Spigelian-cryptorchidism syndrome: Lesson based on a case report.

The Spigelian hernia is a abdominal wall hernia that originates from a discontinuity of the Spigelian fascia located lateral to the rectus abdominis muscle. It can be acquired in adults or congenital in newborns. In very rare cases in male it can be associated with cryptorchidism, in which case it is known as "Spigellian-Cryptorchidism Syndrome". It can be clinically highlighted with abdominal swelling wall along the semilunar line and intestinal obstruction. The diagnosis, as in all pediatric emergencies, must be timely and the method of choice is ultrasound which allows a rapid localization of the hernia breach and herniated structures. The treatment of choice is surgical with herniopexy and repositioning of the testicle into the scrotal sac, or orchipessy in cases of testicular necrosis. We describe ultrasound characteristics of Spigellian-cryptorchidism syndrome presenting with acute intestinal obstruction in a newborn.

Thoracic outlet syndrome: a rare case with bilateral cervical ribs and bilateral anterior scalene hypertrophy.

Thoracic outlet syndrome (TOS) is a rare neurovascular disorder generally caused by the presence of a cervical rib or hypertrophic scalene anterior muscle that can compress the brachial plexus and/or subclavian vessels. In the vascular form, the symptoms are caused by the compression of the artery and/or the subclavian vein. In the first case, the compression is caused by the cervical rib and leads to hypo-perfusion with cooling and cyanosis of the upper limb, while in the second case, the compression is caused by the anterior scalene muscle and leads to congestion, cyanosis, swelling and pain in the higher limb. In this paper, we describe a case with the simultaneous presence of a bilateral cervical rib and bilateral hypertrophy of the anterior scalene muscle. TOS diagnosis is based on neurological, clinical and instrumental tests, such as chest radiography and color Doppler ultrasonography. The treatment of these patients can be surgical or conservative.

Eagle's syndrome: a case report and CT pictorial review.

Eagle's syndrome (ES) refers to symptomatic elongation of the ossified styloid process. A styloid process greater than 2.5 cm in length should be considered abnormal; however, an elongated styloid process is not sufficient for a diagnosis of ES; only an abnormal styloid process in association with symptoms can confirm the syndrome. In this case report, we discuss a 54-year-old man who has come to our attention with various symptoms: dysphagia to both solids and liquids, difficulty swallowing, neck pain, and a foreign body sensation during bilateral neck rotation and mouth opening. The diagnosis is performed radiologically because conventional radiographs have many potential disadvantages, whereas, computed tomography (CT) scans and reconstructions allow the length and angulation of the styloid process to be measured and the relationship between the elongated styloid processes and adjacent anatomical structures to be evaluated. Moreover, CT allows for differential diagnosis and provides detailed information needed for surgical planning.

Thyroid adenolipoma: a case report.

Generally, small quantities of adipose tissue is present in the thyroid gland. The adenolipoma of the thyroid gland is considered a rare finding. It consists in a benign, encapsulated neoplasm composed of mature adipose tissue and glandular elements. We report a case of a 71 year-old female patient presenting with swelling of the anterior neck and history of airway obstruction. Ultrasound (US) examination showed a bulky multinodular goiter which caused dislocation and compression of the trachea. The scans performed at the level of the isthmic region showed the presence of a hyperechoic oval formation with a homogeneous echostructure and regular contours; these characteristics suggested the lipomatous nature of the nodule. The patient was subsequently subjected to a Computer Tomography (CT) of the neck for a pre-operative balance of the goitre and to exclude extra-thyroid pathologies. The CT scan confirmed the sonographic findings, and the probable adipose nature of the isthmic formation. After the patient has been subjected to total thyroidectomy and histological examination confirmed the diagnosis of adenolipoma.

Wilkie's syndrome.

Superior mesenteric artery syndrome, also known as Wilkie's syndrome, is a rare vascular disease caused by the anomalous course of the superior mesenteric artery arising from the abdominal aorta with a smaller angle than the norm (<22°). The reduced angle compresses the structures situated between the aorta and the superior mesenteric artery, such as the duodenum and left renal vein; this can determine painful crises, intestinal subocclusions, and left varicocele. This syndrome can be congenital or acquired. The acquired type is more common and is generally caused by reduced perivascular fat surrounding the abdominal aorta and the superior mesenteric artery; this form is common among anorexic patients that have had a rapid weight loss. We present the case of a female patient who suffered from repeated postprandial vomiting and who lost 12 kg in 4 months. B-mode ultrasound imaging revealed evidence of a reduced angle between the aorta and the superior mesenteric artery, as found in Wilkie's syndrome. After diagnosis, the patient followed a high-calorie diet, and 2 months later an ultrasound scan proved the restoration of the aorto-mesenteric angle as a consequence of increased perivascular fat with regression of symptoms.

Lung cancer invading a single left pulmonary vein requiring extended pneumonectomy.

Unilateral single left pulmonary vein is a congenital anomaly of the pulmonary venous system. Surgical treatment is not commonly required for this anatomical variant except in rare circumstances. No previous cases of lung cancer involving the intrapericardial portion of a single left pulmonary vein have been published in the peer-reviewed literature. We describe the case of a 69-year-old man with lung cancer invading single left pulmonary vein, which required intrapericardial pneumonectomy and partial resection of the left atrium.

Congenital portosystemic shunt: our experience.

Introduction. Congenital portosystemic venous malformations are rare abnormalities in which the portal blood drains into a systemic vein and which are characterized by extreme clinical variability. Case Presentations. The authors present two case reports of a congenital extrahepatic portosystemic shunt (Type II). In the first patient, apparently nonspecific symptoms, such as headache and fatigue, proved to be secondary to hypoglycemic episodes related to the presence of a portosystemic shunt, later confirmed on imaging. During portal vein angiography, endovascular embolization of the portocaval fistula achieved occlusion of the anomalous venous tract. In the second patient, affected by Down's syndrome, the diagnosis of a portosystemic malformation was made by routine ultrasonography, performed to rule out concurrent congenital anomalies. Because of the absence of symptoms, we chose to observe this patient. Conclusions. These two case reports demonstrate the clinical heterogeneity of this malformation and the need for a multidisciplinary approach. As part of a proper workup, clinical evaluation must always be followed by radiographic diagnosis.

Desmoid-type fibromatosis of the mesentery: report of a sporadic case with emphasis on differential diagnostic problems.

Desmoid-type fibromatosis is a rare mesenchymal neoplasm with local aggressiveness. The incidence of desmoid-type fibromatosis is 2-5/million/year with intra-abdominal fibromatosis, such as that which is reported in this clinical case, occurring only in 12-18% of cases. After having analyzed the pathogenetic hypotheses of desmoid-type fibromatosis, the authors point out that the diagnosis of this disease, especially in the intra-abdominal form, is often late, specifically when highly demolitive interventions are needed or when the limits of radical surgery have been exceeded. In the clinical case reported, the tumor was infiltrating both ileus and sigma. The authors consider the differential diagnosis of desmoid-type fibromatosis, especially with GISTs, with regard to both the radiological preoperative diagnostic and histological studies on the surgical specimen. Radical surgical excision is not always, for this disease, a sign of healing; in fact, even when the resection margins are negative, the incidence of recurrence is between 13 and 68%. The average time of recurrence is between 15 and 24 months; in this case report, the patient, who has not been subjected to complementary therapies, is tumor-free for over 30 months since surgery; his prognosis may be satisfactory if we consider the negativity of resection margins, which in any case remains the most important prognostic factor.

Kabuki syndrome and cancer in two patients.

Both hepatoblastoma and neuroblastoma are occasionally associated with congenital syndromes such as Beckwith-Wiedemann syndrome and trisomy 18. There have been no reports of hepatoblastoma in patients with Kabuki syndrome, whereas one patient with neuroblastoma and this syndrome has been reported. In this paper we present two patients with Kabuki syndrome and a neoplasm: a child of 6 years with hepatoblastoma and an infant, of 6 months affected by neuroblastoma.

Late complication from a retrievable inferior vena cava filter with associated caval, aortic, and duodenal perforation: a case report.

Inferior vena cava filters are an excellent therapeutic method for those patients in whom anticoagulant therapy is contraindicated or ineffective. However, filter placement is associated with a high rate of serious complications (>30%), with death occurring in 3.7% of patients. The most common complication is an asymptomatic inferior vena cava penetration and perforation. In some rare circumstances, however, therapeutic intervention may be required because of perforation of adjacent organs. We report a clinical case of a patient with simultaneous caval, duodenal, and aortic perforation resulting from penetration of inferior vena cava filter hooks. A brief review of the literature discusses presenting symptoms and treatment of such rare complications.