Zenker's and epiphrenic diverticula in a patient with cutis laxa: should the surgical technique be modified?

Currently, most of esophageal diverticula arise as the result of a pulsion effect. Some esophageal motor disorders increase the intraluminal pressure and after some time, the diverticula grow through a weak point of esophageal wall. In these cases, the surgical treatment of choice is the myotomy associated with diverticulopexy or diverticulectomy. Adding a fundoplication is accepted to avoid the consequences of gastroesophageal reflux after myotomy in the epiphrenic diverticula surgery. There are other causes of esophageal diverticula that change the resistance of esophageal wall. Cutis laxa, a congenital or acquired connective disease, is a strange one. In our patient, a good result was reached modifying the standard technique accord to its ethiopathogenic mechanism.

Effectiveness of a written clinical pathway for enhanced recovery after transthoracic (Ivor Lewis) oesophagectomy.

BACKGROUND: This study assessed the feasibility of a protocol-driven written clinical pathway for multidisciplinary postoperative management after oesophagectomy for oesophageal neoplasia, and examined whether the application of such a protocol could shorten hospital stay and reduce postoperative morbidity and mortality. METHODS: Consecutive patients undergoing transthoracic oesophagectomy for oesophageal neoplasia were divided into those treated between 2003 and 2008 to whom a clinical pathway was applied for postoperative management (group 1), and a control group treated between 1998 and 2002 when no clinical pathway was applied (group 2). RESULTS: There were 74 patients in each group. Morbidity rates were similar in the two groups: 31 per cent in group 1 and 38 per cent in group 2. There were more pulmonary complications in group 2 (23 versus 14 per cent; P = 0.025). One patient (1 per cent) in group 1 and four (5 per cent) in group 2 died after surgery (P = 0.010). The median (range) length of hospital stay was 9 (5-98) days for group 1 and 13 (8-106) days in group 2 (P = 0.012). CONCLUSION: Use of a written clinical pathway in patients undergoing oesophageal resection significantly reduced pulmonary complications, postoperative mortality and hospital stay.

Ineffective oesophageal motility does not affect the clinical outcome of open Nissen fundoplication.

BACKGROUND: Nissen fundoplication is considered the 'gold standard' in antireflux surgery but some surgeons employ a different surgical strategy when gastro-oesophageal reflux disease (GORD) is associated with motor disorders of the oesophageal body. METHODS: Ninety-three patients undergoing surgery for GORD were divided into two groups: 52 patients (group 1) had normal oesophageal body motility and 41 (group 2) had ineffective oesophageal motility (IOM). All patients had a short Nissen fundoplication via a laparotomy. The median follow-up was 5 years in group 1 and 6.5 years in group 2. RESULTS: The clinical outcome was satisfactory in more than 90 per cent of the patients in both groups. Only one of ten patients with IOM and dysphagia before operation still had dysphagia after surgery. One patient in each group developed postoperative dysphagia. Six of 52 patients with normal motility and eight of 41 with IOM had persistent pathological acid reflux after surgery. Significant increases in contractile wave pressure and a decrease in the percentage of non-propagated waves were found in group 2 after fundoplication. CONCLUSION: Patients with IOM did not have an increased rate of dysphagia after total fundoplication compared with those with normal motility, but they did have a higher rate of recurrence of endoscopic and pH-proven reflux.

Primitive leiomyosarcoma of the breast: case report and review of the literature.

Sarcomas of the breast account for under 1% of breast tumours. Leiomyosarcomas are less common, being a subgroup of sarcomas of the breast. Only 23 cases with immunohistochemical or electron microscopy confirmation are reported in the literature. The case of a 58-year-old woman with a leiomyosarcoma 4 cm in diameter in the upper external quadrant of the right breast is presented. Mammography and sonography were compatible with a fibroadenoma or phylloides tumour, and fine-needle aspiration (FNA) suggested medullary carcinoma. The patient underwent a Madden-type modified radical mastectomy and axillary lymphadenectomy. Adjuvant chemotherapy was implemented with Adriamycin (four cycles of 21 days). One year after surgery the patient is tumour free.

Immunopathology of an hDAF transgenic pig model liver xenotransplant into a primate.

BACKGROUND: hDAF transgenic pigs do not display the inherent hyperacute rejection reactions of pig-to-primate xenotransplants. The purpose of this study was to determine the immunopathologic phenomena following an hDAF transgenic pig hepatic orthotopic xenotransplant into a baboon. METHODS: Donor animals were unmodified pigs (n=4) and hDAF transgenic pigs (n=2). Recipient animals were baboons (Papio anubis). Liver biopsies were immunostained using monoclonal antibodies to C3, C5b-9, IgG, IgM, CD2, CD4, CD8, CD68, CD20, Bric 216, CD31, and fibrin, and polyclonal antibody to C4. RESULTS: hDAF transgenic grafts showed IgG, IgM, and C4 endothelial deposits. However, no fibrin, C3, or C5b9 deposits were observed after reperfusion. hDAF xenografts displayed CD31 staining in the portal spaces, perilobular areas, and at hepatic sinuisoidal levels. The baboon that lived for 4 days displayed either CD4 or CD8 T-cells periportal infiltrate. CONCLUSIONS: Future studies will seek to determine the physiologic role of CD31 hepatic sinusoidal expression in transgenic xenotransplants, and will also study the role of T-cell infiltrates in xenograft rejection.

Diagnosis and treatment of oculopharyngeal dystrophy: a report of three cases from the same family.

Oculopharyngeal muscular dystrophy is a hereditary pathology transmitted in an autosomal dominant manner. The clinical symptoms are palpebral ptosis, oropharyngeal dysphagia and proximal limb weakness. Upper gastro-esophageal endoscopy is recommended to study the dysphagia, a video-radiology study with barium and an esophageal manometry to study the pharyngeo-esophageal motor disorder. Muscle biopsy reveals the presence of atrophic fibers substituted by an increase in fat and connective tissue. In 1998 Brais described the genetic alteration responsible for this pathology, a limited expansion of the triplet of GCG nucleotides in PABP2 gene on chromosome 14q11. Normal individuals have the homozygotic form (GCG)6 of this triplet, whereas patients with the described syndrome have the heterozygotic form (GCG)6-(GCG)9 or (GCG)6-(GCG)10. We present three siblings from the same family with diagnoses and genetic confirmations of oculopharyngeal dystrophy. Two of the patients underwent cricopharyngeal myotomy to relieve the dysphagia.

Primary eosinophilic esophagitis.

Eosinophilic esophagitis is an uncommon pathology that generally affects children with a history of allergies and intrinsic asthma. We present a clinical case of eosinophilic esophagitis in a 16-year-old boy with upper dysphagia for solids since childhood. The analytical study showed only a repeat serum eosinophilia. Barium transit disclosed a reduction in caliber of the whole esophagus. Functional esophageal tests with pH monitoring and manometry were normal. Endoscopy showed a small-diameter esophagus and fibrosis with a very friable mucosa. The histological study of the esophageal biopsies revealed a full thickness major eosinophil infiltration of the esophagus. These findings suggest a differential diagnosis with a great variety of pathologies that can cause similar lesions in the esophagus, especially between primary eosinophilic esophagitis and eosinophilic esophagitis secondary to gastro-esophageal reflux disease (GERD). We implemented medical treatment with oral corticoids and total suppression of allergens from the diet, and the patient was asymptomatic.

Intestinal metaplasia in patients with columnar lined esophagus is associated with high levels of duodenogastroesophageal reflux.

OBJECTIVE: To evaluate the rate of duodenogastroesophageal reflux in patients with columnar lined esophagus compared with patients with gastroesophageal reflux disease without columnar lined esophagus, and to analyze whether it is related to the presence of specialized columnar epithelium in the metaplastic segment. SUMMARY BACKGROUND DATA: The carcinomatous degeneration of columnar lined esophagus originates from a specialized columnar epithelium. The appearance of this metaplastic phenomenon is clearly related to severe prolonged gastroesophageal reflux, but only some of these patients finally develop columnar lined esophagus. For this reason other factors have been suggested, particularly the role played by the reflux of duodenal contents into the esophagus. METHODS: The authors studied 15 healthy volunteers (control group), 10 patients with reflux symptoms but without endoscopic lesions, 20 patients with reflux esophagitis without columnar lined esophagus, and 35 patients with columnar lined esophagus (complicated with ulcers or stenosis in 8 cases), of whom 22 had intestinal metaplasia. To assess the reflux of duodenal contents into the esophagus, all the patients underwent Bilitec 2000 and 24-hour esophageal pH monitoring. RESULTS: The presence of bilirubin in the material refluxed into the esophagus was greater in the patients with columnar lined esophagus than in the rest of the groups. Likewise, duodenogastroesophageal reflux was greater in the columnar lined esophagus patients who had intestinal metaplasia. CONCLUSIONS: Duodenogastroesophageal reflux may play a major role in the development of columnar lined esophagus, especially in patients with intestinal metaplasia.