Value of MR cholangiopancreatography in evaluating choledochal cysts

OBJECTIVE: The aim of this retrospective study was to clarify whether MR cholangiopancreatography (MRCP) is a suitable replacement for ERCP in evaluation of the choledochal cyst. MATERIALS AND METHODS: Sixteen patients (six adult and 10 pediatric) with choledochal cysts underwent MRCP using a half-Fourier acquisition single-shot turbo spin-echo sequence. Extent of the cyst, defects within the biliary tree, and presence or absence of the anomalous junction of the pancreaticobiliary duct were evaluated. Findings were compared with those of ERCP. RESULTS: MRCP better defined the proximal biliary tree than did ERCP in two patients. Defects within the biliary tree were diagnosed correctly on MRCP in eight patients; however, two defects within the distal common bile duct were missed in pediatric patients. The presence of the anomalous junction of the pancreaticobiliary duct was revealed accurately by MRCP in all adult patients but was revealed accurately in only four of the 10 pediatric patients. CONCLUSION: MRCP appears to offer diagnostic information that is equivalent to that of ERCP for assessment of choledochal cysts in adults. In pediatric patients, MRCP should not replace ERCP; however, MRCP can play an important role as a noninvasive examination and should be considered a first-choice imaging technique for evaluation of choledochal cysts.     

Presentation and surgical management of bronchogenic and esophageal duplication cysts in adults

OBJECTIVE: Bronchogenic and esophageal duplication cysts are congenital anomalies of the tracheobronchial tree and foregut that are often asymptomatic at initial presentation in adults. Surgery is always recommended, even for patients with asymptomatic disease, because of the possible development of symptoms and complications during the natural course of the disease and because definitive diagnosis can be established only on surgical specimen. METHODS: Twenty-seven patients with bronchogenic and esophageal duplication cysts were treated in our institution over the last 2 decades. Ten patients (37%) were asymptomatic at initial presentation. Chest pain and dysphagia were the most common complaints in symptomatic patients affected by bronchogenic and duplication cysts, respectively. RESULTS: A complete excision of the cyst was performed in 26 cases, whereas one patient with intrapulmonary cyst underwent a right upper pulmonary lobectomy. A posterolateral thoracotomy was performed in 23 patients, and a video-assisted thoracoscopy using a three-port technique was performed in the last 4 patients. No postoperative morbidity was recorded. All patients, except one, were asymptomatic at a median follow-up time of 4 years. CONCLUSIONS: Surgery is the treatment of choice for bronchogenic and esophageal duplication cysts. Video-assisted thoracoscopy should represent the first-line approach in these patients.     

Laparoscopy for ovarian pathology in infancy and childhood

INTRODUCTION: Nonparasitic splenic cysts are a rare finding: they can be distinguished into true cysts (epidermoid or epithelial) cysts or false (pseudocysts), depending on the presence/absence of an inner epithelial lining. They are usually found during the second and third decade of life, although they can also appear during childhood. They grow bigger in children, becoming clinically apparent due to the symptoms of compression of adjacent structures. The diagnosis is radiological, made with ultrasonography (US) and computed tomography (CT). We report the US and CT patterns of splenic cysts to differentiate true from false cysts. MATERIAL AND METHODS: Our series consists of 5 patients, aged 5-17 years, all examined with US and CT; one of them was also submitted to plain abdominal radiography. Conventional CT was used in 2 cases and helical CT in the extant 3. RESULTS: All examinations yielded useful clinical informations. The only radiograph showed the rightward displacement of the gastric gas bubble US always identified the cyst and its pertinence to splenic parenchyma, although failing to assess the liquid/solid nature of a highly echogenic cyst in one case. CT confirmed the cystic nature of all masses and showed the lack of contrast enhancement of cyst walls and the presence of septa or parietal calcifications. DISCUSSION: Radiological examinations, particularly US and CT, can diagnose splenic cysts unquestionably, correctly defining the relationships with adjacent organs. CT is more sensitive than US in detecting septa or calcifications, which are definitely useful findings to distinguish true from false cysts, since internal septa are more frequent in true cysts while parietal calcifications are typical of pseudocysts. The final diagnosis, however, is made at histology.     

Surgical treatment of extensive skin necrosis secondary to purpura fulminans in a patient with meningococcal sepsis

This study reports an 8-year-old boy with chronic recurring thoracic pain. Magnetic resonance imaging of the spine revealed an intradural cyst at T1-T2. Despite severe compression of the spinal cord, there was no neurologic deficit. After minimally invasive cystectomy, the patient recovered completely. Histological examination established the diagnosis of a neurenteric cyst. The clinical, pathohistologic, and radiologic aspects are discussed, and the decisive role of magnetic resonance imaging in the diagnosis of intraspinal cyst is emphasized.     

Bile duct cysts in adults

INTRODUCTION: Bile duct cysts are rare, congenital dilations of the intrahepatic and/or extrahepatic biliary tract. Most of them present during childhood. The classical triad right upper quadrant pain, jaundice and abdominal mass is present only in a few instances. We report here the bile duct cysts which were diagnosed at our institution from 1989 to 1996. METHODS: 3245 consecutive endoscopic retrograde cholangiopancreatograms (ERCP) were evaluated retrospectively. Diagnosis was made when localized cystic dilations of the intrahepatic and/or extrahepatic biliary tract were present. Diffuse dilations of the intrahepatic and extrahepatic biliary tract were excluded. RESULTS: Bile duct cysts were found in 20 patients (17 females, 3 males) among 3245 ERCPs. Their mean age was 56 +/- 20 (median 64, range 10 to 83) years. The cyst types (according to the Alonso-Lej classification with the Todani modification) were type I in 11 (55%), type II, III and IV in two instances each (10%), and type V (or Caroli's disease) in 3 patients (15%). Leading symptoms were cholestasis in 14 patients, 10 of whom had abdominal pain, jaundice in 4 patients, and single cases of pancreatitis, cholangitis, and abdominal mass. In 2 patients the diagnosis was made incidentally. 10 patients had bile duct stones. We performed endoscopic sphincterotomy in 15 patients with concretions or persistent symptoms, 3 patients had cyst resection. One of these, with a type I cyst, already had a disseminated cholangiocarcinoma. 10 of 17 patients without cyst resection are currently symptom-free after complete removal of all gallstones. One male patient with cholecystolithiasis, who is not operable due to advanced liver disease, has recurrent cholangitis, 4 patients have died from causes unrelated to the bile duct cysts, and 2 patients are lost to follow up. CONCLUSION: Bile duct cysts in adults are rare. There is a preponderance in the female gender, and the most common type is the extrahepatic (choledochal) cyst. The leading symptoms are cholestasis and right upper quadrant pain. There is an increased risk of cholangiocarcinoma. In young patients the cysts should be entirely removed to prevent malignancy. Older persons are usually symptomless after complete removal of gallstones.     

A gene for FG syndrome maps in the Xq12-q21.31 region

AIMS: An increased risk of adenocarcinoma of the oesophagus has been demonstrated in patients with long segments of Barrett's mucosa. The risk of cancer associated with short segments of metaplasia of the oesophagogastric junction is not known. METHODS AND RESULTS: We report a case of early adenocarcinoma of the oesophagus arising on short tongues of Barrett's mucosa associated with an oesophageal cyst. The patient was a 68-year-old man with no previous clinical history of gastro-oesophageal reflux disease. The fortuitous discovery of an oesophageal cyst lead to the diagnosis of short tongues of Barrett's mucosa with high-grade dysplasia. On pathological examination of the resected specimen, an early adenocarcinoma had developed in Barrett's mucosa, localized just above the oesophageal cyst. CONCLUSIONS: As oesophageal cysts can cause symptoms suggestive of reflux, we hypothesize that this association may not be fortuitous.     

p53 tumour suppressor gene mutations in benign prostatic hyperplasia and prostate cancer

OBJECTIVE: Seminal vesicle cysts combined with ipsilateral renal agenesis represent a rare urological anomaly. We searched the literature to review the clinical presentation, diagnosis and therapeutic treatment options of this anomaly. METHODS: A pooled analysis was performed of 52 cases of seminal vesicle cysts combined with ipsilateral renal agenesis, including our own observation. The evaluation included: patient age at diagnosis, race, laterality (R/L), presence of ureteral remnant in the cyst, presenting symptoms, diagnostic examinations, treatment and outcome. RESULTS: The mean age at diagnosis was 30.2 years. The majority presented in the 2nd, 3rd and 4th decade of their lives. Only 2 patients (4%) were of African origin, all others were Caucasians. The distribution R:L was 2:1. Ureteral remnants were present in 14 patients (27%). The most common symptoms were: dysuria (37%), frequency (33%), perineal pain (29%), epididymitis (27%), pain following ejaculation (21%) and scrotal pain (13%). Infertility was found in 9 patients (17%). The cyst was palpable by digital rectal examination in 79%. All patients underwent intravenous urography, and 88% underwent cystoscopy. Other frequently performed investigations are: ultrasonography (27%), CT scanning (27%), vasovesiculography (46%) and urethrocystography (23%). The final treatment was open surgery in 74%, aspiration in 6%, transurethral deroofing of the cyst in 6% and spontaneous rupture in 4%. In 6% no treatment was given and in 4% the treatment is unknown. All patients were free of symptoms after open exploration. The success rates after transurethral deroofing and aspiration were 75 and 30% respectively. CONCLUSION: Seminal vesicle cysts combined with ipsilateral renal agenesis are a rare urological anomaly, occurring in men in the 2nd to 4th decade of their life. They present with symptoms of bladder irritation and obstruction and with pain in the perineum and scrotum. Epididymitis is frequently found. The diagnostic work-up consists of a digital rectal examination, transrectal and abdominal ultrasonography, CT scan and a cystoscopy. Open surgery and transurethral deroofing of the cyst give excellent results (100 and 75% cure respectively). Aspiration of the cyst should only be used for diagnostic purposes.     

Choledochal cyst and neoplasm: a comprehensive review of 106 cases and presentation of two original cases

Choledochal cysts are prone to complications: cholangitis, biliary cirrhosis, portal hypertension, lithiasis, rupture, pancreatitis, and carcinoma. The coincidence of choledochal cysts and neoplasia ranges from 2.5 to 26 per cent. One hundred six cases of choledochal cysts with neoplasms have been collected from the literature. We have tabulated the results of 68 cases found to have a neoplasm at the initial laparotomy and of 38 patients with pristine choledochal cysts treated electively who subsequently developed a neoplasm. Two original cases are presented. The primary site of neoplasia was not confined only to choledochal cysts. There appears to be a propensity for malignancy to develop anywhere in the biliary tract or gallbladder or pancreas in conjunction with the choledochal cyst. Accompanying choledochal cysts is a high incidence of an anomalous relation at the pancreaticobiliary junction with subsequent malignancy formation. A pathogenetic basis is postulated.     

Laparoscopic approach excision of mesenteric cysts

The authors reports a case of a young woman suffering from mesenteric cyst. The incidence of these lesions is 1 to 100,000 admissions in adult patients and 1 to 20,000 admissions in pediatric age. These cysts, with retroperitoneal and omental cysts, have a similar etiopathogenesis as lymphatic ectopic tissue. In the majority of cases these cysts are asymptomatic and often the diagnosis is intraoperative. In this case the cyst was removed successfully with laparoscopic approach. This mini-invasive method allows a complete and radical excision of the cyst and reduces postoperative hospital stay and morbidity.     

Investigations on the distribution of HBs antigen subtypes

The localisations of bone cysts known under the title of "intraosseous ganglion, synovial cyst of bone and solitary unicameral cyst" are summarised from the literature. Two of the authors' patients are added to the few cases of talar cysts recorded up to the present. In addition, a patient with a calcaneal cyst is described. The cysts can be traumatic or non-traumatic in origin. The various theories concerning their etiology are discussed. The clinical findings, differential diagnosis and therapy of subchondral bone cysts particularly in the region of the foot are considered.     

Screening for intracranial aneurysms in patients with isolated polycystic liver disease

BACKGROUND/PURPOSE: Cloacal exstrophy can now be managed with excellent survival rates and reasonable long-term outcomes with many of these patients living into their late teens and early adulthood. In this report, the authors describe for the first time the association of large ovarian cysts with cloacal exstrophy. METHODS: From 1974 to 1996, 12 patients with cloacal exstrophy have been treated at C.S. Mott Children's Hospital. Massive ovarian cysts developed in four of these. These patients represent the subjects of this study. RESULTS: All four patients have been followed up beyond puberty and massive ovarian cysts have developed, which have caused significant morbidity. Three patients have required surgical intervention. All the patients had reached menarche before the development of the cysts. In all cases, the presentation was severe pelvic pain. Urinary tract obstruction from the large pelvic cysts developed in three of the four. The cysts were bilateral in three of four patients and measured 8 to 10 cm in diameter on ultrasound scan or computed tomography (CT). Cyst aspiration was attempted in two cases and was unsuccessful. Three of the four patients have required bilateral salpingo-oophorectomy. The indications for surgery were uncontrollable pelvic pain in one and urinary obstruction and uncontrollable pelvic pain in two. Surgical findings demonstrated massive thin-walled cysts with essentially no normal ovarian tissue in association with duplicated mullerian structures. The pathology findings were corpus luteal cyst in two and mucinous cystadenoma in one. The fourth patient with an 8- x 10-cm unilateral cyst is being followed up. CONCLUSIONS: The authors have described, for the first time, the association of massive ovarian cysts with cloacal exstrophy. These cysts can lead to severe pelvic pain and urinary tract obstruction. Bilateral oophorectomy has been required in most of these patients.     

Epidemiology of strabismus

A 50-year-old man was admitted, because of motor weakness of the lower limbs, dysesthesia of the left lower extremity, and anuresis. He had an episode of pain in his gluteal region 17 years ago, and then, no abnormalities were detected including myelography in a hospital, followed by slowly progressive muscular atrophy of his lower legs. At 50 years of age, dysuria appeared. He was diagnosed as having neurogenic bladder by urologists, and was admitted to our hospital. On admission, abnormal neurologic findings included: severe muscular atrophy in his lower legs, pes cavus, dysesthesia at the left S1 level, and autonomic bladder. Magnetic resonance imaging (MRI) showed mass lesion involving lower conus and cauda equina. After resection, pathological study revealed the mass was a neurenteric cyst. It is said that the neurenteric cyst causes an asymmetrical and sequential loss of specific neurological functions, with a subsequent return of these functions in the reverse order. That mechanism is not clear. However, in our case, the course of the illness was slowly progressive. We speculate that, because of the cyst's adhesion to cauda equina and perforation through the cyst by a nerve root, the cyst was fixed and caused slowly progressive neurological deficits in proportion to increase of the cyst's size. Our report suggests that a neurenteric cyst, involving the lower conus and cauda equina, can produce severe muscular atrophy in the lower legs.     

Apico-aortic shunt: a support technique during surgery on the descending thoracic aorta

Although colloid cysts of the third ventricle are unusual in children, we have recently encountered six examples. Histologically they were lined by cuboidal, pseudostratified or columnar ciliated and mucous-secreting epithelial cells. Two cases showed small microcysts within the fibrovascular stroma surrounding the main cyst. The outermost layer consisted of a glial-ependymal envelope, in keeping with the postulated supraventricular origin of colloid cysts. Scanning electron microscopy showed 10-40% ciliated cells, and no ballooning of non-ciliated cells. Aspiration of cyst contents was performed in three patients, two of whom subsequently required surgical resection 4 months and 8 years after drainage, respectively. In adults colloid cysts may be asymptomatic, whereas in children they have not been documented as incidental findings at autopsy. Two of our six cases died, both before a diagnosis was established. A colloid cyst of the third ventricle must be included in the evaluation of acute neurological deterioration in children, in whom they are more frequently lethal.     

Mediastinal cyst involving the oesophagus: diagnosis and results of surgical treatment

OBJECTIVE: To describe our experience with mediastinal cysts involving the oesophagus. DESIGN: Retrospective study. SETTING: University hospital, Italy. SUBJECTS: 11 patients who presented to our department with a mediastinal cyst from 1976-1994. INTERVENTIONS: Excision of the mass through a posterolateral thoracotomy (n = 10) or by video-assisted thoracoscopy. MAIN OUTCOME MEASURES: Morbidity and mortality. RESULTS: 8 patients presented with retrosternal or epigastric pain, three of whom had mild dysphagia. In the remaining 3 the tumour was asymptomatic and an incidental finding on a chest radiograph. Endoscopic ultrasonography and computed tomography (CT) allowed preoperative diagnosis of an extramucosal cyst in 5 of the 7 patients investigated by both tests. Masses were excised through a formal thoracotomy (n = 10) or by video-assisted thoracoscopy. Histological examination confirmed a benign cyst in all cases. There was no operative morbidity and nine patients are free of symptoms after a median follow-up of 2.3 years. CONCLUSION: Excision, preferably by thoracoscopy, is the treatment of choice for mediastinal cysts that involve the oesophagus. Special attention should be paid to the vagal nerves, and as many as possible of the muscular layers of the oesophagus should be preserved.     

Varicella-zoster virus ORF57, unlike its pseudorabies virus UL3.5 homolog, is dispensable for viral replication in cell culture

PURPOSE: To assess the efficacy of percutaneous minocycline hydrochloride sclerotherapy in symptomatic hepatic cysts. MATERIALS AND METHODS: From November 1992 to June 1994, seven of eight consecutive adults with large symptomatic hepatic cysts (diameter, 55-130 mm) were treated with a single intracystic injection of minocycline hydrochloride in an ambulatory procedure. Five patients had a solitary cyst, and two had polycystic liver disease. The target cyst was punctured under ultrasound guidance and local anesthesia with a 22-gauge Chiba needle. Half of the cyst content was aspirated before injection of 100-500 mg of minocycline hydrochloride diluted in 5-25 mL of saline. The minocycline hydrochloride was left in the cyst at the end of the procedure. RESULTS: After a mean follow-up of 28 months (range, 24-42 months), all five patients with solitary cysts were asymptomatic and four had documented complete cyst regression; the two patients with multiple hepatic cysts showed only transient clinical improvement. CONCLUSION: Single-shot injection of minocycline hydrochloride is an effective treatment for symptomatic solitary hepatic cysts but is less effective in polycystic liver disease.     

Immunolocalization of ion transport proteins in human autosomal dominant polycystic kidney epithelial cells

The kidneys of patients with autosomal dominant polycystic kidney disease become massively enlarged due to the progressive expansion of myriad fluid-filled cysts. The epithelial cells that line the cyst walls are responsible for secreting the cyst fluid, but the mechanism through which this secretion occurs is not well established. Recent studies suggest that renal cyst epithelial cells actively secrete Cl across their apical membranes, which in turn drives the transepithelial movement of Na and water. The characteristics of this secretory flux suggest that it is dependent upon the participation of an apical cystic fibrosis transmembrane conductance regulator (CFTR)-like Cl channel and basolateral Na,K-ATPase. To test this hypothesis, we have immunolocalized the CFTR and Na,K-ATPase proteins in intact cysts and in cyst epithelial cells cultured in vitro on permeable filter supports. In both settings, cyst epithelial cells were found to possess Na,K-ATPase exclusively at their basolateral surfaces; apical labeling was not detected. The CFTR protein was present at the apical surfaces of cyst epithelial cells that had been stimulated to secrete through incubation in forskolin. CFTR was detected in intracellular structures in cultured cyst epithelial cells that had not received the forskolin treatment. These results demonstrate that the renal epithelial cells that line cysts in autosomal dominant polycystic kidney disease express transport systems with the appropriate polarity to mediate active Cl and fluid secretion.     

Repair of traumatic orbital wall defects with nasal septal cartilage: report of five cases

PURPOSE: Arachnoid cysts are sometimes encountered in MRIs performed for a variety of reasons. In patients with epilepsy, particularly those with refractory epilepsy, arachnoid cysts are often assumed to be related to their seizure focus. We conducted a study to investigate this putative relationship. METHODS: A retrospective study on the incidence of arachnoid cysts was performed in patients seen in our Epilepsy Clinic who had CT or MRI scans, interictal EEGs or ictal EEGS. Locations of seizure foci in these patients were defined from clinical and electrophysiologic data. RESULTS: Seventeen of 867 patients had arachnoid cysts. Twelve patients had temporal lobe cysts and only 3 of them had temporal lobe seizures. Four patients had frontal lobe cysts and only 1 had frontal lobe seizures ipsilateral to the cyst. One patient had a cerebello-pontine angle cyst and frontal lobe seizures. Thus, clinical manifestations of seizures and EEG findings (interictal and/or ictal) indicated that the seizure focus was adjacent to the cysts in only 4 patients (23.5%). CONCLUSIONS: Our findings suggest that arachnoid cysts are often an incidental finding in patients with epilepsy and do not necessarily reflect the location of the seizure focus.     

Pericardial cysts. Report on 9 treated cases

From 1976 to 1993, nine patients (5 men, 4 women) with pericardial cysts were treated in Authors' Department. Of the nine cysts, six were located in the right cardiophrenic angle, one in the subcarinal site, one in the right tracheobronchial angle, and one in the para-auricular site just above the diaphragm. Four patients were asymptomatic. A correct diagnosis was possible preoperatively only in patients with cysts typically located in the cardiophrenic angle. Eight patients were surgically treated by a standard posterolateral or axillary thoracotomy. One patient with a large pericardial cyst underwent needle percutaneous aspiration and CT-guided drainage of the cyst with a positive outcome. There was no operative morbidity or mortality.     

Malignant change in the biliary tract after excision of choledochal cyst

BACKGROUND: Choledochal cyst is a rare congenital condition with a high risk of malignant change if untreated. The risk of malignancy after surgical excision of choledochal cyst is not known. METHODS: Forty-eight patients with choledochal cysts managed over a 21-year period were reviewed, to determine the risk of malignant change after cyst excision. Thirty-nine of 48 patients had no carcinoma at first admission; their mean(s.d.) age was 20(18) years. Thirty-seven of 39 patients underwent cyst excision and cholecystectomy followed by hepaticoenterostomy. RESULTS: Cyst excision was incomplete in 28 of the 37 patients because dilated portions of the biliary ducts remained proximally and/or distally. In these 37 patients, no carcinoma has developed in the remnant proximal hepatic duct or the terminal bile duct after mean(s.d.) follow-up of 9.1(6.4) years. In the remaining nine patients, biliary carcinoma was diagnosed at the first visit. Six patients died from recurrence with a mean(s.d.) survival time of 13(11) months, while three patients were alive and free from recurrence 2 months, 1 year and 7 years after operation. CONCLUSION: Malignant change has not been observed after total or subtotal excision of choledochal cysts in this series.     

Arachnoid cyst rupture producing subdural hygroma and intracranial hypertension: case reports

OBJECTIVE: To analyze the association between arachnoid cysts and subdural hygromas. METHODS: We reviewed five cases of arachnoid cysts that ruptured, producing acute subdural hygromas. The surgical management and diagnostic methods used are assessed. RESULTS: Five male patients ranging in age from 6 to 25 years sustained the rupture of arachnoid cysts, which produced acute subdural hygromas. Four of the patients had incurred blunt head trauma. All patients presented with symptoms referable to intracranial hypertension. The pathognomonic features of a middle fossa arachnoid cyst (MFAC) were noted on the computed tomographic scans and/or magnetic resonance images of each patient. The hygroma exerted mass effect on the ipsilateral hemisphere and was noted to be under significant pressure at the time of surgical intervention in each case. Two of the five cases are unique in the literature. In one, a coexisting quadrigeminal cyst ruptured, producing a subdural hygroma ipsilateral to the MFAC and dilating the basal cisterns. In the other, the MFAC ruptured into the basal cisterns as well as into the subdural space. The MFAC in each of the remaining three patients ruptured into the subdural space alone. All patients were treated with drainage of the subdural space. In the two patients in whom the basal cisterns were involved, both the hygromas and the MFACs failed to change significantly in size. The hygromas resolved completely and the MFACs decreased in size considerably in the three patients without cisternal involvement. CONCLUSION: The rupture of an arachnoid cyst can produce a subdural hygroma and intracranial hypertension. The latter mandates emergent drainage of the subdural space. In patients in whom the basal cisterns are not dilated by cyst rupture, both the MFACs and hygromas resolve after subdural drainage.