Bowel perforation in type IV vascular Ehlers-Danlos syndrome. A systematic review.

Spontaneous gastrointestinal (GI) perforation is a well-known complication occurring in patients suffering from Type IV vascular Ehlers-Danlos syndrome (EDS IV). The aim of the present study was to review the current literature on spontaneous GI perforation in EDS IV and illustrate the surgical management and outcome when possible. A systematic review of all the published data on EDS IV patients with spontaneous GI perforation between January 2000 and December 2015 was conducted using three major databases PUBMED, EMBASE, and Cochrane Central Register of Controlled Trails. References of the selected articles were screened to avoid missing main articles. Twenty-seven published case reports and four retrospective studies, including 31 and 527 cases, respectively, matched the search criteria. A case from our institution was added. Mean age was 26 years (range 6-64 years). The most frequent site of perforation was the colon, particularly the sigmoid, followed by small bowel, upper rectum, and finally stomach. The majority of cases were initially managed with Hartmann's procedure. In recurrent perforations, total colectomy was performed. The reperforation rate was considerably higher in the "partial colectomy with anastomosis" group than in the Hartmann group. Colonic perforation is the most common spontaneous GI perforation in EDS IV patients. An unexpected fragility of the tissues should raise the possibility of a connective tissue disorder and prompt further investigation with eventual management of these high-risk patients with a multidisciplinary team approach in dedicated centres. In the emergency setting, a Hartmann procedure should be performed.

Technical difficulties of left colic artery preservation during left colectomy for colon cancer.

PURPOSE: Low-tie ligation in colorectal cancer surgery is associated with technical difficulties in left colic artery preservation. We aimed to evaluate and classify the anatomical and technical difficulties of left colic artery (LCA) preservation at its origin and along its route at the inferior border of the pancreas. METHODS: A vascular reconstruction computed tomography prospective series of 113 patients was analyzed. The inferior mesenteric artery (IMA) branching pattern according to Latarjet's classification (Type I, separate LCA origin, Type II, fan-shaped branching pattern) and the distances between the IMA and the LCA origins and between the LCA and the Inferior mesenteric vein (IMV) at the inferior border of the pancreas were measured. RESULTS: The IMA branching pattern was Type I in 80 (71 %) patients and Type II in 33 (29 %) patients. The IMA-LCA distance was 39.8 ± 12.2 mm. The LCA-IMV distance at the inferior border of the pancreas was 20.5 ± 21.7 mm. When classified based on this distance, 75 (66 %) patients were classified into the Near subgroup (<20 mm) (7.7 ± 4.1 mm) and 38 (34 %) into the Far subgroup (≥20 mm) (45.6 ± 20.4 mm, p < 0.001). A Type I subgroup F accounted for 27 % of the patients. CONCLUSIONS: Left colic artery preservation is highly feasible at its origin in more than two-thirds of cases due to the separate origin. The addition of a high IMV ligation increases the risk of damage to the LCA at the inferior border of the pancreas because the distance to the IMV is less than 20 mm in two-thirds of cases.

Real time shear waves elastography monitoring of thermal ablation: in vivo evaluation in pig livers.

BACKGROUND: Thermal ablation is a widely used minimally invasive treatment modality for different cancers. However, lack of a real-time imaging system for accurate evaluation of the procedure is one of the reasons of local recurrences. Shear waves elastography (SWE) is a new ultrasound (US) imaging modality to quantify tissue stiffness. The aim of the study was to assess the feasibility and accuracy of US elastography for quantitative monitoring of thermal ablation and to determine the elasticity threshold predictive of coagulation necrosis. METHODS: A total of 29 in vivo thermal lesions were performed in pig livers with radiofrequency system. SWE and B-mode images were acquired simultaneously. Liver elasticity was quantified by using SWE data and expressed in kilopascal. After the procedure, pathologic analysis of treated tissues was compared with US images. The sensitivity and positive predictive value of the SWE maps of tissue elasticity were calculated and compared with the boundaries of the pale coagulation necrosis areas found at pathology. RESULTS: The liver mean elasticity values before and after thermal therapy were 6.4 ± 0.3 and 38.1 ± 2.5 kPa, respectively (P < 0.0001). For a threshold of 20 kPa, sensitivity (i.e., the rate of pixels correctly detected as necrosed tissue) was 0.8, and the positive predictive value (i.e., the rate of pixels in the elastographic map >20 kPa that actually developed coagulation necrosis) was 0.83. CONCLUSIONS: Tissue areas with coagulation necrosis are significantly stiffer than the surrounding tissue. SWE permits the real-time detection of coagulation necrosis produced by radiofrequency and could potentially be used to monitor US-guided thermal ablation.

Outcomes of adrenalectomy in patients with unilateral primary aldosteronism: a review.

Aldosterone hypersecretion in primary aldosteronism is unilateral (aldosterone producing adenoma and primary unilateral hyperplasia) or bilateral (idiopathic adrenal hyperplasia). Laparoscopic adrenalectomy is nowadays the preferred approach to treat patients with unilateral primary aldosteronism. We review the outcomes of this intervention in recently published series. Laparoscopic adrenalectomy has a morbidity of 5-14%, mortality below 1%, and a mean hospital stay around 3 days. It generally results in the normalization of aldosterone secretion and in a large decrease of blood pressure and antihypertensive medication, but normotension without treatment is only achieved in 42% of all cases. Normotension following adrenalectomy is more likely in young and lean women with recent low grade hypertension than in obese men with long-standing high grade hypertension or a family history of hypertension. However, individual prediction of the blood pressure outcome is not accurate and predictors of hypertension cure should not be used to select patients for surgery. Age, associated health conditions and preferences of the patient are more relevant to this end.

[Phaeochromocytoma and paraganglioma]. / Phéochromocytome et paragangliome.

Phaeochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors that arise from the adrenal medulla or sympathetic and parasympathetic ganglia. These tumors produce most often catecholamines in excess, causing hypertension and sometimes severe acute cardiovascular complications. The diagnosis is based on plasma or urines metanephrines measurements and on conventional and nuclear medicine imaging. Catecholamines-producing PPGL is very unlikely if levels are normal. The diagnosis of PPGL cannot be made without visualization of a tumor. Therapeutic management consists mostly of surgical excision, after drug preparation, and should be done in referral centers. About 40% of pheochromocytomas and paragangliomas occur in the context of an autosomal inherited syndrome, making genetic testing essential. The follow-up must be prolonged because a metastatic evolution or a recurrence can be observed in about 15% of the cases.

[Not Available]. / Implications immunologiques potentielles du curage ganglionnaire : Exemple du cancer colorectal.

F. Pagès, A. Berger, F. Zinzindohoué, A. Kirilovsky, J. Galon, W.-H. Fridman Lymph node dissection is an integral part of the surgical resection of colon cancers; it completes the wide regional resection of tumor and it allows prognostic evaluation through accurate staging. Studies have demonstrated an immune reaction to the tumoral site which attests to an ongoing dialog between the tumor and systemic defenses. The regional lymph nodes constitute an important first line of immune defense where initial host response is initiated or, inversely, they may participate in a local state of immunosuppression. This article reviews current knowledge on intra-tumoral and nodal immune status in colorectal cancers and attempts to evaluate the potential immunologic implications of lymph node dissection.

[Not Available]. / Implications immunologiques potentielles du curage ganglionnaire : Exemple du cancer colorectal.

F. Pagès, A. Berger, F. Zinzindohoué, A. Kirilovsky, J. Galon, W.-H. Fridman Lymph node dissection is an integral part of the surgical resection of colon cancers; it completes the wide regional resection of tumor and it allows prognostic evaluation through accurate staging. Studies have demonstrated an immune reaction to the tumoral site which attests to an ongoing dialog between the tumor and systemic defenses. The regional lymph nodes constitute an important first line of immune defense where initial host response is initiated or, inversely, they may participate in a local state of immunosuppression. This article reviews current knowledge on intra-tumoral and nodal immune status in colorectal cancers and attempts to evaluate the potential immunologic implications of lymph node dissection.

[Potential immunologic consequences of lymph node dissection in colorectal cancer]. / Implications immunologiques potentielles du curage ganglionnaire: Exemple du cancer colorectal.

Lymph node dissection is an integral part of the surgical resection of colon cancers; it completes the wide regional resection of tumor and it allows prognostic evaluation through accurate staging. Studies have demonstrated an immune reaction to the tumoral site which attests to an ongoing dialog between the tumor and systemic defenses. The regional lymph nodes constitute an important first line of immune defense where initial host response is initiated or, inversely, they may participate in a local state of immunosuppression. This article reviews current knowledge on intra-tumoral and nodal immune status in colorectal cancers and attempts to evaluate the potential immunologic implications of lymph node dissection.

Professional risks when carrying out cytoreductive surgery for peritoneal malignancy with hyperthermic intraperitoneal chemotherapy (HIPEC): A French multicentric survey.

BACKGROUND: Over the last two decades, many surgical teams have developed programs to treat peritoneal carcinomatosis with extensive cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC). Currently, there are no specific recommendations for HIPEC procedures concerning environmental contamination risk management, personal protective equipment (PPE), or occupational health supervision. METHODS: A survey of the institutional practices among all French teams currently performing HIPEC procedures was carried out via the French network for the treatment of rare peritoneal malignancies (RENAPE). RESULTS: Thirty three surgical teams responded, 14 (42.4%) which reported more than 10 years of HIPEC experience. Some practices were widespread, such as using HIPEC machine approved by the European Community (100%), individualized or centralized smoke evacuation (81.8%), "open" abdominal coverage during perfusion (75.8%), and maintaining the same surgeon throughout the procedure (69.7%). Others were more heterogeneous, including laminar flow air circulation (54.5%) and the provision of safety protocols in the event of perfusate spills (51.5%). The use of specialized personal protective equipment is ubiquitous (93.9%) but widely variable between programs. CONCLUSION: Protocols regarding cytoreductive surgery/HIPEC and the associated professional risks in France lack standardization and should be established.

Proinflammatory and antitumor properties of interleukin-18 in the gastrointestinal tract.

Interleukin-18 (IL-18) plays a central role in the immune response by acting on Th1 cell differentiation, cell-mediated cytotoxicity and inflammation. The role of IL-18 in cancers and inflammatory diseases is discussed in the light of our investigations on IL-18 synthesis in normal colonic mucosa, colonic cancer and Crohn's disease (CD).

Adjustable gastric banding in a public university hospital: prospective analysis of 400 patients.

BACKGROUND: Laparoscopic application of an adjustable gastric band (LAGB) is considered the least invasive surgical option for morbid obesity. It has the advantage of being potentially reversible and can improve quality of life. METHOD: Between April 1997 and January 2001, 400 patients underwent LAGB. There were 352 women and 48 men with mean age 40.2 years (16-66). Preoperative mean body weight was 119 kg (85-195) and mean body mass index (BMI) was 43.8 kg/m2 (35.1-65.8). RESULTS: Mean operative time was 116 minutes (30-380), and mean hospital stay was 4.55 days (3-42). There was no death. There were 12 conversions (3%). 40 complications required an abdominal reoperation (10%), for perforation (n = 2), gastric necrosis (n = 1), slippage (n = 31), incisional hernia (n = 2) and reconnection of the tube (n = 4). We noticed 7 pulmonary complications (2 ARDS, 5 atelectasis) and 30 minor problems related to the access port. At 2 years, mean BMI had fallen from 43.8 to 32.7 kg/m2 and mean excess weight loss (EWL) was 52.7% (12-94). CONCLUSION: LAGB is a very beneficial operation with an acceptable complication rate. EWL is 50% at 2 years if multidisciplinary follow-up remains assiduous. Surveillance for late anterior stomach slippage within the band is essential.

Results of re-resection for recurrent thymomas.

BACKGROUND: The treatment of recurrent thymomas remains controversial. PATIENTS: The place for re-resection was retrospectively studied in 28 consecutive patients operated on during the last 40 years. The initial Masaoka staging of the thymoma was stage I, 4; stage II, 8; stage III, 11; and stage IVa, 3. Postoperatively, 14 have had radiation therapy, 1 chemotherapy, and 13 no adjuvant treatment. Seven patients had development of recurrences, 15 had pleuropulmonary metastases, 5 had both, and 1 had thoracotomy scar recurrence. Nineteen patients had a complete resection and 9 an incomplete one. RESULTS: Most local recurrences appeared after resection of stage I or II thymomas. On the other hand, in patients with stage III or IV thymomas pleural or pulmonary metastases mainly developed. No local recurrence occurred in patients who initially received postoperative radiation therapy. Five-year and 10-year survival rates were 51% and 43%, respectively, for the overall population. Among the 19 patients with complete resection, only 3 patients had a subsequent recurrence; 1 of them could be reoperated on and is still alive and free of disease. CONCLUSIONS: Thymoma recurrences often appear as a locoregional rather than a hematogenous spread. Reresection can be recommended in selected patients.

Interleukin-18: biological properties and clinical implications.

IL-18, originally identified as interferon-gamma inducing factor (IGIF), is related to the IL-1 family in terms of its structure, processing, receptor, signal transduction pathway and pro-inflammatory properties. IL-18 is also functionally related to IL-12, as it induces the production of Th1 cytokines and participates in cell-mediated immune cytotoxicity. This review summarizes the recent advances in the understanding of IL-18 structure, processing, receptor expression and immunoregulatory functions, and focuses on the role of IL-18 modulation in tumours, infections, and autoimmune and inflammatory diseases.

Analysis of interleukin-18, interleukin-1 converting enzyme (ICE) and interleukin-18-related cytokines in Crohn's disease lesions.

A local increase of interleukin-18 (IL-18) expression has been recently demonstrated in Crohn's disease (CD), suggesting a role for mature IL-18 (cleaved by ICE protease) in the induction of proinflammatory cytokines and Th1 polarization observed in CD lesions. The aim of this study was to investigate IL-18 modulation and its potential immune consequences in CD lesions. We showed increased IL-18 production in chronic CD lesions and identified epithelial cells and macrophages as IL-18-producing cells. A twofold increase in ICE alpha, beta, and/or gamma mRNA that encodes for the complete mature peptide was required for ICE activity, and a marked increase in IL-18R-positive immune cells was observed in chronic lesions compared to uninvolved areas or normal control samples. Chronic lesions also displayed intense transcription of IL-18-induced cytokines, IFN-gamma, IL-1beta, TNF-alpha, and IL-8. By contrast, when neither IL-18 nor ICE mRNAs were enhanced (early asymptomatic CD lesions), IL-18-induced cytokines were not up-regulated. These results are in accordance with a putative role of mature IL-18 in the pathogenesis of CD.

Allogeneic fetal small bowel graft in pigs treated with cyclosporin A.

BACKGROUND: The functional integrity of transplanted fetal intestine was proven in rodents. The authors examined the morphology and development of intraperitoneally transplanted fetal intestine under cyclosporin A (CsA) monotherapy in a large mammal. METHODS: Allogeneic fetal intestinal grafts were transplanted intraperitoneally in pigs. The graft was wrapped in omentum. Thirteen recipients received grafts harvested at 60 days of gestation and 5 at 105 days of gestation. All recipients received 25 mg/kg/d CsA. CsA blood levels were measured at the end of the study. The development of the grafts was assessed by inspection and histology studying revascularization, maturation, and immune rejection. RESULTS: All grafts developed neovascularization. The intestinal wall in the 105-day-old group was thick enough to lead to complete mucosal destruction, whereas the 60-day-old group showed viable mucosa. All grafts induced an immune rejection. This immune response was correlated with the CsA blood level. The graft was destroyed within 15 days when CsA trough level was below 70 ng/mL, had a subacute rejection with villi atrophy when CsA trough level ranged from 70 to 150 ng/mL, and had a good appearance in spite of mild blunting of villi when CsA trough level was over 150 ng/mL. CONCLUSION: Allogeneic fetal intestinal transplantation from 60-day-old embryos in pig achieved successful graft.

[Surgical morbidity of segmental colectomy ideally performed via laparotomy for complicated colonic diverticulosis]. / Morbidité opératoire de la colectomie segmentaire idéale faite par laparotomie pour diverticulose colique compliquée.

OBJECTIVES: The appraisal of morbidity and mortality for one stage elective colectomy for complicated diverticulosis is difficult and often overestimated, due to the rarity of reports addressing this question. Our results for 100 patients on a recent 30 month period were studied retrospectively. METHODS: One hundred patients were electively operated in a one-stage procedure for complicated diverticulosis in a single institution from January 1993 to June 1995. There were 66 females and 34 males (range: 31-81 years) with a mean age of 61 years. Main indications for surgery were repeated attacks (34 patients), chronic inflammatory mass (26 patients) and stenosis (22 patients). Seventy-eight patients had already been admitted for diverticulitis prior to surgery. There were 13 surgeons including 6 seniors and 7 fellows. RESULTS: There was no mortality. Morbidity was 14% surgical and medical complications accounting for 8% and 6% respectively. One patient had an anastomotic fistula treated conservatively and another patient was reoperated on for early postoperative occlusion There was no perioperative bleeding requiring transfusion. There were no surgical trauma of spleen or uretera. Mean hospital stay was 10 days. CONCLUSION: This study of a collective surgical experience demonstrates that elective one stage left colectomy for benign disease is safe, without mortality and with low morbidity.

[Acute pancreatitis revealing cystic fibrosis in an adult]. / Pancréatite aiguë révélatrice de mucoviscidose chez une adulte.

We report a case of pancreatitis as the first symptom of cystic fibrosis in a 22-year-old woman. The diagnosis was suspected upon the history of nasal polyposis and severe episodes of respiratory infections in infancy. The diagnosis was confirmed by sweat test. Genotyping showed a compound heterozygosity for mutations delta F508 and 5T. Acute pancreatitis is a rare manifestation of cystic fibrosis and an exceptional mode of initial presentation of the disease. It occurs in pancreatic sufficient patients, especially in young adults. This exceptional case shows that cystic fibrosis should be suspected in each case of idiopathic pancreatitis. Indications of cholecystectomy in patients with associated gallbladder lithiasis is discussed.

[A radical treatment of villous tumors of the duodenum]. / Pour un traitement radical des nappes villeuses du duodénum.

Three cases of large villous tumors of the second part of the duodenum treated by pancreaticoduodenectomy are reported. These lesions are in frequent and premalignant. Their treatment can be either local, endoscopic or surgical, or radical. The malignancy of the tumors is often missed preoperatively. The frequent recurrence after local excision and the theoretical risk of lymph node invasion even for carcinoma in situ compared with the low operative risk of whipple's procedure in the authors' experience lead them to propose radical treatment for extensive villous tumors of the second part of the duodenum.

[Rectovaginal fistulas in adults]. / Fistules recto-vaginales de l'adulte.

Recto-vaginal fistulas have multiple causes and a wide range of clinical and anatomical features. Simple fistulas, defined by a low situation, a traumatic origin, and a small size are accessible to simple means of cure. They can be operated from a vaginal approach, with conversion into a third degree perineal tear followed by repair of the perineal body, and the anal canal; they can be managed from a transanal approach, using endorectal flap advancement technique. Complex fistulas, defined by the etiology (IBD, radiation enteritis, cancer, postoperative), a high situation, or a large size, require larger and more sophisticated operations such as a combined abdomino-perineal approach or a muscle flap technique. Among the numerous techniques described for the cure of recto-vaginal fistulas, the authors emphasize those currently used by the most experienced teams of colo-rectal or gynecological surgeons.

[Cystic dystrophy of the duodenal wall in aberrant pancreas]. / Dystrophie kystique sur pancréas aberrant de la paroi duodénale.

Cystic dystrophy of the duodenal wall developing in heterotopic pancreas is rare. Pancreaticoduodenectomy is usually advocated in cases of symptomatic cystic dystrophy without chronic pancreatitis but little is known about long-term results. We report a case of cystic dystrophy of the duodenum wall developing in heterotopic pancreas responsible for abdominal pain and duodenal obstruction associated with chronic pancreatitis. Pancreaticoduodenectomy allowed complete relief of symptoms. Chronic pancreatitis associated with cystic dystrophy may complicate the choice of appropriate therapy.