The Influence of Transabdominal Gastroplasty: Early Outcomes of Hiatal Hernia Repair

OBJECTIVE: The aim of our study was to review our experience with transabdominal gastroplasty to determine the safety and short-term efficacy of the procedure. METHODS: Retrospective review of all patients that underwent transabdominal hiatal hernia repair with concurrent gastroplasty for shortened esophagus between October 1999 and May 2004. RESULTS: There were 63 patients, 27 men and 36 women. Median age was 68 years. The hiatal hernia was classified as type-I in 6 patients, type-II in 10, type-III in 43, and type-IV in 4. The operative approach was laparoscopic in 44 patients and laparotomy in 19. A Nissen fundoplication was performed in 62 patients and a Toupet fundoplication in 1. Wedge gastroplasty was performed in 47 patients and modified Collis gastroplasty in 16. Median hospitalization was 3 days (range, 2-10). Intraoperative complications occurred in 11 patients (17%). One laparoscopic approach (2%) was converted to laparotomy. Postoperative complications occurred in 12 patients (19%), there were no operative deaths. Median follow-up was 12 months (range, 0 to 64). One patient (2%) was found to have a recurrent hiatal hernia diagnosed 14 months, postoperatively. Functional results were excellent in 41 (68%), good in 6 (10%), fair in 12 (20%), and poor in 1 (2%). CONCLUSION: Transabdominal gastroplasty can be performed safely, with good functional results and a low incidence of recurrent herniation during the short-term follow-up period.     

Treatment of symptomatic primary chylous disorders.

PURPOSE: Primary chylous disorders (PCDs) are rare. Rupture of dilated lymph vessels (lymphangiectasia) may result in chylous ascites, chylothorax, or leakage of chyle through chylocutanous fistulas in the lower limbs or genitalia. Chyle may reflux through incompetent lymphatics, causing lymphedema. To assess the efficacy of surgical treatment, we reviewed our experience. METHODS: The clinical data of 35 patients with PCDs treated between January 1, 1976, and August 31, 2000, were reviewed retrospectively. RESULTS: Fifteen men and 20 women (mean age, 29 years; range, 1 day-81 years) presented with PCDs. Sixteen (46%) patients had chylous ascites, and 19 (54%) had chylothorax (20 patients), and of these, 10 (29%) had both. In 16 patients, reflux of chyle into the pelvic or lower limb lymphatics caused lymphedema (14, 88%) or lymphatic leak through cutaneous fistulae (11, 69%). Presenting symptoms included lower-limb edema (19, 54%), dyspnea (17, 49%), scrotal or labial edema (15, 43%), or abdominal distention (13, 37%). Primary lymphangiectasia presented alone in 23 patients (66%), and it was associated with clinical syndromes or additional pathologic findings in 12 (yellow nail syndrome in 4, lymphangiomyomatosis in 3, unknown in 3, Prasad syndrome (hypogammaglobulinemia, lymphadenopathy, and pulmonary insufficiency) in 1, and thoracic duct cyst in 1). Twenty-one (60%) patients underwent 26 surgical procedures. Preoperative imaging included computed tomography scan in 15 patients, magnetic resonance imaging in 3, lymphoscintigraphy in 12, and lymphangiography in 14. Fifteen patients underwent 18 procedures for chylous ascites or pelvic reflux. Ten (56%) procedures were resection of retroperitoneal/mesenteric lymphatics with or without sclerotherapy of lymphatics, 4 (22%) were lymphovenous anastomoses or grafts, 3 (17%) were peritoneovenous shunts, and 1 (6%) patient had a hysterectomy. Six patients underwent eight procedures for chylothorax, including thoracotomy with decortication and pleurodesis (4 procedures), thoracoscopic decortication (1 patient), ligation of thoracic duct (2 procedures), and resection of thoracic duct cyst (1 patient). Postoperative mean follow-up was 54 months (range, 0.3-276). Early complications included wound infections in 3 patients, elevated liver enzymes in 1, and peritoneovenous shunt occlusion with innominate vein occlusion in 1. All patients improved initially, but four (19%) had recurrence of symptoms at a mean of 25 months (range, 1-43). Three patients had postoperative lymphoscintigraphy confirming improved lymphatic transport and diminished reflux. One patient died 12 years postoperatively, from causes unrelated to PCD. CONCLUSIONS: More than half of the patients with PCDs require surgical treatment, and surgery should be considered in patients with significant symptoms of PCD. Lymphangiography is recommended to determine anatomy and the site of the lymphatic leak, especially if lymphovenous grafting is planned. All patients had initial benefit postoperatively and two thirds of patients demonstrated durable clinical improvement after surgical treatment.     

Example of somatic mosaicism in a series of de novo neurofibromatosis type 1 cases due to a maternally derived deletion

Neurofibromatosis type 1 (NF1), affecting primarily the growth of neural crest-derived tissues, is one of the most common autosomal dominant genetic disorders with an unusually high spontaneous mutation rate. In four cases of sporadic NF1, demonstrated by hemizygosity to have a deletion involving the NF1 gene, we were able to assign the deletion event to the maternally derived chromosome. One of these individuals was determined to be a somatic mosaic for NF1, as a trace of the maternally derived haplotype was detected at the NF1 locus. This indicated a postzygotic, as opposed to gametic, deletion event. It may be that somatic mosaicism is more common in NF1 than has hitherto been appreciated and may responsible in part for the high mutation rate in this disorder. In addition, it is suggested that the mechanism(s) of gene deletion is subject to a parent of origin effect, being more frequent on the maternally derived chromosome. This is in contrast to the other types of mutations which, in sporadic NF1, have been found to occur preferentially on the paternally derived chromosome. Hum Mutat 9:452–457, 1997 © 1997 Wiley-Liss, Inc.     

Radiofrequency ablation versus surgical resection for the treatment of oligometastatic lung disease

PurposeThe purpose of this study was to compare efficacy and tolerance between radiofrequency ablation (RFA) and surgery for the treatment of oligometastatic lung disease.Materials and methodsThis retrospective study reviewed patients treated in two institutions for up to 5 pulmonary metastases with a maximal diameter of 4 cm and without associated pleural involvement or thoracic lymphadenopathy. Patient demographics, tumor characteristics, treatment outcome, and length of hospital stay were compared between the two groups. Efficacy endpoints were overall survival (OS), progression-free survival (PFS) and pulmonary or local tumor progression rates.ResultsAmong 204 patients identified, 78 patients (42 men, 36 women; mean age, 53.3 ± 14.9 [SD]; age range: 15–81 years) were treated surgically, while 126 patients (59 men, 67 women; mean age, 62.2 ± 10.8 [SD]; age range: 33–80 years) were treated by RFA. In the RFA cohort, patients were significantly older (P < 0.0001), with more extra-thoracic localisation (P = 0.015) and bilateral tumour burden (P = 0.0014). In comparison between surgery and RFA cohorts, respectively, the 1- and 3-year OS were 94.8 and 67.2% vs. 94 and 72.1% (P = 0.46), the 1- and 3-year PFS were 49.4% and 26.1% vs. 38.9% and 14.8% (P = 0.12), the pulmonary progression rates were 39.1% and 56% vs. 41.2% and 65.3% (P > 0.99), and the local tumour progression rates were 5.4% and 10.6% vs. 4.8% and 18.6% (P = 0.07). Tumour size > 2 cm was associated with a significantly higher local tumor progression in the RFA group (P = 0.010). Hospitalisation stay was significantly shorter in the RFA group (median of 3 days; IQR = 2 days; range: 2–12 days) than in the surgery group (median of 9 days; IQR = 2 days; range: 6–21 days) (P < 0.01).ConclusionRFA should be considered a minimally-invasive alternative with similar OS and PFS to surgery in the treatment of solitary or multiple lung metastases measuring less than 4 cm in diameter without associated pleural involvement or thoracic lymphadenopathy.     

New data concerning the etiology of insulin-dependent diabetes

The mechanism of pancreatic beta-cell destruction in type I (insulin-dependent) diabetes mellitus (IDDM) involves autoimmune events directed against these cells. Anti-beta-cell autoimmunity occurs in genetically predisposed individuals and may precede clinical manifestations of IDDM by several years. Markers for beta-cell autoimmunity, especially islet-cell antibodies, are being used with increasing reliability both for detection of IDDM and for evaluating the risk of development of the disease. HLA alleles associated with IDDM include DR3 and DR4, with the risk of IDDM being especially high in individuals with the heterozygous combination DR3/DR4. Recent advances in molecular biology have resulted in more accurate identification of genetic variants and even of amino acid sequences associated with IDDM. These markers can be used to identify patients with genetic susceptibility to the disease. The mechanism of action of genes associated with IDDM is as yet unknown but probably involves the receptor function of HLA molecules for antigens during immune responses.