Pericardial cyst diagnosed by two-dimensional echocardiography and computed tomography in a newborn

Summary A one-day-old full-term female infant with massive “cardiomegaly” developed marked respiratory distress soon after birth. The two-dimensional (2D) echocardiogram demonstrated a 4×5-cm cystic mass adjacent to the left ventricular free wall. Computed tomography (CT) confirmed the presence and dimension of the cyst and CT number indicated the fluid within the cyst to be serous. A pericardial cyst was successfully removed at thoracotomy. Two-dimensional echocardiography and CT, both noninvasive procedures, are fully adequate tools in the diagnosis of pericardial cysts in the neonate, so that invasive investigations may be avoided.     

Cervical thymic cysts

Thymic cysts are rare embryonic remnants along the course of thymic migration in the neck or the anterior mediastinum which may result in cervical masses in children, often misdiagnosed. We present the experience gained by three European tertiary care medical centers in the treatment of thymic cysts as well as the current data on the embryology, clinical presentation, diagnosis and management of thymic cysts. A retrospective study was carried out in nine patients with thymic cysts during the period 1986–2002 at the departments of Pediatric Surgery of Children’s University Hospital “Federico II” and “Santobono” Pediatric Hospital of Naples in Italy and “Aghia Sophia” Children’s Hospital of Athens in Greece. All cases were asymptomatic, appearing mainly as masses resembling branchial cyst or lymphatic malformation. Laboratory and imaging investigations were not useful for preoperative diagnosis. In one case the mass extended into the mediastinum. The histological findings of thymic tissue and Hassal’s corpuscles in the cystic wall were diagnostic. In all cases, surgery was successful and uneventful. Surgical excision was accomplished by dissection of the cystic masses from the jugular vein, carotid artery and vagus nerve and from the sternocleidomastoid muscle. The presence of a normal thymus in the mediastinum must be documented preoperatively in order to avoid the risk of total thymectomy. If a cervical thymic cyst extends into the normal thymus, attempts should be made to preserve the thymus, especially in younger patients. Thymic cysts should always be included in the differential diagnosis of lateral cervical masses, especially in children. Bruno Cigliano and Nikolaos Baltogiannis contributed equally to the work.     

Choledochal cyst sans cyst--experience with six "forme fruste" cases

 Six children presented with clinical symptoms suggestive of a choledochal cyst (CC), but were found to have only mild dilatation of the common bile duct. Further imaging revealed a long common pancreaticobiliary channel in all cases. There was complete relief after surgery provided a pancreaticobiliary disconnection was incorporated in the operative management. A high index of suspicion is required to diagnose this condition, which has been previously reported as “forme fruste CC”. Accepted: 12 February 2001     

Necrotizing myofasciitis: an atypical cause of “acute abdomen” in an immunocompromised child

We report the case of an immunocompromised 15-year-old boy who presented with symptoms mimicking an “acute abdomen” related to necrotizing myofasciitis of the anterior abdominal wall. CT demonstrated the abdominal wall process as the cause of the patient's symptoms and sonographically guided aspiration confirmed the diagnosis. Despite prompt diagnosis and aggressive surgical debridement, the infection continued to progress and the patient died within 24 h of presentation. Received: 12 August 1997 Accepted: 25 September 1997     

紧贴囊壁剥离法切除胆总管囊肿的临床评价

目的：对紧贴囊壁剥离法（或称疝囊剥离法）切除胆总管囊肿的安全性及可行性进行评估。方法：回顾1984－1999年的胆总管囊肿113例，其中106例行囊肿切除、胆肠Roux-en-Y吻合术，均是采用敞开囊腔紧贴囊壁剥离法切除囊肿。结果：106例手术均顺利，无意外损伤，失血量、输血量绝大多数在200ml以内，3例未输血，无严重并发症及死亡.结论：敞开囊腔紧贴囊壁剥离法简化了手术操作和降低了手术难度，增加了手术安全性，因而被认为是切除胆总管囊肿的安全、有效、易掌握的方法。     

Temporal dermoid – an unusual presentation

 It is rare for a frontotemporal dermoid cyst to present as a discharging sinus, and even more rare for it to have intracranial extensions. Only a few cases of intraorbital extension have been reported. We report a 14-month-old girl who presented with all the aforementioned features. She had a temporal dermoid with three discharging sinuses over the temporal area, lower lid, and cheek. It also had an intraorbital extension through the lateral orbital wall and an intracranial extension through the temporal bone. Preoperative computed tomography was done as there was a history of “orbital cellulitis”. Excision of the cyst was done using a hemicoronal-preauricular incision. A lateral orbitotomy was required to remove the orbital component in continuity with the rest of the cyst. Accepted: 13 September 1999     

Duplication of the rectum: report of four cases and review of the literature

Rectal duplications are rare anomalies. Recently, we observed four cases of rectal duplication, each presenting with different clinical features including chronic constipation, a prolapsing rectal “polyp,” a “growth” from the vulva, and acute retention of urine. The variety of clinical presentations may lead to delay in diagnosis and multiple operations. Accepted: 28 October 1997     

Overview of vascular disease

Vascular disease in the pediatric population is a poorly understood process which is often underestimated in its incidence. The common beginnings of such ubiquitous diseases as atherosclerosis manifest themselves at a cellular level shortly after birth. Other common systemic disorders, including congestive heart failure and sepsis, are also intricately associated with dysfunctional vasculature. Progress in the understanding of normal and pathophysiologic processes within the vascular system begins with the “control center”– the endothelial cell. The purpose of this review is to consolidate a body of knowledge on the processes that occur at the cellular level within the blood vessel wall, and to simplify the understanding of how imbalances in these physiologic parameters result in vascular disease. Received: 16 September 1997 Accepted: 12 October 1997     

Computerised tomography in the evaluation of expansile lesions arising from the skull vault in childhood — a report of 5 cases

Expansile lesions of the skull vault are rare in childhood, and often present as relatively asymptomatic calvarial swellings. The cases of 5 children with expansile lesions of the skull vault due to both benign and malignant primary bone lesions are described. The value of computerised tomography in demonstrating that the “tumour” arises primarily from the skull vault as opposed to the underlying brain, and in demonstrating clinically unsuspected endocranial extension of the mass is described. The CT findings in 2 cases of aneurysmal bone cyst, including the significance of the presence of “fluid levels” in reaching a definitive diagnosis are discussed. The successful pre-operative embolisation in one case of aneurysmal bone cyst is reported.     

Cerebellopontine angle arachnoid cyst harbouring ectopic neuroglia

Arachnoid cysts are collections of cerebrospinal fluid covered by arachnoidal cells and collagen. They may have a congenital or acquired origin. We present the case of a 7-year-old boy with a cerebellopontine angle arachnoid cyst harbouring a portion of neuroglia. Microscopically, the cyst wall consisted of a delicate fibrovascular membrane lined by a layer of arachnoidal cells. A thin layer of glial tissue ran the whole length of the cyst wall. This finding supports a congenital origin for this case. Cerebellopontine angle arachnoid cysts harbouring neuroglia without ependymal elements are very rare lesions and may be successfully treated by surgical resection.     

Gonadal preservation in a simple testicular cyst

The authors report a case of a simple testicular cyst in a 16-week-old infant. Preoperative ultrasonography helped to diagnose this condition accurately. Gonadal preservation and enucleation of testicular cysts are possible with a careful surgical approach, and orchiectomy is not necessary. Accepted: 18 March 1997     

Pneumonostomy in the surgical management of bilateral hydatid cysts of the lung

 During the period 1994–1998, three patients with bilateral hydatid cysts of the lung (HCL) underwent operative removal of the cysts. In three of the six lungs operated upon the conventional technique was used: after removal of the cyst and suture closure of bronchial leaks, the chest was closed with an intercostal drainage tube. Two of these patients developed bronchopleural fistulae requiring rethoracotomy and prolonged hospital stays. The other three lungs were operated upon using the pneumonostomy technique: after excision of the cyst a separate catheter is fixed within the residual lung cavity and brought out through the adjacent chest wall, effectively marsupialising the residual cavity to the atmosphere. All these patients had an uneventful postoperative recovery. We conclude that the pneumonostomy technique is a very useful method of treating HCL surgically, especially when the cysts are bilateral and complicated. Accepted: 13 January 2000     

Third ventricular ependymal cyst presenting with acute hydrocephalus

Ependymal cysts are generally located in the cerebral parenchyma but rarely found in the third ventricle. A 4-year-old boy presented with headache, vomiting, and upward gaze palsy. His consciousness gradually deteriorated in the course of 6 h. A magnetic resonance imaging study disclosed dilation of the lateral ventricle and a cystic mass in the third ventricle. We performed an endoscopic resection of the cyst wall. The cyst originated on the lateral wall of the third ventricle and obstructed the aqueduct. Histological examination confirmed a diagnosis of ependymal cyst. The patient recovered quickly and his headache and nausea disappeared. Third ventricular ependymal cysts are a rare cause of acute hydrocephalus but an important differential diagnosis. Their neuroendoscopic resection can resolve disturbances in cerebrospinal fluid circulation, is useful for cyst wall removal, and appears to be superior to shunt placement.     

Head-wag autotomography of the upper cervical spine in infantile torticollis

Background. Infants with torticollis require evaluation of the entire cervical spine for vertebral anomalies as part of the work-up, but open-mouth views to assess occiput to C2 are difficult to obtain. Objective. To evaluate a “head wag” autotomographic technique for the frontal projection of this region. Materials and methods. Twenty infants under one year of age were evaluated using this technique. Results. Sixteen of 20 could be adequately evaluated using this simple, low-radiation-dose procedure. Conclusion. For infants with torticollis in whom vertebral anomalies need to be excluded, the “head wag” technique is useful. Received: 18 November 1997 Accepted: 31 December 1997     

Paratubal cysts in young females as an incidental finding in laparotomies performed for right lower quadrant abdominal pain

Paratubal cysts represent remnants of the paramesonephric or mesonephric duct, the former being more common. Most of them are asymptomatic, but they may rarely give rise to clinical problems due to enlargement or torsion. In this study, we looked for paratubal cysts or other pathological lesions of the right uterine adnexa in young females operated upon for right lower quadrant (RLQ) abdominal pain. Our material consisted of 338 white female patients aged 4–14 years. Laparotomy revealed only acute appendicitis in 283 cases, while 44 had acute appendicitis plus a coincidental paratubal cyst. Most of these cysts were smaller than 1 cm in diameter. However, 2 additional cases had torsion of a large paratubal cyst, while 5 had a ruptured corpus luteum. We suggest that a thorough search of the right adnexa for the presence of paratubal cysts during laparotomies performed for RLQ pain is a useful procedure. This approach also allows detection of other pathological lesions of the right adnexa that often produce symptoms similar to acute appendicitis. Accepted: 15 March 1997     

Large, septated ileal duplication cyst in a 4-year old, simulating the urinary bladder

A 4-year-old boy presented with acute abdominal pain and a 1-year history of intermittent umbilical complaints. Ultrasonographic examination demonstrated a large cyst in the lower abdomen divided by a thin membrane. With micturition, impaired bladder function was expected. Initial suprapubic drainage produced 1,000 ml dark-brown liquid. Laparotomy revealed an ileal duplication cyst. Preoperative computed tomography and laparoscopy with the cyst drained did not further clarify the diagnosis. Accepted: 5 August 1997     

Ectopic choroid plexus within a juvenile arachnoid cyst of the cerebellopontine angle: cause of cyst formation or reason of cyst growth

The unusual and rare case of a 6-year-old boy is reported who presented with an arachnoid cyst located in the cerebellopontine angle incorporating an ectopic piece of choroid plexus tissue. A microneurosurgical cyst wall resection was performed and the plexus tissue identified and removed. The rare occurrence of ectopic choroid plexus tissue within cysts of the CNS is discussed.     

Growth, puberty and hypothalamic-pituitary function in children with suprasellar arachnoid cyst

A suprasellar arachnoid cyst may cause disorders of growth, puberty and hypothalamic-pituitary function, due to the proximity of the cyst to the hypothalamic-pituitary area. A total of 30 patients (17 boys) with cyst diagnosed at 4.3 ± 1 years were routinely evaluated at 5.4 ± 1 years; 24 of them had one or multiple cyst derivations. Some 23 cases had an abnormal height, weight or puberty: short (<−2SD, 5 cases) or tall (>2SD, 10 cases) stature, overweight (body mass index, BMI, >2SD, 6 cases), central precocious puberty (10 cases) and/or no progression of pubertal development (3 cases). The growth hormone (GH) peaks after pharmacological stimulation test were low (<10 μg/l) in 16 patients, confirmed by a second evaluation in 8/11 of them. The plasma free thyroxine was low in five patients, prolactin was high in two and the cortisol and concomitant plasma and urinary osmolalities were normal. BMI was correlated negatively with the GH peaks (r=−0.37, P < 0.01) and positively with the plasma leptin concentrations (r=0.55, P < 0.01). The plasma fasting insulin concentrations were also correlated negatively with the GH peaks (r=−0.55, P < 0.02) and positively with the plasma insulin-like growth factor I concentrations (r=0.64, P < 0.002). The adult height (12 cases) was at 4SD in 1 and <−2SD in 4 patients, two of whom had precocious puberty untreated with gonadotropin releasing hormone (GnRH) analogue, and two had untreated GH deficiency. The adult height of those treated was normal. One girl had primary amenorrhoea and two boys had low plasma testosterone, despite a normal gonadotropin response to a GnRH test. Conclusion Suprasellar arachnoid cysts may cause deficiencies of growth hormone and thyrotropin, stimulation of the hypothalamic-pituitary-gonadal axis, tall stature and/or overweight. These last two disorders may be due to hyperinsulinism, itself due to suprasellar arachnoid cyst. Received: 5 May 1999 / Accepted: 28 October 1999     

Hemisplenectomy for giant splenic cysts in children

To analyse the clinical features and outcome following surgery of giant splenic cysts in␣children, all patients presenting to a children's hospital over the years 1987–1996 were reviewed. Variables analysed included presenting symptoms, method of diagnosis, operative procedure, and complications. Six patients (aged 8–16 years) presented with abdominal pain or a large abdominal mass. Ultrasound examination demonstrated large (>5 cm) unilocular splenic cysts. Five of the six children underwent hemisplenectomy without complication or blood transfusion. The remaining patient had an infected cyst, for which total splenectomy was necessary because of major intraoperative haemorrhage. Hemisplenectomy is based on accurate dissection of the splenic hilum with clear knowledge of the intracapsular vascular anatomy of the spleen. Preoperative vaccination is recommended in the event that splenectomy is required. This retrospective review concludes that hemisplenectomy for splenic cysts is a safe procedure in children. Accepted: 3 November 1997     

Transformation of corneal epithelial cyst into anterior chamber implantation cyst and scleral cyst: a rare occurrence

An 8-year-old boy underwent a penetrating keratoplasty for a corneal epithelial cyst. After surgery an anterior chamber implantation cyst appeared. Repeated Neodymium:YAG (Nd:YAG) laser treatments failed to eradicate this cyst; it later extended into the sclera together with the collapse of the anterior chamber cyst. The possible mechanism underlying the development of these cysts is discussed.