[Spontaneous rupture of a superior mesenteric artery: report of two cases]. / Rupture spontanée d'un anévrisme de l'artère mésentérique supérieure : à propos de deux cas.

Superior mesenteric artery aneurysms are rare. Diagnosis and treatment are a real challenge. We report two cases of this type of vascular anomaly revealed by spontaneous rupture. The diagnosis was made by abdominal computed tomography angiography and treatment consisted in aneurysmorraphy in one case and bowel resection with the distal aneurysm in the second.

T-tube drainage of the common bile duct choleperitoneum: etiology and management.

External drainage of the common bile duct by placement of a T-tube is a common practice after choledochotomy. This practice may result in the specific complication of bile peritonitis due to leakage after removal of the T-tube. This complication has multiple causes: some are patient-related (corticotherapy, chemotherapy, ascites), and others are due to technical factors (inappropriate suturing of the drain to the ductal wall, minimal inflammatory reaction related to some drain materials). The clinical presentation is quite variable depending on the amount and rapidity of intra-peritoneal spread of of bile leakage. Abdominal ultrasound (US), with US-guided needle aspiration and occasionally Technetium(99) scintigraphy are useful for diagnosis. Traditional therapy consists of surgical intervention including peritoneal lavage and re-intubation of the choledochal fistulous tract to allow for a further period of external drainage. When leakage is walled off and well-tolerated, a more nuanced and less invasive conservative therapy may combine percutaneous drainage with endoscopic placement of a trans-ampullary biliary drainage.

Gas gangrene of the abdominal wall due to late-onset enteric fistula after polyester mesh repair of an incisional hernia.

The occurrence of enteric fistulae after wall repair using a prosthetic mesh is a serious but, fortunately, rare complication. We report the case of a 66-year-old diabetic man who presented with gas gangrene of the abdominal wall due to an intra-abdominal abscess caused by intestinal erosion six years after an incisional hernia repair using a polyester mesh. The aim of this case report is to illustrate the seriousness of enteric fistula after parietal repair using a synthetic material.

Surgical treatment of late dysfunction of an esophagocoloplasty.

Replacement of the esophagus by colon interposition often has late complications, frequently due to technical defects. We report the case of a patient who presented 12 years after surgery with complex dysfunctions of a colonic interposition, including cervical and retrosternal strictures associated with a redundant portion of the colon graft. The interest of this case lies in its combination of many late complications of this surgery in a single person together with the simplicity of the treatment.

Rectal leiomyosarcoma: report on two cases and a practical approach to differential diagnosis.

Rectal leiomyosarcoma is an uncommon malignancy. Herein, we describe the clinicopathological features and biological behaviour of these tumours, and provide a practical approach to differential diagnosis, particularly with gastrointestinal stromal tumours (GIST). We report two cases in elderly men. In the first, the lesion was 2 cm from the anal sphincter, while it was located in the rectal ampulla in the second case. Histologically, both tumours were characterized by pleiomorphic, large spindle cells, presenting numerous mitoses and marked nuclear atypia. Immunohistochemical analysis showed that tumour cells coexpressed both actin and desmin, whereas CD117 and S100 protein were negative. The final diagnosis was leiomyosarcoma. One of the patients died of pulmonary metastasis within six months. The second patient had bone metastasis, but was lost to follow up. This report underlines the potential diagnostic problems raised by rectal leiomyosarcoma and emphasizes the role of immunohistochemistry in achieving correct diagnosis, which has important clinical, therapeutic and prognostic consequences.

A benign cystic mass of the pancreas mimicking a malignant lesion.

Herein, we highlight the diagnostic challenges of cystic pancreatic tumours, and report a case of chronic pancreatitis caused by a cystic tumour, which consisted in a canal dilatation--and not a pseudocyst. The case thus demonstrates a rare association between a cystic form of chronic pancreatitis and adrenal adenoma. We report the case of a 46-year-old patient with no particular past medical history who presented with long lasting symptoms consisting in an abdominal pain and deterioration in general health. Imaging findings (ultrasound, CT-scan, MRI) showed a 3-cm cystic lesion of the tail of the pancreas associated with a 3-cm adrenal mass. Because of the suspicion of a malignant disease, surgical treatment was performed. Pathological findings consisted in fibrotic chronic pancreatitis with canal dilatation and an adrenal adenoma. Pancreatic cystic lesions are rare tumours. Despite of the multiplicity of imaging techniques, differential techniques lack sensitivity and specificity. Final diagnosis must be based on pathological features.

[Beta 2-microglobulin amyloidosis presenting as intestinal perforation in a haemodialysis patient]. / Amyloïdose de type beta2-microglobuline révélée par une perforation intestinale chez une hémodialysée chronique.

Long term haemodialysis patient is subject to several complications such as generalised amyloidosis which is the result of deposits of beta2-microglobulin not depurated by haemodialysis. Digestive location causes ischemic accidents such as ulcer, infarctus, digestive haemorrhage, pseudo-obstruction and perforation manifested by a surgical emergency. Our observation is the 6th case of intestinal perforation caused by amyloidosis deposit reported in the literature.

[Extragastrointestinal stromal tumors: a report of 4 cases]. / Les tumeurs stromales extradigestives: à propos de quatre observations.

Gastrointestinal stromal tumors (GIST) are mesenchymal tumors that arise from the wall of the gastrointestinal tract expressing CD117 and/or the CD34. Similar tumors were described in the soft tissue of the abdomen and are so-called extragastrointestinal stromal tumors (EGIST). We report 4 new cases of EGIST. The tumors occurred in 2 women and 2 men, who ranged in age from 42 to 71 years. Three tumors arose from the soft tissue of the abdominal cavity, and the remainder arose from the retroperitoneum. They ranged in size from 10 to 27 cm. Three cases were composed purely of short fusiform cells tumors, the last case showed an epithelioid pattern. All the tumors expressed CD117. One patient presented with local recurrence and metastasis, one was lost to the follow-up and two patients were doing well. Immunohistochemistry is useful in distinguishing EGIST from other mesenchymal tumors. Intra-abdominal aggressive fibromatosis may express actin and CD117 but catenin is also positive, leiomyosarcoma expresses the actin and/or desmin but CD117 is usually negative, retroperitoneal dedifferentiated liposarcoma is CD117 negative and PS100 positive, inflammatory myofibroblastic tumor is negative for CD117 and CD34. The solitary fibrous tumor expresses CD34 and is negative to CD117. Some tumors expressing CD117 such desmoplastic round cell tumor should not be confused with EGIST. On the occasion of these four observations, we will discuss the clinical aspects and the main differential diagnoses of this tumor.

[Functional outcome after resection followed by a coloanal anastomosis for cancer of the rectum]. / Resultats fonctionnels des resections avec anastomose colo-anale dans les cancers du rectum.

The aim of the study was to asseses the operative, functional results of total proctectomy with coloanal anastomosis (CAA). Between 1990 and 2002, 24 patients (14 males and 10 females ) with a mean age of 50,6 years were operated on for cancer of the rectum and they underwent a total proctectomy with CAA. There was one operative death. 3 patients developed non specific complications. 4 patients experienced a supra anastomosis stenosis and underwent a new straight CAA Functional outcome were evaluated by a scoring system fonction and were considered good in 87 and 84% at 1 and 2 years respectively. Low anterior resection combined with CAA provides good treatment for mid-rectal cancers and for some distal rectal cancers. It had a good functional outcome. However Both procedures and selection of patients who underwent radiotherapy must be carefully performed.

Early feeding resistance: a possible consequence of neonatal oro-oesophageal dyskinesia.

UNLABELLED: Paediatricians frequently find early feeding disorders in neonates and infants that result in poor weight gain and which sometimes have no clear organic basis. For many years, we have observed infants with unexplained poor feeding skills and excessive regurgitation, and since 1992 we have prospectively performed oesophageal manometry in infants hospitalized for retarded growth and "unexplained" feeding disorders. From the group of infants hospitalized for growth failure in the General Paediatric Unit of Necker-Enfants Malades Hospital from 1992 to 1997, we identified 16 children (3%) with abnormal feeding behaviour who had an abnormal oesophageal manometry. The manometric data of these children were compared with those of a group of 16 age-matched children who underwent oesophageal manometry for other reasons, and served as controls. The affected children had precocious feeding skills disorders: prolonged bottle-feeding (75%), bottle refusal (75%), unexplained crying (63%) and excessive regurgitation (94%). Half of them had mild anatomical facial consequences of their poor foetal sucking, and mild pharyngolaryngeal hypotonia, which could not be considered as malformations. Apart from these disorders, their clinical status was normal. At inclusion, their oesophageal manometry was abnormal, showing in 70% of cases specific anomalies: lower oesophageal sphincter hypertonia and/or partial failure to relax, and giant waves of oesophagus body. Their course was good and their feeding difficulties decreased around the end of the first year, with the acquisition of normal voluntary mastication. CONCLUSION: We suggest that the early feeding resistance of this group of children could be related to an organic and transient neonatal oro-oesophageal dyskinesia.

Oroesophageal motor disorders in Pierre Robin syndrome.

BACKGROUND: Feeding disorders are one of the main clinical features in PRS, which combines a posterior U-shaped cleft palate, retrognathia, and glossoptosis. The aim of this study was to evaluate the oral and esophageal motor function of children with PRS without additional neurologic symptoms. METHODS: All children hospitalized with Pierre Robin syndrome either isolated (n = 27) or associated with Stickler syndrome (n = 8) were included. Clinical evaluation of their oroesophageal disorders and systematic esophageal manometry were performed. RESULTS: Feeding disorders were always present, but type of disorder varied from one child to another. Esophageal disorders were frequent and seemed to be resistant to classic gastroesophageal reflux treatment. Eighty-six percent of the children required nasogastric tube feeding for a mean duration of 8.6 months. Esophageal manometric abnormalities were noted in 50% of the children: lower esophageal sphincter hypertonia, failure of lower esophageal sphincter relaxation at deglutition, and esophageal dyskinesia. These clinical and manometric disorders showed a trend to spontaneous regression after 12 months. CONCLUSION: In the current Pierre Robin syndrome series, clinical and manometric anomalies of oroesophageal motility were always present. The authors identified an unusual manometric pattern that has also been described in situations of neurovegetative instability. It could reflect dysregulation of the control of the central pattern generators of swallowing in the brain stem.