Hepatic lymphangiomatosis mimicking polycystic liver disease

Hepatic lymphangiomatosis is a rare disorder characterized by cystic dilatation of the lymphatic vessels in the hepatic parenchyma. It can occur in the liver alone, in the liver and spleen, or in multiple organs. Clinically, diagnosis can be difficult because of the rarity and protean manifestations of this disorder. We describe a 53-year-old woman with hepatic lymphangiomatosis in whom polycystic liver disease had been previously diagnosed. In addition, we review 12 cases of hepatic, splenic, and hepatosplenic lymphangiomatosis with or without systemic lymphangiomatosis and discuss the differential diagnosis.     

Lymphangiomatosis with splenic involvement

Severe splenomegaly and anemia developed in a 5-year-old girl with diffuse lymphangiomatosis of the upper part of the body. Radioisotope scanning and celiac angiography demonstrated lymphangiomatosis of the spleen, a rare but diagnosable condition. Intractable infection in areas of ulcerated skin led to her death from overwhelming sepsis.     

Gorham's disease: a literature review and case reports

Gorham's disease (disappearing bone disease, massive osteolysis, idiopathic osteolysis, essential osteolysis, progressive atrophy of bone, spontaneous absorption of bone, phantom bone, hemangiomatosis/lymphangiomatosis of bone, progressive osteolysis) is an extremely rare occurrence. There are fewer than 150 reported cases in the literature. This disorder can be characterized by spontaneous or posttraumatic progressive resorption of bone. The etiology is still very speculative, the prognosis unpredictable, and any effective therapy still unknown. This paper presents a review of the literature and two case reports of suspected Gorham's disease of the bones of the foot.     

Bilateral hydatid cyst of pulmonary arteries: MR and CT findings

The location of hydatid cysts inside pulmonary arteries has been reported only on rare occasions in the literature and is caused, in the majority of cases, by embolization due to the rupture of hydatid cysts located in the heart; more rarely, hematogenous dissemination from a hepatic focus is the cause. We report a case of a 44-year-old patient with hydatid cysts located in both the right and left pulmonary arteries, whose first clinical reference was hemoptysis. The patient had undergone surgery because of a hepatic hydatid cyst 5 months previously. The importance of this case lies in the infrequent bilateral location in both pulmonary arteries and in the absence of intracardiac hydatid cysts.     

Precision of plasma clearance of iohexol for estimation of GFR in patients with renal disease

Dural spinal cysts are exceedingly rare lesions. The authors are aware of only seven cases reported in the literature. This report comprises three patients with dural cysts of the cervical spinal canal who presented with atypical neurological signs and symptoms, including a long history of dysesthesias and atrophic pareses in the proximal upper extremities. Surgical intervention disclosed two ventrally and one dorsally located cystic lesions of the spinal dura mater. The cystic wall was fenestrated and kept open by a silicone catheter in each case. Histological examination of the excised lesions revealed fibrous connective tissue consistent with dural tissue. No epithelial lining was detected. The lesions were classified as dural cysts. The patients' postoperative courses were uneventful. Although dural cysts are rare lesions, they must be considered in the differential diagnosis of spinal cystic lesions. Because they are nonaggressive and can be safely drained by microsurgical techniques, patients have a favorable prognosis. Early surgical intervention is mandatory to prevent severe neurological deficits.     

Parasellar epidural hydatid cysts

We report two rare cases of parasellar epidural hydatid cysts. Among the 64 patients with intracranial hydatid cysts we have treated in the past 20 years, only these two cases were located epidurally. Because the location and the computed tomographic characteristics were so different from the other cysts, the preoperative diagnosis was difficult to make. We were unable to remove the cysts without rupturing them because they were tightly surrounded. Both patients underwent surgery twice because of a recurrence. The treatment of this kind of case requires further discussion.     

Dentigerous cyst with inflammatory etiology from a deciduous predecessor--report of a case

Dentigerous cysts arising from overlying infected predecessor teeth are very rare. This report describes one such case where extraction of the infected deciduous tooth with marsupialization led to the eruption of the premolar. Periodic radiographic evaluation is required to note and prevent recurrence and other serious complications arising from these cysts.     

Comparison of five methods for the determination of arginine hydrolysis by viridans streptococci

The authors describe a rare case of cystic nephroma treated by partial surgical excision. Because there is concurrence in the association of multilocular cysts with Wilms tumors and others tumors of the kidney, it is underlined the importance of a differential diagnosis to avoid nephrectomy for treatment of this benign neoplasm.     

Bilateral lutein cyst in normal single-child pregnancy

A rare case of bilateral multiple lutein cysts in pregnancy is reported. They do not require a radical operation because of spontaneous regression after delivery.     

Immunogenetics of episcleritis in leprosy

BACKGROUND: Neurenteric cysts are rare endothelium-lined structures. Two patients with symptomatic neurenteric cysts at the craniovertebral junction are presented. CASE PRESENTATION: Intermittent progression of neurologic symptoms delayed diagnosis for both patients. In one case, marked enlargement of the cyst was detected on serial imaging studies, and pathological examination of the excised lesion indicated rupture of the cyst. These cysts were totally resected by transoral or suboccipital approaches, as they were not firmly adherent to surrounding neural structures. The diagnosis of neurenteric cyst was confirmed by immunohistochemical analysis of the cyst wall. CONCLUSION: In view of the clinical course of these patients, we recommend early surgical resection of neurenteric cysts located at the craniovertebral junction.     

Urinary tract infections in children due to Pseudomonas aeruginosa in Enugu, Nigeria

Ileal duplication cysts within a giant omphalocele are very rare. Only a few cases have been reported in the English literature (4). We report one case of giant omphalocele, which included a huge ileal duplication cyst, detected by prenatal US, and diagnosed at surgery after birth. This case illustrates the diagnostic and therapeutic problems occurring during pregnancy and the neonatal period.     

A case report of glomerulocystic kidney disease with hypothyroidism in a new born infant

We report a rare case of glomerulocystic kidney disease (GCKD) with congenital hypothyroidism. A gigantic abdominal mass was noted at birth. There was no family history of renal cystic disease. Ultrasonography revealed diffuse granular cysts in the markedly enlarged kidneys. Blood examination showed moderate renal failure and hypothyroidism. Bilateral nephrectomy was conducted at 47 days of age to relieve respiratory failure and severe abdominal distention caused by the growing cystic kidneys. Histological findings of the kidney showed numerous glomerular cysts without renal dysplasia. There were no other malformations. These findings were compatible with GCKD.     

The schwannoma: an uncommon type of cystic lesion of the pancreas

Schwannomas of the pancreas are rare and their number is lower than is usually reported in the literature since some were probably neurofibromas. We report a further case characterized by cystic feature, clinically mimicking a pancreatic pseudocyst. Cystic lesions of the pancreas also comprise the schwannomas, both benign or malignant, as has been previously reported and is stressed by the present case. Cyst formation characterizes many diseases of the pancreas, both neoplastic and not. Difficulties in the differential diagnosis of cysts are fortunately reduced by the preponderance of pseudocysts (i.e. non-neoplastic cysts) and, among neoplastic ones, of cystadenomas and cystadenocarcinomas. As a consequence, uncommon cystic neoplasms are neglected and very often misdiagnosed. We reported a case of solitary schwannoma mimicking a pancreatic pseudocyst, interesting both because of its rarity and because of its cystic feature which was previously reported in the literature but not emphasized.     

Disseminated intra-abdominal cystic lymphangiomatosis with severe intestinal bleeding. A case report

We describe cystic lymphangiomatosis with intestinal bleeding developing multiple lymphangiomas in the small intestine, mesentery, mesocolon, omentum, retroperitoneum, and spleen. Small intestinal fluorography showed multiple polypoid lesions, mainly in the jejunum. Ultrasonography, computed tomography, and magnetic resonance imaging showed diffuse cystic tumors in the mesentery and spleen. Cystic lymphangiomatosis was proved by histologic findings of the biopsied specimen at laparotomy.     

Calcified solitary renal cyst in childhood

There is a 20% incidence of malignancy in renal masses with rim calcification in adults. A case of rim calcification in a solitary renal cyst in a child is presented and the diagnostic approach and surgical management are described. Solitary renal cysts are extremely rare in children, with no reported cases of rim calcification.     

Diverticula of the female urethra. Comparison of imaging techniques

Splenic epidermoid cysts are rare lesions traditionally treated by splenectomy. Concerns about overwhelming postsplenectomy sepsis have led to the development of splenic preservation procedures in the treatment of cystic diseases of the spleen. We present the first case report of successful laparoscopic complete excision of a splenic epidermoid cyst.     

Orbital lobe lacrimal ductal cyst

Lacrimal ductal cysts are rare, especially in the orbit. A case of lacrimal ductal cyst in the orbital lobe, revealed by computed tomography and magnetic resonance imaging, is described in a 16-year-old man who was successfully treated by complete surgical excision. The radiologic and clinical features of this type of lacrimal ductal cyst are discussed.     

Papillary cystadenoma of the minor salivary glands

Papillary cystadenoma of the minor salivary glands is a rare benign tumor that clinically resembles a mucous cyst. We demonstrate its histologic features and differential diagnosis by a case report. Benign and malignant neoplasms of the minor salivary glands are not well acknowledged in the dermatologic literature, but should be considered in the differential diagnosis for mucous cysts.     

Pericardial cyst: an incidental finding

Pericardial cysts are rare mediastinal cysts occurring with an incidence of 1 in 100, 000. Characteristically, they occur along the right border of the heart. Their size varies from 1.0 cm to 15 cm, and they are often asymptomatic. Patients with symptoms usually have atypical chest pain. In the case reported here, a 37-year-old man complained of nonproductive cough. Chest x-ray film revealed a pericardial cyst that appeared as a large echolucent unilocular mass along the left border of the heart. Diagnosis was confirmed with the use of both computed tomography and transthoracic echocardiography.     

Prepubertal ovarian cyst formation: 5 years' experience

OBJECTIVE: To document the frequency of ovarian cyst formation in the prepubertal female and to report on the clinical implications of these cysts. METHODS: We evaluated cases from a retrospective chart review of prepubertal females with ovarian cysts. RESULTS: Over a 5-year period, 1818 ultrasound studies were completed in prepubertal females, from which 99 patients were identified as having ovarian cysts. The majority (82 of 99, 83%) were small, unilocular cysts averaging 2-3 mm in diameter. The incidence of these small cysts ranged at 2-5% in females between birth and age 8. Large ovarian cysts (ie, greater than 2 cm in diameter) are rare in young girls over age 2, with most occurring within the first year of life. Of the 17 large ovarian cysts, two presented with torsion and both were complex on ultrasound examination. Five (29%) of the large ovarian cysts were treated conservatively and demonstrated regression on follow-up; these were unilocular cysts. Although functioning cysts resulting in precocious pseudopuberty are expected to be rare, we identified five cases (5%) in our series. CONCLUSIONS: Small, unilocular ovarian cysts less than 1 cm in diameter are found in prepubertal females with a frequency of 2-5% and are clinically insignificant. Ovarian cysts greater than 2 cm are rare. Unilocular ovarian cysts less than 5 cm may be followed conservatively with ultrasound surveillance until regression, without a major risk of torsion. Ovarian cysts associated with precocious pseudopuberty are generally larger than 2 cm and may be recurrent.