Differentiation between pediatric spinal arachnoid and epidermoid-dermoid cysts: is diffusion-weighted MRI useful?

Background Appropriate differentiation between epidermoid-dermoid and arachnoid cysts is important for patient management. MRI has had limitations in differentiating these two types of cysts, especially in the region of the spinal cord. Objective To evaluate the role of diffusion-weighted (DW) MRI for differentiation between spinal arachnoid and epidermoid-dermoid cysts. Materials and methods Four children underwent conventional and DW MRI. Turbo spin-echo imaging was used to obtain DW images. Results On conventional MRI, low T1-W and high T2-W signal was present in all lesions. DW imaging showed restricted diffusion in two patients with epidermoid-dermoid cysts and no water restriction in two patients with arachnoid cysts. Three of four children had extramedullary lesions and one patient had an intramedullary lesion. Three children had surgery, two with epidermoid-dermoid cysts and one with arachnoid cyst. Diagnosis was confirmed by histopathology. One child with a thoracic arachnoid cyst had stable clinical imaging findings during a follow-up of 21 months. Conclusion On conventional MRI, epidermoid-dermoid and arachnoid cysts have similar signal characteristics. Initial experience with spine DW imaging shows promise in differentiating epidermoid-dermoid cysts from arachnoid cysts. Preoperative differentiation is important because it changes management and surgical approach.     

小儿椎管内肠源性囊肿的诊断与治疗

目的探讨小儿椎管内肠源性囊肿的临床表现、MRI影像及显微手术治疗。方法回顾性分析1996年至今8年间5例椎管内肠源性囊肿的临床资料，并结合文献加以分析。结果全部病例采用显微外科手术治疗，其中2例全切，2例次全切除，1例大部切除。无一例死亡，经病理报告证实均为肠源性囊肿，术后神经根痛症状基本消失。随访0．5～2年，无一例复发；格拉斯哥预后评分，良好恢复4例，中残1例；脊髓损伤的Frankel分级，E级4例，D级1例。结论小儿椎管内肠源性囊肿有典型的临床表现和MRI特征，作为一种先天性病变，其最佳治疗方法是早期确诊并采取显微外科手术方法切除。     

Spinal neurenteric cyst. Report of 4 cases and review of the literature

Neurenteric cysts are rare congenital lesions of the spine that are lined with entodermal epithelium. We describe 4 unusual cases of neurenteric cysts in patients aged between 3 and 16 years. The cyst was situated anteriorly in 3 patients and posteriorly in 1. All the patients had varied clinical and radiological features. The child with dorsal neurenteric cyst had stigmata of spinal dysraphism along with other vertebral anomalies. Another child had an associated syringomyelia along with evidence of severe arachnoiditis. All 4 patients underwent laminectomy and total microsurgical excision of the cysts through a posterior approach. The patient with diastematomyelia developed early recurrence of the cyst and underwent reoperation. Three patients showed an excellent recovery after surgery. The child with arachnoiditis failed to recover neurologically.     

Terminal myelocystocele

Terminal myelocystocele is an unusual form of occult spinal dysraphism. It consists of a cystic dilatation of a low-lying terminal cord hemiated posteriorly through a skin covered lumbosacral spina bifida. This condition is often associated with OEIS complex i.e. opmphalocele, exstrophy of the bladder, imperforate anus and spinal abnormality. We studied 4 cases of terminal myelocystocele. They revealed no preoperative neurological deficit. None of these had associated OEIS complex. One of the cyst was unique due to presence of copious amount of pus in its cavity. All four cases underwent successful repair and surgery remained uneventful in all of them. No child showed neurological deterioration during the follow-up.     

Terminal myelocystocele

Terminal myelocystocele is an unusual form of occult spinal dysraphism. It consists of a cystic dilatation of a low-lying terminal cord herniated posteriorly through a skin covered lumbosacral spina bifida. This condition is often associated with OEIS complex i.e. opmphalocele, exstrophy of the bladder, imperforate anus and spinal abnormality. We studied 4 cases of terminal myelocystocele. They revealed no preoperative neurological deficit. None of these had associated OEIS complex. One of the cyst was unique due to presence of copious amount of pus in its cavity. All four cases underwent successful repair and surgery remained uneventful in all of them. No child showed neurological deterioration during the follow-up.     

Symptomatic spinal intradural arachnoid cysts in the pediatric age group: description of three new cases and review of the literature.

Spinal arachnoid cysts are a relatively uncommon lesion that may be either intra- or extradural, and intradural spinal arachnoid cysts are even less common. These cysts are usually asymptomatic but may produce symptoms by compressing the spinal cord or nerve roots suddenly or progressively. We present three cases in the pediatric age group with spinal intradural arachnoid cysts without a preceding history of trauma. Three patients with symptomatic intradural arachnoid cysts were investigated with conventional T1- and T2-weighted magnetic resonance imaging (MRI). The MRI scans demonstrated the intradural arachnoid cysts with slightly lower CSF signal intensity on the gradient echo images and slightly higher signal intensity on T1-weighted images. The first cyst was located at the level T12-L1 and compressed the conus medullaris, with neurogenic bladder and cauda equina syndrome for 2 months. The second was located at the level C5-T1 ventrally, with spastic gait and neurogenic bladder for 4 years. The other was located at T2-3 ventrally, with sudden onset of quadriplegia after jumping rope. The combined treatment of total resection and wide fenestration in our three patients produced an excellent return of neurologic function in each one, except for residual urinary disturbance in case 2. Intradural spinal arachnoid cysts appear to result from an alteration of the arachnoid trabeculae; some such cysts are ascribed anecdotally to previous trauma or arachnoiditis, whereas the majority are idiopathic and congenital. The majority of intradural spinal arachnoid cysts occur in the thoracic region and most are dorsal to the neural elements. Only 10 cases have been reported in which the intradural arachnoid cysts were located anterior to the cervical spinal cord, of which 8 were in the pediatric age group, like our case 2. Myelography, postcontrast CT myelography and MRI have been demonstrated as useful for the diagnosis of intradural arachnoid cysts. MRI is the imaging modality of choice, and the extent, size and nature of the lesion in our cases were well demonstrated by MRI. Surgical treatment is necessary if progressive neurological dysfunction appears in the course of spinal cord compression. Complete surgical excision of the cysts is the best choice of treatment, and wide fenestration and shunting of the cyst to the peritoneum, pleural cavity or right atrium were the modalities of choice. MRI offers a noninvasive and effective means to make the diagnosis of arachnoid cysts easier. Intradural arachnoid cysts may cause progressive myelopathy; however, the postoperative prognosis is good if the operation is performed prior to neurologic deficits.     

Intramedullary spinal neurenteric cyst with fluid-fluid level

Spinal neurenteric cysts are rare intradural extramedullary lesions of the spine, commonly located in the cervical and thoracic regions. The majority localize ventral to the spinal cord and are associated with other vertebral anomalies. Here, the authors report a rare case of a 3-year-old boy presenting with a 1-week history of meningismus followed by rapid-onset (over a few hours) paraplegia. Magnetic resonance imaging revealed an intramedullary cystic lesion with a fluid-fluid level in the cervicothoracic region of the spinal cord without associated bony or soft tissue abnormalities. To the best of the authors' knowledge, such clinical and radiological presentation of a spinal neurenteric cyst has never been reported. A brief review of the pertinent literature is presented, and the possible pathophysiology of such a presentation is also discussed.     

Spinal intramedullary ependymal cyst. Report of two cases and review of the literature

Two pediatric patients (4 and 5 years of age) with spinal intramedullary ependymal cysts located at the cervical and dorsal cord are reported here. One patient was admitted with subtle signs, while the other had disabling spastic quadriparesis. In both patients, MRI depicted a well-demarcated, localized, nonenhancing intramedullary lesion isointense with CSF on T(1)- and T(2)-weighted images. Total excision of the covering membrane of the cyst was not possible in either case. The child with spastic quadriparesis had a remarkable recovery, while the subtle signs of the other patient regained normalcy at the follow-up of 4 and 5 months, respectively.     

Arachnoid cysts in childhood with endocrinological outcomes

Arachnoid cysts are mainly manifested with the consequent neurological disorders. Even though these cysts may interfere in many systems due to their localizations, there is little information concerning their involvement in endocrinological disorders. We emphasize endocrinological functions together with cyst localizations and neurological disorders in childhood. A total of 50 patients diagnosed with arachnoid cysts were screened for cyst localizations, neurological symptoms and endocrinological outcomes evaluated by pubertal and growth status and hypothalamopituitary insufficiency. We investigated the interactions of these parameters. Arachnoid cysts were localized mostly in the middle fossa (54%) and posterior fossa (26%). Middle fossa cysts had a strong predilection for male gender (66.7% male) and left hemispheric dominancy (77%). We detected growth hormone deficiency in six patients, obesity in six patients and central precocious puberty in one patient; cysts were in the temporal area in three of the obese cases. All patients in our study had normal levels of cortisol, thyroid hormones and prolactin. In pediatric patients with arachnoid cysts, endocrinological follow-up is crucial as neurological outcomes and further evaluations are needed, mainly to confirm pubertal and growth status.     

Spinal cord injuries in older children: is there a role for high-dose methylprednisolone?

We present a retrospective case series of 15 children (aged 8-16 years) with blunt traumatic spinal cord injury who were treated with methylprednisolone as per the National Acute Spinal Cord Injury Study protocol. Of all patients, 12 (80%) were male. Causes were sports injuries (n = 9), motor vehicle crashes (n = 2), and falls (n = 4). Most injuries were nonskeletal (n = 14), and all patients had incomplete injury of the spinal cord. The most common location of tenderness was cervical (n = 7). Of the 15 patients, methylprednisolone was initiated within 3 hours in 13 patients and between 3 and 8 hours in 2 patients. All patients received the medication for 23 hours as per the National Acute Spinal Cord Injury Study protocol. Of the 15 patients, 13 recovered completely by 24 hours and were discharged with a diagnosis of spinal cord concussion. One patient had compression fracture of T5 and T3-T5 spinal contusion but no long-term neurological deficit. One patient was discharged with diagnosis of C1-C3 spinal cord contusion (by magnetic resonance imaging) and had partial recovery at 2 years after injury. All patients with a diagnosis of cord concussion had normal plain films of the spine and computed tomographic and magnetic resonance imaging findings. None of the patients had any associated major traumatic injuries to other organ systems. The high-dose steroid therapy did not result in any serious bacterial infections.     

Extreme lateral approach in a case of acute-onset quadriplegia due to high cervical neurenteric cyst

Spinal neurenteric cysts are very rare intradural developmental lesions, predominantly localized anterior to the cervical cord and arising from misplaced endodermal cells in the 3rd week of embryonic life. An acute onset of symptoms can occur due to hemorrhage, but has not as yet been reported in relation to infection in the cyst. We report an 18-year-old male who presented with a rapid onset of respiratory distress and quadriplegia over a period of 6 h requiring intubation and ventilatory support. There was no respiratory effort with a dense sensory level from the neck. MRI of the cervical spine revealed an intradural extramedullary ventrally located cystic lesion at the C(2-3) level. There was no evidence of systemic infection elsewhere in the body. The lesion was radically excised using an extreme lateral approach as it provided excellent visualization of the cyst, thus permitting safe dissection without retraction of the cord. The cyst was tense and the contents turbid, thick, creamy and mucinous without any evidence of fresh or altered blood. Histopathological examination confirmed the cyst to be a neurenteric cyst. The cystic fluid, which was sent for culture, grew coagulase-negative Staphylococcus aureus. This is the first documented case of rapid neurological deterioration due to infection in a neurenteric cyst. In spite of an early effective surgical management, outcome was poor due to the rapid and profound neurological deficit.     

Spinal intramedullary arachnoid cyst

Spinal arachnoid cysts are a relatively uncommon lesion. They may be either intra or extradural; intradural cysts being less common. Symptomatic spinal arachnoid cysts in the pediatric age group are rare. To the best of our knowledge only 2 cases of intramedullary arachnoid cysts have been reported to date. We report an unusual case of intramedullary cyst diagnosed in a 10-year-old female who presented with progressive quadriparesis. MRI scans revealed an intramedullary cystic lesion from C4 to T2. She underwent a C3 to T2 laminectomy with partial excision of the cyst. Histopathological findings confirmed the diagnosis of an arachnoid cyst. Postoperatively the patient showed dramatic recovery with marked improvement in neurological status. Arachnoid cysts should be considered in the differential diagnosis of progressive quadri / paraparesis in the pediatric age group as surgical decompression leads to marked improvement in the symptoms.     

儿童椎管内肠源性囊肿的诊治体会

目的 总结儿童椎管内肠源性囊肿的临床特点和治疗经验。方法 回顾性分析我院6例儿童椎管内肠源性囊肿的临床、影像学资料。结果 本组6例均有肢体活动障碍,4例伴有神经根性疼痛,5例感觉障碍。囊肿位于颈段4例,胸段2例。MRI检查表现为稍长T1、长T2均匀信号。5例作全切或大部切除术,1例行部分切除术。5例术后神经功能恢复良好。结论 儿童椎管内肠源性囊肿是少见的先天性疾患,多发生于颈段椎管硬膜下脊髓腹侧。MRI是有效的检查工具。早期诊断和显微手术是治疗的最佳方案。     

Traumatic spinal cord injury: unusual recovery in 3 children

We report on 3 children with traumatic spinal cord injury. All of them had normal x-rays of the vertebral column. There were no abnormalities of the spinal cord in myelography or magnetic resonance imaging. Although these situations are often associated with a poor neurological prognosis, we observed very unusual recoveries in these 3 cases.     

Atypical and equivocal sonographic features of the spinal cord in neonates

Thanks to the wider use of sonography to examine the spinal content in the neonate, normal anatomy and anomalies may be detected easily. Yet, unusual sonographic patterns are also observed. These must be differentiated from true pathologies. During a prospective study of 103 neurologically asymptomatic neonates, atypical sonographic patterns were found in 16 patients, corresponding to normal variants in 13. Nine of these 13 patients presented with a widening of the distal part of the central echo complex (one had a dilated ventriculus terminalis). Nerve roots of the cauda equina were disposed asymmetrically in three patients; the spinal cord movements were still present. In two of these babies, this distribution was associated with thin arachnoid pseudocysts. One patient presented with transitorily hyperechoic and narrow subdural spaces, probably related to neonatal dehydration. None of the 13 patients showing normal variants required any treatment. The other 3 patients (of 16) presented with equivocal entities of unknown evolution: sonographic tethered cord, fibrolipoma of the filum terminale and epidural varices. Sonography is highly accurate in evaluating the spinal cord content and aids differentiation of normal and normal variants from equivocal or pathological entities.     

INTRACARDIAC EPITHELIAL CYST ASSOCIATED WITH ESOPHAGEAL ATRESIA

We describe an intracardiac epithelial cyst in association with esophageal atresia. The case is unusual in that the cyst was symptomatic and ultimately fatal. In addition, there was no other cardiac anomaly, although a range of extrathoracic malformations was present. There are three types of intracardiac epithelial cysts: congenital polycystic tumor of the atrioventricular node, a cyst as part of a teratoma, and, as in this case, a gross cyst. All of them are very rare. The association of a cardiac cyst and esophageal atresia in our case supports the theory that intracardiac cysts are derived from misplaced foregut.     

Ultrasound-guided percutaneous drainage in the treatment of children with hepatic hydatid disease

The present study was designed to establish the safety and efficacy of ultrasound-guided percutaneous drainage in the treatment of hepatic hydatid cysts in children. A total of 14 cysts in eight patients were drained under aseptic conditions and continuous ultrasound guidance. An 18-G needle was used for puncture and silver nitrate solution as the scolecoidal agent. All procedures were successful. During follow-up, repeated ultrasound examinations revealed a progressive decrease in cyst size and an alteration in the cyst echopattern. None of the patients had a rise in hydatid antibody titer. There were no immediate or late complications. We conclude that the safety and efficacy of ultrasound-guided percutaneous drainage are satisfactory for the treatment of hepatic hydatid cysts in children. Received: 6 December 1995 Accepted: 3 October 1996     

Spinal cord compression caused by extradural arachnoid cysts. Clinical examples and review

Most spinal arachnoid cysts are asymptomatic and detected incidentally during magnetic resonance imaging or myelography. The etiology of intraspinal arachnoid cyst is not yet clear. We present two children with three spinal extradural arachnoid cysts and each cyst protruded from a separate dura defect. In both patients, plain radiographs demonstrated widening of the interpedicular distance, which suggested progressive widening of the spinal bony canal. Limited laminectomy was performed to remove the intraspinal cysts. Separate dura defects, the apparent predisposing factors, were also found and repaired. The patients completely recovered neurologically. Radical cyst removal and dura defect closure are the surgical intervention of choice in patients with symptomatic extradural arachnoid cyst.     

Ovarian cysts in precocious puberty

We describe a 7-year-old girl with precocious puberty in whom a single large cyst (5 cm) and several small cysts (8-10 mm) in the single remaining ovary were detected by the ultrasound examination. Endocrinological examinations confirmed the diagnosis of central precocious puberty. Pathologic findings after the removal of the cystic lesions revealed that the large cyst was derived from degenerated follicular cysts and the small cysts were identical to follicular cysts: all were considered to have been formed by gonadotropin stimulation. In general, surgical removal of an ovarian follicular cyst in central precocious puberty is inappropriate. However, in this unusual patient who had a degenerated large cyst, surgery seemed to be appropriate because of a previously removed teratoma in the contralateral ovary.     

Incidence, presentation, and outcome of spinal cord disease in children with systemic cancer

During a 40-month period, in 24 of 643 (4%) newly diagnosed patients with systemic cancer younger than 18 years of age (range: 3 months to 17 years) spinal cord disease developed. Patients with spinal cord disease included 21 children with metastatic spinal cord compression, two with treatment-related transverse myelopathies, and one with an anterior spinal artery stroke. Spinal cord disease occurred in 13 of 102 children (12%) with sarcomas, six of 82 (7%) with neuroblastomas, and four of 94 (4%) with lymphomas. Spinal cord compression occurred as the presenting sign of malignancy in six children (four with sarcomas and two with lymphomas). In the remaining 15 patients, cord compression occurred a median of 13 months after initial diagnosis, and in four patients it occurred at the time of first relapse. Symptoms of metastatic cord compression included back pain in 17 patients (80%), weakness in 14 (67%), sphincter dysfunction in 12 (57%), and sensory abnormalities in three (14%). Findings on plain radiographs of the spine were abnormal in only seven of 20 patients with cord compression, and myelography was needed to differentiate compression from other causes of spinal cord disease. Treatment included high-dose corticosteroids followed by operation (seven patients) or radiotherapy (14 patients). After treatment, nine of 15 nonambulatory patients became ambulatory, and five of 10 incontinent patients regained sphincter control. None of the patients with nonmetastatic spinal cord disease had a satisfactory outcome.(ABSTRACT TRUNCATED AT 250 WORDS)