skull. An underlying bone defect is found in approximately 20% of patients. Most skull defects close spontaneously. However, when there are no signs of ossification, closure is mandatory. We present our experience in three patients. Our first patient had an aplasia cutis with a skull defect. The split rib graft procedure was used without complications, and a good cosmetic and functional result was achieved. The second patient was operated on for cerebral bleeding after an arteriovenous aneurysm, and a bony defect could not be closed after that procedure. At a later stage, the defect was filled with split rib grafts, and sufficient protection was achieved and documented after more than 30 years. The third patient was born with an aplasia cutis congenita with a skull defect. The bony defect was filled with split rib grafts without complications at an age of 5 years. Follow-up shows a functional result with a firm skull. Patients with aplasia cutis may have skull defects that will not close by themselves. We present three patients with a bony defect who were reconstructed with split rib grafts. After a long period of follow-up, there remains good cosmetic and functional results. Defects of the skull in children can be reconstructed with split rib grafts that will accommodate the growing skeleton and give good protection of the brain from an early age on.     

Oxidative tissue injury in multiple sclerosis is only partly reflected in experimental disease models

Recent data suggest that oxidative injury may play an important role in demyelination and neurodegeneration in multiple sclerosis (MS). We compared the extent of oxidative injury in MS lesions with that in experimental models driven by different inflammatory mechanisms. It was only in a model of coronavirus-induced demyelinating encephalomyelitis that we detected an accumulation of oxidised phospholipids, which was comparable in extent to that in MS. In both, MS and coronavirus-induced encephalomyelitis, this was associated with massive microglial and macrophage activation, accompanied by the expression of the NADPH oxidase subunit p22phox but only sparse expression of inducible nitric oxide synthase (iNOS). Acute and chronic CD4⁺ T cell-mediated experimental autoimmune encephalomyelitis lesions showed transient expression of p22phox and iNOS associated with inflammation. Macrophages in chronic lesions of antibody-mediated demyelinating encephalomyelitis showed lysosomal activity but very little p22phox or iNOS expressions. Active inflammatory demyelinating lesions induced by CD8⁺ T cells or by innate immunity showed macrophage and microglial activation together with the expression of p22phox, but low or absent iNOS reactivity. We corroborated the differences between acute CD4⁺ T cell-mediated experimental autoimmune encephalomyelitis and acute MS lesions via gene expression studies. Furthermore, age-dependent iron accumulation and lesion-associated iron liberation, as occurring in the human brain, were only minor in rodent brains. Our study shows that oxidative injury and its triggering mechanisms diverge in different models of rodent central nervous system inflammation. The amplification of oxidative injury, which has been suggested in MS, is only reflected to a limited degree in the studied rodent models.     

Healthcare Intensity at Initiation of Chronic Dialysis among Older Adults

Little is known about the circumstances under which older adults initiate chronic dialysis and subsequent outcomes. Using national registry data, we conducted a retrospective analysis of 416,657 Medicare beneficiaries aged ≥67 years who initiated chronic dialysis between January 1995 and December 2008. Our goal was to define the relationship between health care intensity around the time of dialysis initiation and subsequent survival and patterns of hospitalization, use of intensive procedures (mechanical ventilation, feeding tube placement, and cardiopulmonary resuscitation), and discontinuation of dialysis before death. We found that most patients (64.5%) initiated dialysis in the hospital, including 36.6% who were hospitalized for ≥2 weeks and 7.4% who underwent one or more intensive procedures. Compared with patients who initiated dialysis in the outpatient setting, those who received the highest intensity of care at dialysis initiation (those hospitalized ≥2 weeks and receiving at least one intensive procedure) had a shorter median survival (0.7 versus 2.1 years; P<0.001), spent a greater percentage of remaining follow-up time in the hospital (median, 22.9% versus 3.1%; P<0.001), were more likely to undergo subsequent intensive procedures (44.9% versus 26.0%; adjusted hazard ratio, 2.33; 95% confidence interval [CI], 2.27 to 2.39), and were less likely to have discontinued dialysis before death (19.1% versus 26.2%; adjusted odds ratio, 0.68; 95% CI, 0.65 to 0.72). In conclusion, most older adults initiate chronic dialysis in the hospital. Those who have a prolonged hospital stay and receive other forms of life support around the time of dialysis initiation have limited survival and more intensive patterns of subsequent healthcare utilization.Over the last decade, a growing number of older adults are initiating chronic dialysis.¹ Survival among these older patients is extremely limited,^2,3 and many experience functional decline,^4,5 frequent hospitalization,⁶ and a high symptom burden⁷ after initiation of chronic dialysis. In this setting, patients must often make trade-offs between interventions intended to lengthen life and those directed at other treatment goals, such as maximizing quality of life and maintaining independence.Rates of hospitalization and use of intensive procedures, such as mechanical ventilation, feeding tube placement, and cardiopulmonary resuscitation (CPR), at the end of life are exceptionally high in older dialysis patients compared with other older Medicare beneficiaries with life-limiting illness.⁸ Discussions about prognosis, goals, and preferences are often lacking, and patients may have little appreciation of their likelihood of clinical deterioration or knowledge of more conservative alternatives, such as hospice.⁹ Uncertainty about disease trajectory and prognosis can hamper formulation of future plans and treatment preferences.^10–12Prior studies have evaluated the association of patient characteristics (e.g., comorbid conditions and functional status)^5,13,14 and treatment practices (e.g., early nephrology referral and type of vascular access)^15,16 before and at the time of dialysis initiation with subsequent outcomes. However, many of these analyses did not capture information on clinical circumstances around the time of dialysis initiation. In reality, chronic dialysis is often initiated in the context of acute illness^17,18 and rapid or unexpected loss of renal function.¹⁴We hypothesized that illness severity around the time of dialysis initiation—as reflected in measures of health care intensity, such as length of hospitalization and use of intensive procedures—might provide information useful for anticipatory guidance and supporting treatment decisions in older adults newly initiated on chronic dialysis. To evaluate this hypothesis, we used data from the U.S. Renal Data System (USRDS), a national registry of ESRD, to identify 416,657 Medicare beneficiaries aged ≥67 years and describe the intensity of care they experienced around the time of initiation of chronic dialysis and its association with survival and patterns of future healthcare utilization.     

Clinical Practice Guidelines for the Medical and Surgical Management of Primary Intracerebral Hemorrhage in Korea

The purpose of this clinical practice guideline (CPG) is to provide current and comprehensive recommendations for the medical and surgical management of primary intracerebral hemorrhage (ICH). Since the release of the first Korean CPGs for stroke, evidence has been accumulated in the management of ICH, such as intracranial pressure control and minimally invasive surgery, and it needs to be reflected in the updated version. The Quality Control Committee at the Korean Society of cerebrovascular Surgeons and the Writing Group at the Clinical Research Center for Stroke (CRCS) systematically reviewed relevant literature and major published guidelines between June 2007 and June 2013. Based on the published evidence, recommendations were synthesized, and the level of evidence and the grade of the recommendation were determined using the methods adapted from CRCS. A draft guideline was scrutinized by expert peer reviewers and also discussed at an expert consensus meeting until final agreement was achieved. CPGs based on scientific evidence are presented for the medical and surgical management of patients presenting with primary ICH. This CPG describes the current pertinent recommendations and suggests Korean recommendations for the medical and surgical management of a patient with primary ICH.     

Acute Onset of Intracerebral Hemorrhage due to Autonomic Dysreflexia

Autonomic dysreflexia is a clinical emergency syndrome of uncontrolled sympathetic output that can occur in patients who have a history of spinal cord injury. Despite its frequency in spinal cord injury patients, central nervous system complications are very rare. We report a man with traumatic high level incomplete spinal cord injury who suffered hypertensive right thalamic hemorrhage secondary to an episode of autonomic dysreflexia. Prompt recognition and removal of the triggering factor, the suprapubic catheter obstruction which led to hypertensive attack, the patient had a favorable functional outcome after the resorption of the hematoma and effective rehabilitation programme.