Modified Presurgical Nasoalveolar Molding in the Infants with Complete Unilateral Cleft Lip and Palate: A Stepwise Approach

Presurgical orthopedics in one or the other form has been an important part of the multidisciplinary approach towards the better cleft care. Presurgical nasoalveolar molding (PNAM) was described as a modified approach to the conventional form of orthopedics. PNAM not only reduces the severity of the alveolar defect before surgery, it also reduces the nasal deformity bringing it near to the normal. Nonsurgical nasal correction forms an important adjunct to the primary nasal repair at the time of primary lip repair. However, acceptance of nasoalveolar molding in Indian set-up has not been wide spread due to various reasons such as lack of the resources and inability of the parents to comply with the frequent adjustment schedule. At the Nitte Meenakshi Institute of Craniofacial surgery at the Nitte University, Mangalore, authors have developed a modification of the PNAM appliance previously described in the literature. The key modification is done at the time of fabrication of the occlusal prosthesis. This modification has made the overall procedure simpler and at the same time helped to reduce the recall visits by half of the originally required. This article describes the fabrication procedure of the modified nasal alveolar molding appliance and the modified treatment protocol in a stepwise manner.     

Short-Term and Long-Term Outcomes for Patients with Autoimmune Pancreatitis After Pancreatectomy: A Multi-institutional Study

Background

Autoimmune pancreatitis (AIP) is a rare subtype of chronic pancreatitis that may mimic adenocarcinoma of the pancreas. The aim of this study was to evaluate the short-term and long-term outcomes of pancreatectomy for patients with AIP.

Methods

In this multi-institutional study, we identified all patients who underwent pancreatectomy for AIP from 1986 to 2011. AIP was confirmed by pathology review. Clinical presentation, operative details, and postoperative outcomes were analyzed.

Results

Seventy-four patients (median age, 60 years; 69 % male) with AIP underwent pancreatectomy. The main indication for operation was concern for malignancy (n?=?59, 80 %). No patients were found to have pancreatic adenocarcinoma on final pathology. Major complications occurred in ten (14 %) patients, with one perioperative death (1 %). Clinically relevant (grade B/C) pancreatic fistulae occurred in two patients. No patients required reoperation for AIP and 11 (17 %) patients developed recurrent AIP.

Conclusion

Although we do not advocate pancreatectomy for AIP, strong suspicion of malignancy may require an operation in selected patients. For patients with AIP, pancreatectomy resulted in few pancreatic fistulae, a low rate of re-intervention, and a 17 % recurrence rate.     

Morbidity profile and functional outcome of modified facial translocation approaches for skull base tumors

The primary objective of this study was to evaluate morbidity associated with facial translocation approaches for skull base and results of various technical modifications. Forty consecutive patients who underwent facial translocation approaches for accessing skull base tumors from July 2005 to June 2010 were included in this study. There were 25 patients who underwent standard facial translocation, 4 patients medial mini, and 11 patients underwent extended facial translocation. Thirteen patients had benign disease and 27 patients had malignant disease. Resection was R0 in 36 and R1 in 4 patients. Most patients had acceptable cosmetic results. None of the patients had problems related to occlusion or speech and swallowing. The commonest complication observed was nasal crusting in 16 patients. Grade 2 trismus and exposure of mini plate was seen in three patients. Two patients developed necrosis of translocated bone. Three patients developed palatal fistula before modification of palatal incision. Facial translocation provides a satisfactory access for adequate clearance of skull base tumors with satisfactory aesthetic and functional results. With modifications of the surgical technique and implementation of new surgical tools, the morbidity of facial translocation approaches will continue to decrease.     

Lymphoepithelial cysts and cystic lymphangiomas: Underrecognized benign cystic lesions of the pancreas

AIM: To identify their diagnostic and prognostic clinical characteristics in a large series.METHODS: Retrospective review of clinicopathologic and imaging characteristics of patients diagnosed with lymphoepithelial cysts and cystic lymphangiomas of the pancreas at Massachusetts General Hospital.RESULTS: Twelve patients were identified between 1/1/1997 and 8/1/2007. Their median age was 55.5 years(range 19-78 years), and 6 were females. The le-sion was incidentally discovered in half of the patients.Contrast enhanced computed tomography demonstrat-ed that the cysts had thin walls, without calcifications, pancreatic duct dilation or pancreatic parenchyma inva-sion. Endoscopic ultrasound with fine needle aspiration(EUS/FNA) confirmed the diagnosis of a lymphoepithe-lial cyst in 3 patients, one of whom was spared an op-eration and continues to do well after 6 years. Eleven patients had a resection: 3 pancreaticoduodenecto-mies, 7 distal pancreatectomies, and 1 enucleation. The median size of the cysts was 3 cm(range 2-20 cm). At a median follow-up of 57 mo no recurrences or other pancreas-related conditions occurred.CONCLUSION: Lymphoepithelial cysts and cystic lymphangiomas of the pancreas can be diagnosed with a combination of contrast-enhanced computed tomog-raphy scans and EUS/FNA. If the lesion is asymptom-atic, an operation might be avoided.     

Novel PEGylated Basal Insulin LY2605541 Has a Preferential Hepatic Effect on Glucose Metabolism

The impact of the novel basal insulin LY2605541 (LY) on hepatic and nonhepatic glucose uptake (non-HGU) was evaluated. Conscious dogs underwent euglycemic clamps with tracer and hepatic balance measurements. Clamp period infusions were peripheral venous regular insulin (0.1 nmol ⋅ kg⁻¹ ⋅ h⁻¹ [control], n = 6) or LY (bolus [nmol/kg], continuous [nmol ⋅ kg⁻¹ ⋅ h⁻¹]: 0.5, 0.5 [n = 6]; 0.375, 0.375 [n = 5]; 0.25, 0.25 [n = 4]), somatostatin, and glucose, as well as intraportal glucagon (basal). During the clamp, the dogs switched from net hepatic glucose output to uptake (rates reached 2.1 ± 1.2, 0.9 ± 2.1, 8.6 ± 2.3, and 6.0 ± 1.1 µmol ⋅ kg⁻¹ ⋅ min⁻¹ within 5 h in control, LY_0.25, LY_0.375, and LY_0.5, respectively). Non-HGU in LY increased less than in control; the ratio of change from basal in non-HGU to change in net hepatic glucose balance, calculated when glucose infusion rates (GIRs) were ~20 µmol ⋅ kg^-1 ⋅ min⁻¹ in all groups, was higher in control (1.17 ± 0.38) versus LY_0.25 (0.39 ± 0.33), LY_0.375 (−0.01 ± 0.13), and LY_0.5 (−0.09 ± 0.07). Likewise, the change from baseline in glucose R_d-to-R_a ratio was greatest in control (1.4 ± 0.3 vs. 0.6 ± 0.4, 0.5 ± 0.2, and 0.6 ± 0.2 in LY_0.25, LY_0.375, and LY_0.5, respectively). In contrast to exogenously administered human insulin, LY demonstrated preferential hepatic effects, similar to endogenously secreted insulin. Therefore, the analog might reduce complications associated with current insulin therapy.     

Genomic Profiling of Intrahepatic Cholangiocarcinoma: Refining Prognosis and Identifying Therapeutic Targets

Background

The molecular alterations that drive tumorigenesis in intrahepatic cholangiocarcinoma (ICC) remain poorly defined. We sought to determine the incidence and prognostic significance of mutations associated with ICC among patients undergoing surgical resection.

Methods

Multiplexed mutational profiling was performed using nucleic acids that were extracted from 200 resected ICC tumor specimens from 7 centers. The frequency of mutations was ascertained and the effect on outcome was determined.

Results

The majority of patients (61.5 %) had no genetic mutation identified. Among the 77 patients (38.5 %) with a genetic mutation, only a small number of gene mutations were identified with a frequency of >5 %: IDH1 (15.5 %) and KRAS (8.6 %). Other genetic mutations were identified in very low frequency: BRAF (4.9 %), IDH2 (4.5 %), PIK3CA (4.3 %), NRAS (3.1 %), TP53 (2.5 %), MAP2K1 (1.9 %), CTNNB1 (0.6 %), and PTEN (0.6 %). Among patients with an IDH1-mutant tumor, approximately 7 % were associated with a concurrent PIK3CA gene mutation or a mutation in MAP2K1 (4 %). No concurrent mutations in IDH1 and KRAS were noted. Compared with ICC tumors that had no identified mutation, IDH1-mutant tumors were more often bilateral (odds ratio 2.75), while KRAS-mutant tumors were more likely to be associated with R1 margin (odds ratio 6.51) (both P < 0.05). Although clinicopathological features such as tumor number and nodal status were associated with survival, no specific mutation was associated with prognosis.

Conclusions

Most somatic mutations in resected ICC tissue are found at low frequency, supporting a need for broad-based mutational profiling in these patients. IDH1 and KRAS were the most common mutations noted. Although certain mutations were associated with ICC clinicopathological features, mutational status did not seemingly affect long-term prognosis.