Undifferentiated embryonal sarcoma of the liver: a case report of a rare case in an adult patient.

Undifferentiated embryonal sarcoma of the liver is a rare primary mesenchymal hepatic tumor that usually occurs in pediatric patients. In adulthood, this aggressive neoplasm represents only 7% of the liver sarcomas. This case reports a liver sarcoma occurring in a 49-year-old female patient. The patient was admitted in the emergency room with abdominal pain. Computerized tomography scan and magnetic resonance imaging showed a giant lobulated cystic mass in the right hepatic lobe, suggesting an atypical hemangioma. Right hepatectomy was performed. This rare case promotes a review of the differential diagnosis of liver primary neoplasms including sarcoma. The histological examination revealed an undifferentiated embryonal sarcoma. The patient underwent adjuvant chemotherapy. Currently, our patient is in complete sustained remission 4 years after chemotherapy.

Diaphragmatic hernia as a rare cause of bowel obstruction.

Mechanical intestinal obstruction is a common cause of acute abdominal pain that brings patients to the emergency department. One of the main causes is adhesion in the abdomen after abdominal surgery, but rarer causes exist and are a diagnostic challenge due to the similarity of the presenting symptoms. Here, we present a case of intestinal obstruction caused by diaphragmatic hernia.

Low-Grade Dysplastic Intracholecystic Papillary Neoplasia: A Case Report.

BACKGROUND The World Health Organization classification of premalignant gallbladder lesions includes adenomas, intraductal papillary neoplasms, biliary intraepithelial neoplasia, and intracystic papillary neoplasms. Noninvasive neoplastic lesions >1 cm that originate from the pancreatobiliary system are defined as intraductal papillary neoplasia when they occur in the biliary ducts. The clinical and pathological features of preinvasive lesions arising in the gallbladder are not yet well defined. However, the most widely accepted classification is that of intracholecystic papillary neoplasm (ICPN). CASE REPORT We present the case of a 71-year-old woman referred to a General Surgery outpatient clinic for suspicious findings on imaging of the gallbladder, namely irregular infundibular parietal thickening. The patient underwent a laparoscopic cholecystectomy and histological examination revealed a thickened gallbladder with mucosa partially surrounded by ICPN with an intestinal pattern and some foci of low-grade dysplasia but no foci of high-grade dysplasia or invasive neoplasia. At follow-up at 30 months, the patient remains clinically well, with no changes visible on computed tomography scan. CONCLUSIONS ICPN of the gallbladder appears to be part of a spectrum of alterations encompassing bile duct or pancreatic lesions. Although it is uncommon, more than half of the lesions are known to have foci of invasive neoplasia at the time of diagnosis. Despite that, the prognosis for these neoplasms is more favorable than for gallbladder neoplasia that originates from another type of lesion. Pathological study of ICPN is essential to define the main characteristics that impact prognosis and survival in these patients.

Intrathoracic Acute Cholecystitis.

The authors present the case of a 51-year-old woman with no history of surgical or traumatic injury or accident, who presented with right hypochondrium and epigastric discomfort, malaise, nausea, loss of appetite and episodes of dark urine and greenish stools. Initial laboratory work-up revealed elevated inflammatory markers including leucocytosis with left shift and C-reactive protein, and a slight elevation of gamma-glutamyltransferase and alkaline phosphatase, with no other significant alterations. Computed tomography (CT) showed intrathoracic acute cholecystitis with a large diaphragmatic hernia. A literature search revealed only one other case of acute cholecystitis complicated by intrathoracic gallbladder due to a non-traumatic diaphragmatic hernia. Symptoms are uncharacteristic and the absence of pain or fever, explained by the altered location of the gallbladder, makes the diagnosis a challenge. LEARNING POINTS: Only one other case of acute cholecystitis complicated by intrathoracic gallbladder due to a non-traumatic diaphragmatic hernia has been reported.Uncharacteristic symptoms make the diagnosis of intrathoracic acute cholecystitis a challenge.

Duodenal neuroendocrine tumour in a young patient with von Recklinghausen disease.

Von Recklinghausen disease (neurofibromatosis type 1-NFT1) is a genetic disorder with autosomal dominant inheritance pattern, caused by mutation of a tumour suppressor gene. Its main features include multiple cutaneous café-au-lait spots and neurofibromas. It is associated with an increased risk of developing neuroendocrine tumours, for instance, in the duodenum. The authors present a case of a 23-year-old male patient admitted to the emergency department due to persistent vomiting. Imaging and biopsy studies revealed an obstructive and large duodenal neuroendocrine tumour; hence the patient underwent a pancreaticoduodenectomy.

Laparoscopic approach for a spontaneous posterior rectus sheath hernia.

Spontaneous posterior rectus sheath hernia is perhaps one of the rarest hernias described only in a few published cases. We present the case of a small bowel herniation through the posterior rectus sheath in a woman without previous abdominal surgical history, resulting in acute small bowel obstruction who was successfully treated by laparoscopy. This is the first case report of this kind of hernia managed with laparoscopic technique. Although the laparoscopic approach is not considered standard treatment for strangulated hernias, in selected cases, it allows the identification and repair of the defect.

Splenomesenteric bypass as revascularisation technique after iatrogenic injury of the superior mesenteric artery during radical nephrectomy: A case report.

INTRODUCTION: Iatrogenic vascular injuries during radical nephrectomy are rarely reported. In the case of an injury of the superior mesenteric artery, the consequences for the patient are potentially catastrophic. It occurs more frequently in patients with large renal tumors, due to the presence of bulky perihilar adenopathies, or in cases of pyelonephritis. In most cases, the inadvertent injury of the artery occurs due to the difficulty in distinguishing it from the left renal artery. CASE: 69-year-old male, with a malignant neoplasm of the left kidney with the involvement of the tail of the pancreas, tumor thrombus in the left renal vein and multiple left para-aortic adenopathies, whose histological examination revealed to be a renal sarcomatoid carcinoma pT4N1M0G3. A radical nephrectomy was performed with caudal splenopancreatectomy and left paraaortic lymphadenectomy, with an iatrogenic injury of the superior mesenteric artery at its origin. A terminoterminal anastomosis was performed from the proximal stump of the splenic artery. The postoperative period went uneventfully. Control imaging in the follow-up showed permeability of the celiac trunk and the superior mesenteric artery. CONCLUSIONS: Intra-operative superior mesenteric artery injury should be promptly identified and repaired to prevent gut ischemia and all its dire consequences. There are different repair options, and the technique should be chosen according to the degree of injury. The splenomesenteric bypass has numerous advantages.

Boerhaave's syndrome: A case report of damage control approach.

INTRODUCTION: Boerhaave's syndrome is a life-threatening oesophageal perforation that carries a high mortality rate (20-50%). Diagnosis is difficult by its rarity and the absence of typical symptoms. Treatment of this condition usually requires surgical intervention. PRESENTATION OF CASE: We report the case of a 77-year-old man that resorted to the emergency room with dyspnoea and thoracic pain after vomiting. CT scan revealed pneumomediastinum, left collapse lung and loculated pleural effusion. A left intercostal chest tube was inserted with food drainage. Hence, Boerhaave's syndrome was suspected. Thoracotomy with mediastinum debridement, pleural drainage and oesophageal T-tube drainage was performed. Patient was admitted on the Intensive Care Unit with septic shock, with need for ventilatory support and vasopressor therapy. Two days later, a second look thoracotomy was done with definitive oesophageal repair and pleural patch. The post-operative course was complicated by pneumonia and stroke. Patient was discharged home on the 38th day and remains well at 3 month of follow-up. DISCUSSION: Delayed diagnosis and treatment are the principal causes of high mortality in Boerhaave's syndrome. The classic Mackler's triad (vomiting, lower thoracic pain and subcutaneous emphysema) is present in less then 50% of cases. A thoracic drainage may be useful to confirm diagnosis promptly. There is no standard treatment option. In this case report, the authors used a damage control approach to control sepsis, allowing for a delayed definitive oesophageal repair. CONCLUSION: Prompt diagnosis with thoracic drainage and a damage control treatment plan might lead to good prognosis for patients with this rare and potentially fatal condition.

Laparostomy during pregnancy: A case report.

INTRODUCTION: Acute appendicitis is the main indication for surgery during pregnancy. Physiologic changes during pregnancy and fear of using ionising radiation exams are some of the reasons to delayed diagnosis and consequently to higher morbidity and mortality rates for mother and fetus. PRESENTATION OF CASE: We present the case of a 38-year-old woman that resorted to the emergency room on the 13th week of pregnancy with abdominal discomfort, nausea and vomiting that lasted for 7 days. She had been in the Obstetric Emergency Department 6 days prior with the same complaints. She had no fever and she was discharged home following normal obstetric ultrasound. On this second visit, after surgical consultation, septic shock with abdominal source was recognised and patient was taken for emergency exploratory laparotomy. Intraoperatively we found generalised purulent peritonitis secondary to perforated acute appendicitis. Appendectomy, thorough abdominal washing and laparostomy were performed. Patient was admitted on the Intensive Care Unit with septic shock, need for vasopressor therapy and dialysis. Four days after the first intervention the abdominal cavity was closed. She was discharged home on the 14th post-operative day and maintained obstetric follow-up for the remaining uncomplicated pregnancy. DISCUSSION: In the presented clinical case, diagnostic delay evolves to abdominal sepsis that demanded a damage control approach. Laparostomy constitutes a damage control gesture, limiting abdominal contamination, preventing abdominal compartment syndrome and allowing subsequent surgical revisions. CONCLUSION: Acute abdominal approach using laparostomy allowed for a good outcome, maintaining ongoing pregnancy and with incisional hernia as the only observed morbidity.

Laparoscopic choledochoscopy with Ambu® aScope 3™ in the treatment of coledocholithiasis: a series of cases.

The progresses made in minimally invasive surgery, make it not only possible to perform isolated cholecystectomy, but also to provide a totally laparoscopic treatment of common bile duct lithiasis. In this approach, the use of choledochoscopy is indispensable for diagnostic and therapeutic success. This study describes the use of a disposable endoscope for choledochoscopy in the laparoscopic treatment of choledocholithiasis. We have analyzed the use of the Ambu® aScope 3™ disposable endoscope, normally used for bronchoscopy, in nine consecutive patients needing choledochoscopy in our Hospital. The patient average age was 78 years. Total clearance of the bile duct was obtained in seven patients. Bile duct vacuity was not obtained in two patients, but this is not attributed to equipment failure. There was one case of hemoperitoneum not related to equipment usage. This device is easy to use and looks promising for choledochoscopy in laparoscopic treatment of coledocholithiasis.

Wunderlich's Syndrome, or Spontaneous Retroperitoneal Hemorrhage, in a Patient with Tuberous Sclerosis and Bilateral Renal Angiomyolipoma.

BACKGROUND Wunderlich's syndrome, or spontaneous non-traumatic retroperitoneal hemorrhage, can be a life-threatening event. Renal angiomyolipoma is a rare benign tumor that can occur sporadically, or in association with tuberous sclerosis. A case of spontaneous retroperitoneal hemorrhage in a patient with tuberous sclerosis and bilateral renal angiomyolipoma is presented. CASE REPORT A 33-year-old female Caucasian patient, with a known medical history of tuberous sclerosis, was admitted to hospital as an emergency, with right-sided abdominal pain. Abdominal computed tomography (CT) imaging showed bilateral renal tumors, consistent with bilateral renal angiomyolipoma. The larger tumor, involving the enlarged right kidney (24.0 cm in length), had a diameter of 21.0 cm and was associated with hemoperitoneum and retroperitoneal hemorrhage, and contrast 'blush' on CT confirmed arterial bleeding. An initial urgent exploratory laparotomy with renal packing was initially performed, but right nephrectomy was required for hemorrhage control. The patient was discharged from hospital on the 23rd postoperative day, without further complications. CONCLUSIONS This report describes a case of Wunderlich's Syndrome, or spontaneous retroperitoneal hemorrhage, in a patient with tuberous sclerosis and bilateral renal angiomyolipoma, presenting as an emergency. An early diagnosis and timely treatment are important in cases of retroperitoneal hemorrhage to prevent life-threatening complications.