Tailgut cysts

Retrorectal cystic hamartomas (tailgut cysts) are rare congenital lesions thought to arise from remnants of the embryonic postanal gut. They predominantly occur as asymptomatic retrorectal multicystic masses in women. The treatment of choice is by complete surgical excision. The most important complications of these cysts are infection with a secondary fistula and malignant degeneration. The differential diagnosis includes a wide variety of conditions that occur in the retrorectal space. In this article, 3 cases showing different surgical technical aspects of treatment are presented. In addition, the aetiopathogenic features and histopathological appearance, clinical presentation and complications, imaging features and differential diagnosis of tailgut cysts are described.     

Bronchogenic cysts

Between 1967 and 1987, 26 patients with bronchogenic cysts were treated in our institution: 15 females and 11 males, ranging in age from 2 months to 14 years (average 6 years, 4 months). Only four patients (15%) were 1 year old or less. Seven patients presented with asymptomatic lesions discovered on chest film. Nineteen patients presented with respiratory symptoms. Of note are the four patients aged 1 year or less, who presented with dyspnea and cyanotic spells. Only one of them presented with dysphagia. All patients underwent routine chest films and tomography and, later, computed tomography (CT) scan of the thorax once the study became available. Barium esophagogram and bronchoscopy were performed as required. Contrary to what has been published concerning the intraparenchymal location of bronchogenic cysts, the majority of the cysts (65%) were located in the mediastinum, usually in close proximity to the carina, mainstem bronchi, trachea, esophagus, or pericardium. Only 27% (7 of 26) were intraparenchymal, and 8% (2 of 26) were situated in the inferior pulmonary ligament. A correct preoperative diagnosis was made in 19 patients (73%). Intrapulmonary lesions were removed by segmentectomy or lobectomy. Cysts located elsewhere were simply excised. Among the cysts adhering to the trachea, 50% (two of four) required suture of the membranous trachea after excision. Postoperative complications included one pneumonia, which responded to antibiotics, and one transient paresis of the right phrenic nerve. Follow-up averaged 17 months (range, 0 to 17 years). No sequelae were noted and all patients were cured.     

Discal cysts

Discal cysts are rare lesions that can result in refractory sciatica. Because they are so rare, their exact origin and details of the clinical manifestations are still unknown. The authors report on five men treated for discal cysts. The mean age of the patients at the time of the surgery was 32 years (range 25-38 years). All patients suffered from lower-extremity pain and the results of the straight leg-raising test were positive in all cases. Three patients reported motor weakness and four had sensory disturbance--symptoms similar to those found in patients with lumbar disc herniation. Magnetic resonance imaging demonstrated spherically shaped extradural lesions of various sizes with low and high signal intensities on T1- and T2-weighted images, respectively. Discography revealed obvious communication between the cyst and the intervertebral disc with reproducible leg pain in all patients. All patients underwent posterior decompression and excision of the cysts either with or without additional discectomy. The radicular symptoms were remarkably improved in all patients immediately after surgery, and no recurrent lesions were noted during follow up.     

Urachal cysts

The work analyses observation findings in three patients with urachal cysts. On admission, two of the patients had suppurating cysts simulating the picture of the acute abdomen, one of which presented the clinical signs of acute appendicitis so vividly as to require revision of the abdominal organs. The diagnostic errors that have been made are also analysed. A conclusion is derived by the authors on the expediency of an active surgical tactics in cases of suppurating urachal cysts. They suggest extensive cyst excision with application of continuous suture and drainage of the wound to be the operation of choice.     

Parathyroid cysts

Two cases of parathyroid cysts are described. One cyst was symptomatic, the other was discovered while performing thyroidectomy. In both cases, the cysts were non-functional. There have been reports of functional cysts occurring in a lesser proportion and causing primary hyperthyroidism. These benign cysts are rare (300 cases reported in the literature). Surgery is the treatment of choice in most cases.     

Mesenteric cysts

Mesenteric cysts continue to be of special interest because of their diverse presentation and the lack of definitive diagnostic tests. Nine patients with mesenteric cysts were treated at St. Agnes Hospital between 1973 and 1984. The most common location was in small bowel mesentery (67%) followed by the mesocolon (33%). The mesenteric cysts were incidental findings in four of the nine patients (45%). Enucleation was the treatment of choice in eight patients (88%), followed by right hemicolectomy in one patient. Two patients had complications, one wound infection and one urinary tract infection. Simple enucleation has proven to be adequate treatment in most instances. Large bowel resection may be necessary when mesenteric cysts are located in the mesocolon and enucleation is not feasible without compromising the blood supply to the adjacent bowel.     

Splenic cysts

The treatment of splenic cysts is a difficult challenge to surgeons and physicians. This paper reviews the literature on splenic cysts, with special attention to the pathogenesis, diagnosis, and various options of surgical treatment. Splenic cysts are classified as primary or secondary cysts, according to the presence of an epithelial lining. The primary cysts are further subdivided as parasitic or non-parasitic. Secondary cysts are in most cases posttraumatic. Symptoms are usually correlated to the size of the cyst. Prior to surgery, imaging with ultrasound and computer tomography or magnetic resonance should be performed. A cyst puncture should be conducted for diagnostic purposes (amylase and bacteria) as well as to reduce the size of the cyst. Furthermore, the titer of Echinococcus and other biomarkers can be measured. Surgeons should make every possible effort to preserve splenic tissue and spleen-saving techniques with laparoscopic techniques are recommended.     

Parathyroid cysts

Fourteen new cases of parathyroid cyst are presented. Two distinct groups were identified: functioning and nonfunctioning. Patients with functioning cysts presented with hypercalcemia, tended to be men and were older. Patients with nonfunctioning cysts were all women, had normocalcemia and all presented with neck masses. Ultrasonography proved useful while radionuclide thyroid scans were often misleading. In the functioning group removal of parathyroid cysts resulted in postoperative normocalcemia in all cases. In the nonfunctioning cysts thyroid lobectomy was performed in five of six instances. Parathyroid cysts usually contain clear or serous fluid and are surrounded by a well-delineated plane of dissection. We believe that preoperative aspiration of cystic neck masses and consideration of the diagnosis of parathyroid cysts may avoid needless thyroid lobectomy.     

Parathyroid cysts

From a review of the world literature, 91 cases of parathyroid cysts have been identified. The majority present as asymptomatic lumps in the lower part of the neck to the right or, more frequently, the left of the midline and are diagnosed as thyroid nodules before surgery. The 4 cases discussed in this article were all derived from the left lower parathyroid gland or left upper pole of the thymus. Three are the largest on record, 2 of which produced pressure symptoms and one, scabbarding of the trachea.

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Résumé La revue de la littérature mondiale a permis de recenser 91 cas de kystes parathyroidiens. Ces lésions se traduisent le plus souvent par la présence d'une masse au niveau de la partie inférieure du cou située soit à droite soit plutôt à gauche ou au niveau de la ligne médiane. Elles sont considérées le plus souvent comme des nodules thyroïdiens. Les 4 cas de kystes rapportés dans l'article se situaient au niveau de la glande parathyroidienne inférieure gauche ou du pôle supérieur du thymus. 3 revê-taient la taille la plus importante qui ait été décrite, 2 entrainaient des manifestations de compression, 1 se traduisait par un engainement de la trachée.

     

Parathyroid cysts

Cystic neck masses may be accurately diagnosed by sonography, and some nonfunctioning parathyroid cysts can be cured by percutaneous aspiration. Primary hyperparathyroidism should be considered in all patients with cystic neck masses. Parathyroid cysts can often be recognized from the characteristics of the cyst fluid which is usually clear and colorless, contains elevated parathyroid hormone levels and normal or low thyroid hormone levels, and may contain parathyroid cells. Parathyroid cysts may be multiple; all four parathyroid glands should therefore be identified and appropriately removed or hyperparathyroidism may persist.     

Adrenal cysts

Suprarenal cysts may be clinically silent and discovered by chance, or they may present clinically if they attain marked dimensions by means of compression of surrounding formations. Examinations that lead to precise diagnosis are: direct abdominal X-ray integrated with scanning, echoscanning, urography and selective arterial and especially venous vasography. Two clinical cases that may be considered typical of the two varieties, true cyst and pseudo-cyst, are reported.     

Enterogenous cysts

This case of an enteric cyst is presented, first because of its extreme rarity, and second for the many interesting theories of development that can be followed from the embryologic state to the pathologic culmination.     

Retroperitoneal mesenteric cysts

Retroperitoneal cysts are most commonly found in relation to the small bowel but may be found in the mesentery of the colon or in the omentum. Only a few hundred cases have been reported. In the present analysis four more cases are reported and the pertinent literature is reviewed. The diagnosis is made by clinical suspicion, barium meal and/or enema, sonography and axial computerized tomography. Treatment of choice is by enucleation of the cyst, if possible. Morbidity and mortality should be very low, because of recent advances in surgery and intensive care of the patient.     

Intrapulmonary bronchogenic cysts

Bronchogenic cysts are commonly found in the mediastinum, but occasionally in lung parenchyma. We report 3 patients with intrapulmonary bronchogenic cysts. A female presented back pain and a large cystic lesion with an air-fluid level on chest X-ray and computed tomography (CT). Another female was asymptomatic. A well-defined round mass with soft tissue intensity on magnetic resonance imaging (MRI) was found. A girl with repeated pulmonary infection and treatment in hospital for several years presented pulmonary infiltrate, air-fluid level, and oval linear shadow on X-rays. All patients underwent lobectomy because of the size and the non-anatomical location of their lesions. Definite diagnosis on their lesions was determined by pathological study. Several small communications between the cyst and adjunctive parenchyma was found in the symptomatic girl, while the cyst was isolated from surrounding parenchyma in the asymptomatic female. We suggest symptoms and various presentations on imaging modalities in patients with intrapulmonary bronchogenic cysts might relate to the amount of original communication between their cysts and native lung.     

Subcutaneous bronchogenic cysts

Recent experience with a bronchogenic cyst occurring in a subcutaneous suprasternal location presents an additional entity to be considered in the differential diagnosis of head and neck lesions, and correlates with the embryology of the developing tracheobronchial tree and sternum. A review of subcutaneous bronchogenic cysts shows 41 cases have been described in the English literature. Most were noted in the suprasternal location; all but one were first noted in infancy or childhood. There is a definite male predominance. Sinus tracts were present in four cases, but none extended into the chest or mediastinum. Although local excision led to cure in all cases, incomplete excision in two cases required reoperation. Embryologic maldevelopment is suggested as the etiology for subcutaneous bronchogenic cysts, with a "pinching off" of pulmonary parenchyma by closure of the developing lateral sternal bars, fusion of which results in an isolated bronchogenic cyst in the subcutaneous tissues of the anterior chest wall. Awareness of this lesion by pediatric surgeons in the differential diagnosis of masses or sinuses presenting in the suprasternal region may prevent incomplete excision of components, which can extend deep into the site of origin. The possibility of extension into the mediastinum should be entertained.     

Complicated choledochal cysts

Choledochal cysts have a potential for causing a variety of complications. This article presents the incidence, types, and presentation of complications in patients with choledochal cysts and the results of surgery in these patients. Prospectively collected data of 81 patients with choledochal cysts treated at a tertiary care center were retrospectively analyzed. Sixteen (20% of surgical patients; 100% of study group) patients were found to have a complicated choledochal cyst that either manifested as an atypical presentation or necessitated a change in the treatment planning. Eight (50%) of these patients had complications of infective nature, and another 8 (50%) had noninfective complications. Fifteen (94%) patients with complications were adults and 11 (63%) patients had Todani type-IVa cysts; a cystic type of intrahepatic component in 10 patients. Five (31%) patients had undergone prior cystenteric drainage operations. Excision of the choledochal cyst was possible in 14 (88%) patients under both emergency and elective situations; cyst excision was performed in the second stage after an initial external drainage procedure in two of these patients. There was no mortality and no major postoperative complications. Complications of choledochal cysts are more likely in adult patients and in those with both intra- and extrahepatic cysts (Todani type-IVa). Despite the presence of complications, the choledochal cyst can be safely excised in a majority of patients.     

Infratentorial arachnoid cysts

The infratentorial compartment represents the second most common location of arachnoid malformations. Ten arachnoid cysts of the posterior fossa, operated on between 1970 and 1983, are reviewed. These lesions, although congenital and developmental in nature, may present at any age, and males are more frequently affected. A high rate of birth-related trauma (50% in this series) is conceivably due to fetal macrocranium, and the enlarged head and psychomotor retardation prevail in infancy and childhood. In arachnoid cysts occurring during adulthood, symptoms and signs more clearly indicate a dysfunction of the posterior fossa. Besides computerized tomography, pneumoencephalography and metrizamide techniques are recommended to rule out a Dandy-Walker syndrome in doubtful cases, and to obtain information about the cerebrospinal fluid (CSF) circulation. It is particularly important to establish the presence and type of communication of cysts with the CSF pathways. Although infratentorial cysts often communicate, they can be space-occupying masses because of increasing CSF retention, which may be due to a ball-valve mechanism or to inadequate communication. The frequently associated hydrocephalus (seven of the 10 cases in this series had hydrocephalus) seemed to be dependent mainly upon mechanical factors. The authors discuss the indications for intracranial surgery versus shunting procedures and report the results achieved by direct cyst excision.     

Unicameral bone cysts

Unicameral, or solitary, bone cysts are unusual tumors seen in the ends of long bones in skeletally immature persons. The etiology of these lesions is poorly understood. Various hypotheses have included dysplastic processes, synovial cysts, and abnormalities in the local circulation. Most patients present with a nondisplaced pathologic fracture, but occasionally cysts are found incidentally. Plain radiographs typically show a symmetric lesion with cortical thinning and expansion of the cortical boundaries. Once diagnosed, unicameral bone cysts continue to be a treatment dilemma. Traditional methods, such as prednisolone therapy, usually involve multiple anesthetics and injections and are associated with high recurrence rates. Major surgical procedures, such as wide exposure, curettage, and bone grafting, may be somewhat more effective, but still carry with them significant morbidity and recurrence rates. Newer techniques involving percutaneous grafting with allograft or bone substitutes or a combination of the two are promising in light of their low complication rate and lower reoperation rate.     

Biliary cysts.

This review brings the total number of biliary cysts reported in the world literature to 955. Eighty-one per cent of patients are females and 61% were discovered before age ten. The classical triad of right upper quadrant pain, right upper quandrant mass, and juandice is present in 38% of cases. The duration of symptoms prior to diagnosis ranged from less than one week to more than 40 years. The etiology is multifaceted and evidence of the existence of both acquired and congenital cysts is presented. The most useful diagnostic tool is fiberoptic endoscopy with retrograde contrast injection of the common bile duct and pancreatic duct. The incidence of biliary carcinoma in patients with biliary cysts is found to be 2.5%; 24 cases have been reported. Considerable controversy has existed concerning the best operative procedure for biliary cysts; no treatment or medical treatment yielding a 97% mortality rate. In an analysis of 235 patients presented since 1968 with an average followup of 5.2 years, the best procedure appears to be excision with either choledochocholedocostomy or Roux-en-Y hepaticojejunostomy. The operative mortality for all procedures is now 3 to 4%.     

Supratentorial dermoid cysts

Supratentorial dermoid cysts are rare lesions. In eight cases presented here, the lack of recurrence after subtotal removal of the capsule and the good long-term prognosis are emphasized. This finding is in agreement with the literature. The frequent relationship of these lesions with the cavernous sinus suggests a vascular genesis in the development of intracranial dermoid cysts.