Splanchnic vein thrombosis occurred in 1.8% patients of AP. The use of AC was reasonably safe with no fatal bleeding complications. However, there was no significant difference in the recanalization rates in those with and without AC.

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The labral classification system and its associated analytical tools serve as useful references in consultation, simplify discussion of patients with upper lip defects, furnish a practical alternative to complex algorithms, enable documentation of changes, and facilitate analysis of large sample sizes. When implemented judiciously, the tools described in this article will help surgeons confidently address upper lip problems by streamlining accurate diagnosis and guiding proper treatment.

Given the shortcomings of current methods to shorten the cutaneous upper lip, the senior authors modified the traditional subnasal lip lift with endonasal flaps, performing 311 such lifts over a 15-year period. Ideal surgical candidates are adults with tall philtra, negligible dental show, and no maxillary abnormalities. Benefits of the procedure include improved scar appearance and pout, absence of tension in visible areas, increased dental and vermilion display, and minimal scar migration (ie, loss of lift). Moreover, caudal rhinoplasty procedures are facilitated via the same incision sites. Undercorrection, sill widening, and alar distortion are mostly avoidable (or at least corrigible) complications. Nasal sill disruption, however, remains an inevitable drawback that may be minimized through meticulous technique.

Primary visceral myopathy caused by a pathogenic mutation in the gene encoding the enteric smooth muscle actin gamma 2 ( ACTG2) affects gastrointestinal and genitourinary tracts and often presents as chronic intestinal pseudoobstruction. We present a case of pediatric onset chronic intestinal pseudoobstruction associated with a novel missense ACTG2 mutation c.439G>T/p.G147C. In addition to the known disease manifestations of feeding intolerance and intestinal malrotation, our patient had a late-onset hypertrophic pyloric stenosis and a late-onset choledochal cyst, the former of which has not previously been described in patients with ACTG2-associated visceral myopathy.

The pathologic records of 182 consecutive patients who had mandible resections were reviewed to determine the incidence of positive margins in the bone specimens and the risk factors associated with positive margins. Of the 182 cases reviewed, 82 (45%) were found to have involvement of the mandible at the time of resection and four (2%) were found to have positive margins. The predominant tumor histology was squamous cell carcinoma, 148 of 182 (81%), followed in frequency by osteosarcoma 12 of 182 (7%), salivary gland tumors 13 of 182 (7%), and miscellaneous other tumors (nine of 182 (5%). Of the four tumors with positive margins, two (50%) were squamous cell carcinomas, one (25%) was an osteosarcoma, and one (25%) was a salivary gland tumor. All four tumors were large tumors that had failed to respond to previous therapy. All obviously involved the mandible at the time of presentation. This study demonstrates that the incidence of bone margin involvement after mandibulectomy is rare and predictable and that clinical selection of candidates for immediate reconstruction is reliable in preventing inappropriate use of free bone flaps in patients at risk for positive bone margins.

Introduction: Long-term data regarding kidney transplantation (KTx) patients with monoclonal gammopathy of undetermined significance (MGUS) are scarce. We evaluated the long-term outcomes of these patients in a single-center retrospective study from the Mayo Clinic, Rochester, Minn., USA. Methods: Patients who had an MGUS before transplant or developed one after KTx were selected. Monoclonal protein was screened as part of the KTx evaluation by serum protein electrophoresis. Screening for posttransplant lymphoproliferative disorder (PTLD) or MGUS after transplant was not required by protocol. Patients with multiple myeloma, dysproteinemia-related kidney disease or no pretransplant serum protein electrophoresis were excluded. Results: Between 1963 and 2006, 3,518 patients underwent KTx. MGUS was identified in 42 patients, with 23 before transplant and 19 after transplant. Median follow-up for these patients was 8.5 years (range 0.3–37). Four (17.4%) pretransplant MGUS patients developed a hematologic malignancy: 2 smoldering multiple myeloma and 2 PTLD – an Epstein-Barr virus-positive diffuse large cell lymphoma and a Hodgkin lymphoma. None of the 19 patients who developed an MGUS after transplant progressed to multiple myeloma, but 2 (10.5%) developed Epstein-Barr virus-negative T cell lymphoproliferative disorders at 16 and 26 years after transplant. Median survival was 26.1 and 28.0 years for the pretransplant and posttransplant MGUS groups, respectively. Conclusion: Progression from true MGUS to multiple myeloma is rare after KTx. KTx appears safe in true MGUS patients if the monoclonal gammopathy was not the cause of the kidney disease. None of the patients progressed to multiple myeloma, but 2 developed smoldering multiple myeloma and several developed PTLD. Further studies are needed to explain the relationship between MGUS and PTLD.

Background The identification of autoantibodies directed against neuronal antigens has led to the recognition of a wide spectrum of neurological autoimmune disorders (NAD). With timely recognition and treatment, many patients with NAD see rapid improvement. Symptoms associated with NAD can be diverse and are determined by the regions of the nervous system affected. In addition to neurological symptoms, a number of these disorders present with prominent gastrointestinal (GI) manifestations such as nausea, diarrhea, weight loss, and gastroparesis prompting an initial evaluation by gastroenterologists. Purpose This review provides a general overview of autoantibodies within the nervous system, focusing on three scenarios in which nervous system autoimmunity may initially present with gut symptoms. A general approach to evaluation and treatment, including antibody testing, will be reviewed.