Giant uterus in a patient with Klippel-Trenaunay syndrome. Report of a case.

INTRODUCTION: Klippel-Trenaunay syndrome is an uncommon congenital disease also called angio-osteodystrophy syndrome for its typical disorders characterized by abnormal growth of the soft tissues and bones and vascular malformations. CASE REPORT: In this report, we present a rare case of a 46-year-old nulliparous woman with Klippel-Trenaunay syndrome. She suffered from an abnormal uterine bleeding accompanied by severe anemia with need for multiple blood transfusions. At the time of admission, physical examination revealed port-wine stains and varicose veins on her lower limbs and hypertrophy of left lower extremity. We carried out an open bilateral hysteroannexectomy. Histopathology examination revealed a uterus weighing 6300 g with diffuse abnormal vasculature, leiomyomatosis and diffuse venous malformation. DISCUSSION: Klippel-Trenaunay syndrome is considered a rare disease as it has an incidence of 2-5 on 100,000 with no differences between the both sexes. The clinical manifestations are related to the organs involved in the pathological angiogenesis therefore patients may also present cerebral, retinal, gastrointestinal and genitourinary anomalies with consequent occult or significant bleeding. Uterine involvement is very rare. The main clinical manifestations are represented by irregular episodes of hematuria and/or menometrorrhagia associated with important anatomical anomalies of the female reproductive system. CONCLUSION: Uterine involvement in patients with Klippel-Trenaunay syndrome, althought rare and with extremely variable clinical manifestations, is actually a relevant event that not only significantly affects the reproductive capacity of the woman but which potentially puts patients' lives at risk because of an unpredictable bleeding.

Uterine Involvement in Klippel-Trenaunay Syndrome: A Rare But Relevant Event. Review of the Literature.

Introduction: Klippel-Trenaunay syndrome (KTS) is a rare vascular congenital disorder characterized by the classical triad of port-wine stains, abnormal growth of soft tissues and bones, and vascular malformations. The involvement of the genitourinary tract and of the uterus in particular is extremely infrequent but relevant for possible consequences. Methods: We performed an extensive review of the literature using the Pubmed, Scopus and ISI web of knowledge database to identify all cases of KTS with uterine involvement. The search was done using the MeSH term "Klippel-Trenaunay syndrome" AND "uterine" OR "uterus." We considered publications only in the English language with no limits of time. We selected a total of 11 records of KTS with uterine involvement, including those affecting pregnant women. Results: Klippel-Trenaunay syndrome was described for the first time in the year 1900 in two patients with hemangiomatous lesions of the skin associated with varicose veins and asymmetric soft tissue and bone hypertrophy. Uterine involvement is a rare condition and can cause severe menorrhagia. Diagnosis is based on physical signs and symptoms. CT scans and MRI are first-choice test procedures to evaluate both the extension of the lesion and the infiltration of deeper tissues before treatment. The management of Klippel-Trenaunay syndrome should be personalized using careful diagnosis, prevention and treatment of complications. Conclusion: Klippel-Trenaunay syndrome is a rare vascular malformation with a wide variability of manifestations. There are no univocal and clear guidelines that suggest the most adequate monitoring of the possible complications of the disease. Treatment is generally conservative, but in case of recurrent bleeding, surgery may be needed.

Successful laparoscopic management of congenital diaphragmatic relaxation: A case report.

INTRODUCTION: Diaphragmatic relaxation is an infrequent condition characterized by a permanent elevation of all or part of an hemidiaphragm which maintains its insertions on the ribs and which does not have continuous solutions but a reduced thickness. PRESENTATION OF CASE: We studied a 65 years old male patient with mild dyspnea and chest pain occurred during the last 12 months after moderate efforts. He underwent a contrast enhanced CT thorax scan that showed a left diaphragmatic relaxation with the transposition in the cranial sense of the hypochondriac abdominal organs associate with an ipsilateral subtotal atelectasia. A laparoscopic plication of the diaphragm was performed to repair the congenital defect. DISCUSSION: The relaxatio diaphragmatica is probably caused by a congenital defect, but there are also idiopathic causes or cases of acquired relaxation due to phrenic nerve damage because of neoformations, traumas, thoracic and cardiac surgery. In cases of asymptomatic relaxatio nothing is necessary, but in symptomatic cases it is possible the plication of the diaphragm with a remission of symptoms. CONCLUSION: The plication can be performed through thoracotomy or laparotomy and recently also in thoracoscopy or laparoscopy. In our experience the laparoscopic repair of the relaxatio was accomplished successfully with a left pneumothorax compatible with the intervention, but the operative strategy should be always individualized with attention on diagnosis, patient characteristics, availability of resources and experience of surgical team.

Recurrent intussusception of small bowel in a young patient due to metastases from cardiac undifferentiated pleomorphic sarcoma: A first ever case report.

INTRODUCTION: Undifferentiated metastatic pleomorphic sarcoma (Malignant Fibrous Histiocytoma) is a rare entity in the small intestine, especially when the primary tumor is of cardiac origin. CASE REPORT: We report a case of metastatic intestinal undifferentiated pleomorphic sarcoma in a young patient with a history of primary cardiac tumor in the left atrium and recurrent small bowel intussusception. He was admitted for abdominal pain and constipation. A segmental resection of the small intestine was performed with side-to-side entero-enteroic anastomosis. DISCUSSION: Intussusception of the small bowel is rare in adults and it represents about 1-3% of intestinal obstructions. It mainly affects the fifth decade with a male/female ratio of 1:5 More than 60% of patients with intussusception have a tumor with 50% being malignant. This type of intussusception can be diagnosed on the CT abdominalscan. Radiological features include a typical "target" sign with overdistention of the proximal intestine and air-fluid levels, but the diagnosis of certainty is made by exploratory laparotomy. CONCLUSION: The metastatic tumors that cause intussusception represent a rare clinical condition in adult patients, but much more common than primary ones. Metastasis to the small intestine are part of differential diagnosis in patient with a history of tumor who present with intussusception.

Small bowel obstruction after caesarean section: Laparoscopic management. Two case reports.

INTRODUCTION: Caesarean section is the most common abdominal surgery performed on women worldwide. Adhesions represent a severe complication of cesarean section and can cause different degrees of bowel obstruction. CASE REPORTS: We report two unusual cases of small bowel obstruction treated with laparoscopic approach after caesarean section performed for gynecological pathologies. In the first one small bowel obstruction was due to volvulus caused by a severe pelvic adhesion syndrome; in the second one, occlusive picture was related to presence of multiple adhesion phenomena between the sigmoid colon and the right ovary as result of abdominal hysterectomy. DISCUSSION: The incidence of small bowel obstruction after caesarean section is very low and postoperative adhesions represent the main cause. Diagnosis was established by clinical signs, radiological and intraoperative findings. Laparoscopic approach can be the treatment of choice only in selected patients. In presence of dense adhesions, inability to visualize the site of obstruction, iatrogenic intestinal perforation, bowel necrosis and technical difficulties, conversion to open surgery is mandatory. CONCLUSION: In selected patients with small bowel obstruction laparoscopy is a safe and feasible procedure if conservative measures fail.

Small bowel volvulus due to a large intestinal lipoma: A rare case report.

INTRODUCTION: A lipoma of the small bowel mesentery is a uncommon clinical entity. It rarely causes obstruction and volvulus of the small bowel. CASE REPORT: A 63 year old man was admitted to the emergency department with acute abdominal pain. Contrast-enhanced CT abdominal scan revealed small bowel obstruction due to a large fat density lesion suspected to be a lipoma. We performed a laparotomy in urgent setting that confirmed a small bowel volvulus secondary to a large antimesenteric lipoma. En-bloc resection with antiperistaltic side-to-side ileal anastomosis was done. DISCUSSION: Mesenteric lipoma is rare. They are usually asymptomatic but when have large sizes can cause several symptoms related to small bowel obstruction or volvulus. The diagnosis is difficult and is rarely made prior to exploratory laparoscopy or laparotomy. CT scan is the gold-standard imaging technique. It can shows the typical characteristics of tumor and may demonstrate the typical "vortex" pattern of a volvulus. In patients with acute clinical presentation en-bloc resection of the lipoma with the affected small bowel loops is often necessary. This treatment may also be reserved in asymptomatic patients with large mesenteric lipomas to avoid future complications. CONCLUSION: Volvulus of the small bowel caused by an antimesenteric lipoma is a very rare entity. It is diagnosed by CT scan and surgery represents the standard treatment.

Small bowel obstruction after laparoscopic gastrectomy: An atypical clinical presentation. Report of a case.

INTRODUCTION: Postoperative adhesions represent the most common cause of acute small bowel obstruction (80%) and are usually a consequence of abdomino-pelvic surgery performed with open technique. PRESENTATION OF CASE: A 45-year-old black man arrived at the emergency room with abdominal pain and distension three months after laparoscopic distal gastrectomy with Roux-en-Y anastomosis performed for benign pyloric stenosis. CT abdominal scan revealed some air-fluid levels in the center of the abdomen with distension of proximal jejunal loops caused by intestinal adhesions. Laparoscopic adhesiolysis was performed to restore the intestinal transit. DISCUSSION: The formation of adhesions is more frequent after abdomino-pelvic surgery. CT abdominal scan is very useful tool to identify the level and the aetiology of obstruction and it may predict the need for surgery, the location of different adhesive bands in order to identify wich patients are likely candidates for laparoscopic treatment. CONCLUSION: In selected cases, laparoscopic approach for small bowel obstruction is a good surgical option. In patients with adbominal dense adhesions or clinical signs of intestinal ischemia, conversion to laparotomy should be considered an alternative.