Fourth ventriculostomy: an alternative treatment for hydrocephalus due to atresia of the Magendie and Luschka foramina

Atresia of the foramina of Magendie and Luschka is a rare cause of obstructive hydrocephalus. Although this condition has been classically treated by CSF shunting, recent treatments have also included endoscopic third ventriculostomy. In the present study, the authors present the case of a patient with hydrocephalus in whom an alternative method was used following a CSF shunt malfunction. A young female patient in whom a shunt was placed during the patient's 1st year of life was faring well until she was 8 years old. She was admitted to the emergency department 5 times with signs of CSF shunt malfunction. Each time, the CT scan showed a slight dilation of the lateral and third ventricles and a large increase in the size of the fourth ventricle. In comparison, ventricles were smaller in a previous imaging study obtained when the patient was asymptomatic. Magnetic resonance imaging showed the same slight dilation of all the ventricles and a significant increase in the fourth ventricle. There was no aqueductal stenosis. An important enlargement of both lateral recesses of the fourth ventricle suggested the possibility of an atresia of the foramina. The foramen of Monro and the width of the third ventricle would not allow the passage of an endoscope. The decision was made to open those foramina endoscopically through the fourth ventricle. After induction of general anesthesia, with the patient in the prone position, a bur hole was made in the left paramedian and suboccipital region. The endoscope was introduced underneath the cerebellar hemisphere. The authors were then able to distinguish the floor of the fourth ventricle and other anatomical landmarks. Navigation through the lateral recesses allowed them to see the fine membranes closing the foramina. These membranes were opened with a monopolar cautery as a blunt instrument. The orifice was then enlarged with a 3 Fr Fogarty catheter. The authors also opened a bulging thin membrane located at the foramen of Magendie. During the postoperative period, the authors observed a marked improvement in the state of the patient's alertness as well as a disappearance of her headaches and cessation of vomiting. In addition, the patient's gait ataxia improved slowly. Six-month postoperative MR imaging demonstrated an unequivocal reduction in the size of the fourth ventricle. The patient was still doing well 36 months after the surgery. Endoscopic fourth ventriculostomy, the opening of the 3 foramina of the fourth ventricle, may be an alternative treatment in cases in which these structures are congenitally closed.     

Ommaya储液囊植入治疗早产儿脑室内出血后脑积水的疗效及其影响因素分析

目的：观察Ommaya储液囊植入治疗早产儿脑室内出血后脑积水的临床疗效并探讨影响其疗效的相关因素。方法：采用双向性队列研究的方法分析20例因脑室内出血后脑积水而接受Ommaya储液囊植入术治疗的早产儿（胎龄＜32周,出生体重＜1500 g）的临床资料及随访资料,观察手术的治疗效果。根据治疗效果的不同分为治愈组和治疗失败组,采用单因素分析的方法探讨影响其疗效的相关因素。结果：(1)Ommaya储液囊植入术后 30 d,脑积水情况明显改善。(2)术后3个月,部分患儿临床疗效显著下降：术后3~6个月内7例转行脑室-腹腔分流术,4例因经济原因放弃治疗,1例因头皮下血肿伴皮肤坏死转行第三脑室底造瘘术;其余8例至12~18个月时脑室恢复正常大小。(3)并发症情况：术后继发性脑室内出血8例,颅内感染2例,头皮下血肿伴皮肤坏死1例。(4)单因素分析显示胎龄、出生体重、Ommaya储液囊植入时脑积水时间在治愈组和治疗失败组比较差异有统计学意义（P＜0.05）。结论：Ommaya储液囊植入术治疗早产儿颅内出血后脑积水短期内疗效显著,但随时间延长部分患儿治疗效果降低。小胎龄、低出生体重、脑积水时间长可能是影响疗效的主要因素。     

Migration of a ventricular tapping reservoir into the third ventricle

The authors present a case of migration of a ventricular tapping system into the third ventricle of a 6-year-old boy who is was born prematurely. As an infant, the patient was treated with serial lumbar punctures for hydrocephalus associated with intraventricular hemorrhage. The patient progressed to requiring a ventricular tapping reservoir and eventually a ventriculoperitoneal shunt. The patient presented for suspected shunt malfunction 4 years after placement of his ventriculoperitoneal shunt, and the migrated ventricular tapping system was discovered. The authors discuss several management strategies that could be used to prevent this complication.     

Specific enlargement of the fourth ventricle after ventriculo-peritoneal shunt for post-haemorrhagic hydrocephalus.

Disproportionate enlargement or isolation of the fourth ventricle are rare complications of ventriculo-peritoneal shunt for post-haemorrhagic hydrocephalus. Obvious features such as ataxia may indicate raised intracranial pressure in the posterior fossa. The child may, however, present with less specific signs of vomiting, anorexia, and lethargy and these symptoms may be misinterpreted as secondary to dilation of the lateral ventricular system due to malfunction of the ventriculo-peritoneal shunt. Two children with disproportionate enlargement of the fourth ventricle and two children with isolation of the fourth ventricle are described to illustrate the wide variations in clinical presentation. These rare complications can be diagnosed by real time ultrasound examination of the brain or computed tomography of the head. They should be considered in the differential diagnosis of a child who deteriorates after lateral ventriculo-peritoneal shunting for post-haemorrhagic hydrocephalus, and dealt with specifically to avoid the risk of upward herniation of the enlarged fourth ventricle.     

Acute obstructive hydrocephalus due to a large posterior third ventricle choroid plexus cyst

We present the case of a child in whom acute hydrocephalus developed secondary to obstruction of the foramen of Monro by a choroid plexus cyst. The patient was seen in the emergency department with fevers, acute onset of headaches, and lethargy. Computed tomography demonstrated dilated lateral and third ventricles with a relatively normal-sized fourth ventricle. An external ventricular drain was placed. Despite decompression of the lateral ventricles, follow-up magnetic resonance imaging demonstrated a dilated third ventricle with a possible thin-walled mass extending from the foramen of Monro into the posterior portion of the third ventricle. The patient subsequently underwent endoscopic fenestration of the cyst with endoscopic third ventriculostomy. Although two other cases of symptomatic choroid plexus cysts of the third ventricle have been previously reported in children, our paper highlights the possibility of endoscopic cyst fenestration together with a third ventriculostomy as a treatment option in cases where the cyst extends into the posterior third ventricle. Despite adequate decompression, we were concerned that due to CSF pulsations the remnant cyst wall could result in acute aqueduct obstruction and subsequent hydrocephalus.     

Endoscopic third ventriculostomy through the interfornicial space

Endoscopic third ventriculostomy (ETV) is the most common cranial endoscopic surgery being done worldwide for a variety of conditions resulting in hydrocephalus. We report ETV in a case of congenital hydrocephalus with deficient third ventricular roof and wide interfornicial space. A 6-month-old male child presented with gradual progressive head enlargement and signs of raised intracranial pressure. Computed tomography of the head showed aqueductal stenosis with enlargement of third and lateral ventricles. The patient was operated under general anesthesia with endotracheal intubation. A right coronal burr hole and free-hand entry were made into the right lateral ventricle. The anterior part of the septum and the third ventricular roof were deficient with a narrow Monro's foramen and the third ventricular floor was visible through the space between both fornices. ETV was done in standard fashion. Postoperatively the patient showed uneventful recovery clinicoradiologically. We review the literature and discuss the findings and technique of ETV in this rare entity.     

Value of the new Doppler-derived myocardial performance index for the evaluation of right and left ventricular function following repair of tetralogy of fallot

The systolic and diastolic function in both ventricles may be altered even after successful corrective surgery of tetralogy of Fallot. The aim of this study was to assess the combined diastolic and systolic function of both ventricles using the Doppler-derived myocardial performance index (MPI) in patients with operated tetralogy of Fallot (TOF). We performed a prospective analysis of 51 patients following corrective surgery of TOF: 21 had a subannular patch, 20 had a homograft implantation at initial operation, and 10 were reoperated with secondary homograft implantation. Patients were examined with Doppler echocardiography, and the MPI, which incorporates ejection and isovolumetric relaxation and contraction times and is an index of global ventricular function, was calculated 10.2 +/- 8.0 (0.89-36) years after surgery. In 86.4% of the examined patients the right ventricular isovolumetric relaxation time was shortened compared to the normal published range or even did not exist (negative value) (p <0.01). The right ventricular MPI was paradoxically below the normal published range in 76.5% of the examined patients. The left ventricle global function was impaired in 23.5% of the examined patients, mainly due to altered systolic function with a prolonged left ventricular isovolumetric contraction time. The z score of the comparison between patients' left ventricular isovolumetric contraction time and the normal published values was 3.03. Patients with severe pulmonary regurgitation also had a prolongation of the isovolumetric relaxation time compared to patients with mild to moderate pulmonary regurgitation. The noncompliant right ventricle may shorten the right ventricular isovolumetric relaxation time, resulting in a paradoxically low right MPI. This may reduce the sensitivity of the index in recognizing patients with right ventricular dysfunction following corrective surgery of TOF. Additional diastolic impairment occurs in patients with right ventricular volume overload.     

Clinical Significance of Thrombosis in an Intracardiac Blind Pouch After a Fontan Operation

The univentricular heart after the Fontan operation may have a blind pouch formed by the pulmonary stump or rudimentary ventricle according to the anatomy before surgery. Thrombosis in an intracardiac blind pouch of patients with a univentricular heart is a hazardous complication. Because only a few reports have described this complication, the authors evaluated the clinical significance of thrombosis in an intracardiac blind pouch of a univentricular heart. They performed a retrospective review of medical records from August 1986 to December 2007. Four patients were confirmed as having thrombosis in a pulmonary artery stump and one patient as having thrombosis in a rudimentary ventricle shown by cardiac computed tomography (CT). This represents 1.85% (5/271) of patients with ongoing regular follow-up evaluation after the Fontan operation. The median age at diagnosis was 14.2 years. Two of the five patients were taking aspirin and one patient was taking warfarin when they were identified for the development of thrombosis. None of the patients demonstrated thrombosis in the Fontan tract or venous side of the circulation. Brain magnetic resonance imaging (MRI) showed that three patients had cerebral infarction and one patient had suggestive old ischemia. Three patients with thrombus in the pulmonary stump underwent pulmonary artery stump thrombectomy and pulmonary valve obliteration. One patient with thrombus in the rudimentary ventricle underwent ventricular septal defect (VSD) closure with thrombectomy. Thrombus in a blind pouch could cause systemic thromboembolism despite little blood communication. Therefore, surgical modification of the pulmonary stump and VSD closure of the rudimentary ventricle are required to reduce the risk of later thrombus formation. Clinicians should not overlook the possibility of thrombus in a ligated pulmonary artery stump or a rudimentary ventricle after the Fontan operation, which may increase the risk of embolic stroke for patients with single-ventricle physiology.     

Bile duct atresia following extended right hepatectomy because of a tumor

A 19 month old male infant with a mesenchymal hamartoma of the liver underwent an extended right hepatectomy. Serum bilirubin gradually rose until 3 months after the surgery, and obstructive jaundice and acholic stools were manifested at 6 months. Percutaneous transhepatic cholangiodrainage was performed. Cholangiography showed dilation of the intrahepatic bile duct of the residual lateral segment and complete obstruction of the extrahepatic bile duct. A second operation for reconstruction of the biliary tract was performed 10 months after the first surgery. No aspect of an extrahepatic biliary tract was found. Histological inspection of a surgical specimen of remnant tissue revealed only cicatricial connective tissue without any biliary structures. The clinical course has been uneventful for 18 months since the second surgery. The cause of bile duct atresia in this case is strongly suggested to be an ischemic change due to devascularization of the extrahepatic biliary tract following hepatic resection because of a tumor. To prevent this kind of complication, hepaticoenterostomy should be performed close to the cut surface of the liver.     

经尿道电切治疗有症状的前列腺囊

目的介绍一种治疗前列腺囊的微创方法—尿道镜下电切扩大前列腺囊开口。方法自2003年2月至2005年10月我们共收治9例有并发症的前列腺囊患儿,平均年龄7.4岁(4～20岁)。术前6例尿道造影证实。9例患儿均行尿道镜检查 电切术。前列腺囊开口明显者,直接将电刀插入开口,切除开口周围组织,扩大开口;开口不明显者,先用环形电灼头在精阜正中电灼胀大的精阜,显露出前列腺囊开口后,再电灼扩大开口。手术完成后,置双腔导尿管4～7d。结果平均手术时间35min(20～45min);平均住院时间5d(4～7d)。平均随访2年2个月(6个月～3年1个月)。7例患儿经1次电切,2例患儿经2次电切,临床症状消失,排尿恢复正常。结论对表现有临床症状的病例主张积极的手术干预,尿道镜下电灼去顶、扩大囊肿开口,是一种简单、安全有效的治疗方法。     

Novel Approach to Transvenous Pacemaker Implantation in a Post-Fontan Adolescent

Postoperative bradycardia is not uncommon following the Fontan procedure in patients with a functional single ventricle. The surgical connections created with various Fontan modifications may complicate access to the atria for transvenous implantation of a permanent pacemaker. We describe approaches to overcoming problems with atrial access in an adolescent with complex congenital heart disease who required permanent transvenous atrial pacing for tachycardia-bradycardia after Fontan surgery.     

Endoscopic transtentorial ventriculocystostomy and cystoventriculoperitoneal shunt in a neonate with Dandy-Walker malformation and associated aqueductal obstruction

OBJECTIVE: Shunting of the lateral ventricle and the posterior fossa cyst is the advocated surgical therapy for children with Dandy-Walker malformation (DWM) and associated aqueductal obstruction. The high rate of complications of combined shunting stimulated the authors to search for an alternative surgical solution. CLINICAL PRESENTATION/INTERVENTION: After transtentorial endoscopic ventriculocystostomy, a cystoventricular catheter, connected to a peritoneal shunt, was placed in a neonate with DWM and associated aqueductal obstruction. Immediately prior to ventriculocystostomy, the presence of a blocked third ventricular outflow was reconfirmed by contrast medium injection. Neuronavigation was required to define the surgical path from the lateral ventricle through the tentorium and the overlying small rim of brain parenchyma into the posterior fossa cyst. The postoperative clinical course was uneventful with radiologically proven reduction of the size of the ventricular system and the cyst. CONCLUSION: Cystoventriculoperitoneal shunt placement after transtentorial endoscopic ventriculostomy is a surgical alternative in very young children with DWM and associated aqueductal obstruction.     

Primary idiopathic chylopericardium

Primary idiopathic chylopericardium is rare and represents a fraction of all pericardial effusions in the pediatric population. Most cases of chylopericardium occur as a complication of cardiac surgery or after trauma. They may also be caused by tuberculosis, neoplasm, or congenital lymphangiomatosis. Primary idiopathic chylopericardium is a rare disease with unknown etiology. In assessing pediatric patients with an enlarged cardiac silhouette one should be aware of this entity and its presentation. We report two cases of primary idiopathic chylopericardium, one of which at 6 weeks of age is the youngest case to our knowledge so far reported.     

Pulmonary Atresia with Intact Ventricular Septum Causing Severe Left Ventricular Outflow Tract Obstruction

We describe an infant with pulmonary atresia with intact ventricular septum (PAIVS) and severe left ventricular outflow tract (LVOT) obstruction secondary to a suprasystemic right ventricle causing leftward displacement of the interventricular septum. Imaging demonstrated an aneurysmal dilation at the base of the proximal main pulmonary artery (MPA) with no forward flow from the right ventricle. During transannular patch and central shunt placement, the communication between the pulmonary artery and the right ventricle was enlarged to ensure adequate decompression. We report this successful palliation and resulting complete elimination of the LVOT obstruction in a very unique presentation of PAIVS in a newborn.     

Percutaneous closure of pseudoaneurysm in the left ventricle in a young child

Pseudoaneurysm in the left ventricular outflow tract is a rare complication occurring after cardiac surgery. We report on the successful percutaneous device closure of a pseudoaneurysm in the left ventricular outflow tract, which developed in a 23‐month‐old girl after resection of a subaortic ridge. The pseudoaneurysm was closed using an Amplatzer Vascular Plug II.     

The Goldston Syndrome: Report of a Case

We present a case of the Goldston syndrome—that is, cystic renal dysplasia and the Dandy- Walker malformation. The condition was diagnosed by ultrasound in a 635 g fetus in the seventeenth week of gestation. Ultrasound studies showed the fetal head to be somewhat enlarged with slight dilatation of the lateral ventricles and marked dilatation of the fourth ventricle. The kidneys were symmetrically enlarged and multicystic. Autopsy revealed evidence of the oligohydramnios syndrome. The kidneys were typical of the Goldston syndrome as were the microscopic lesions in the liver. To our knowledge this is the first reported case in which this diagnosis was made during intrauterine life.     

Intracerebral hemorrhage at 2 different times in neonatal alloimmune thrombocytopenia

We demonstrate the sonographic and computer-tomographic findings in a newborn with neonatal alloimmune thrombocytopenia (NAITP). Initial examination on the third day of life showed an extensive old and already partially resolved haematoma as well as a new perinatal haematoma in the left cerebral white matter. Follow-up investigations revealed enlargement of the cystic defect, midline-shift to the right and progressive dilatation of the right lateral ventricle caused by compression of the Foramina Monroi. Peritoneal drainage of the cyst resulted in prompt diminution of the enlarged right lateral ventricle. The case report emphasises that in patients with NAITP intracranial haemorrhage may occur in utero and during the perinatal period as well. In pregnant women suspected to produce antibodies against PLA 1 positive thrombocytes, we recommend regular ultrasonic cranial examinations of the fetus. Delivery should be as careful as possible.     

The Use of Valved Conduits in Pediatric Cardiac Surgery

Extracardiac valved conduits were introduced in 1966. Currently, both aortic and pulmonary homografts, preserved in antibiotic/nutrient solution or cryopreserved, are used. Conduits are implanted between the right ventricle and pulmonary artery, left ventricle and pulmonary artery, right atrium and right ventricle, and left atrium to left ventricle. Several factors can influence longevity of valved conduits: young age at implantation, small size of homograft, and immunological response. In the recent study from our department, we evaluated 405 homografts implanted between 1971 and 1993 in patients who survived 90 days after surgery. Freedom from conduit replacement at 5 and 15 years was 84% and 31% (95% confidence limits: 80–88% and 19–43%), respectively. In multivariate analysis, there were only two predictors of conduit longevity: (1) conduits used at reoperation lasted less well than those used at original operation; and (2) conduits used earlier in the series lasted longer. Conduits may have to be replaced because of obstruction, conduit valve regurgitation, aneurysm/pseudoaneurysm, and endocarditis. Obstructed conduits are either replaced or outflow tract patch is used after removal of the conduit with or without pulmonary valve implantation. Current mortality of conduit insertion is low (5–6%). The risk of conduit replacement has also decreased in recent years to 2–3.5%     

The Goldston syndrome: report of a case

We present a case of the Goldston syndrome--that is, cystic renal dysplasia and the Dandy-Walker malformation. The condition was diagnosed by ultrasound in a 635 g fetus in the seventeenth week of gestation. Ultrasound studies showed the fetal head to be somewhat enlarged with slight dilatation of the lateral ventricles and marked dilatation of the fourth ventricle. The kidneys were symmetrically enlarged and multicystic. Autopsy revealed evidence of the oligohydramnios syndrome. The kidneys were typical of the Goldston syndrome as were the microscopic lesions in the liver. To our knowledge this is the first reported case in which this diagnosis was made during intrauterine life.     

Intracerebral sewing needle

A 9-year-old male patient complaining of seizure attack was admitted to the neurosurgery department. Radiologic investigations revealed a 5-cm-long metallic sewing needle extending from the right frontal cortex to the right lateral ventricle. Burr hole surgery was performed and the needle was grasped with biopsy forceps and removed with endoscopic guidance. The patient recovered without any complications.