Schwannomes vestibulaires kystiques géants : intérêt du drainage kystopéritonéal

Introduction

Surgical removal of giant cystic vestibular schwannomas is difficult because of adherences between the cyst membrane, brainstem, and the VII-VIII nerve complex. The recurrence of the cyst is frequent and requires reoperation. The aim of this study was to analyze the role of the palliative cystoperitoneal shunt in giant cystic vestibular schwannomas.

Materials and methods

Eighty-eight patients with a diagnosis of stage III or IV vestibular schwannoma were managed surgically from January 2000 to December 2005 in our department. Six schwannomas were deemed to be cystic according to the following criteria: a voluminous cystic component with mass effect causing symptoms as well as radiological and intraoperative identification of cystic elements.

Results

Complete tumor removal was achieved in two patients. After a follow-up of 5 and 7 years, these patients were asymptomatic. In four cases, we performed cyst drainage. For three patients, we implanted a permanent cystoperitoneal shunt. These patients were asymptomatic with a mean follow-up of 19.7 months.

Conclusions

The cystoperitoneal shunt with no valve is a valid palliative surgical solution to remove brain stem compression. Neuronavigation allows proper positioning of the drain and secures the procedure.     

Appendicitis in the child with a ventriculo-peritoneal shunt: a 30-year review

Purpose

Each year, about 270 children are treated at our hospital for appendicitis, and there are 200 ventriculo-peritoneal (VP) shunt procedures. The incidence of primary peritonitis after a VP shunt is 8% to 12%. The purpose of this article is to try and differentiate these 2 entities.

Methods

From 1973 to 2003 inclusive, appendicitis was diagnosed in 8 children with a VP shunt at our hospital; there were 7 boys and 1 girl with 5 acute appendicitis and 3 ruptured appendices. The first case was diagnosed on purely clinical grounds, whereas the last 7 were confirmed by ultrasonography and/or computed tomography.

Results

All 8 had appendectomy and the shunt was exteriorized in the 3 children with a ruptured appendix. There were no postoperative problems, and the 8 children remained well.

Conclusion

Acute appendicitis can and does rarely occur in children with VP shunts; however, in such situations, the correct diagnosis can be confirmed by imaging. The shunt must be temporarily exteriorized if the appendix is ruptured.     

Persistent ascites can be effectively treated by peritoneovenous shunts

Aim

The aim the study was to review our experience and determine the effectiveness of peritoneovenous shunts in the management of persistent ascites.

Methods

A retrospective review of all patients who had a peritoneovenous shunt inserted for persistent ascites in our institution over 32 years (1977-2010) was performed.

Results

Thirty-one peritoneovenous shunts were inserted into 25 patients. Records from 22 were available. Median age was 34 months (range, 1 month-12 years), with median duration of ascites of 1.75 months (range, 3 weeks-2.5 years). Sixteen had previous abdominal surgery, whereas one had previous thoracic surgery. Other underlying pathologies included hepatitis (n = 2), lymphohistiocytosis, lymphatic hypoplasia, and carcinomatosis. One was regarded as idiopathic. Previous management consisted of paracentesis (n = 15), dietary modification (n = 11), diuretics (n = 9), and total parenteral nutrition (TPN) (n = 4). One underwent fetal drainage of ascites.No intraoperative complications occurred. Eight (36%) had postoperative complications, including shunt occlusion (n = 2), pulmonary edema (n = 2), infection (n = 2), and wound leakage (n = 1). One developed a varix following shunt removal. The ascites resolved after shunting in 20 (91%) of the 22 children. Four died from their underlying pathology. In children with malignancy, the shunt allowed prompt resumption in treatment schedule.

Conclusion

This represents the largest series of children receiving peritoneovenous shunt for persistent ascites. It is a safe and effective treatment which should be considered early.     

Influence of the type of operations on the development of latex sensitization in children with myelomeningocele

Background

A genetic tendency to develop latex IgE responses in children with spina bifida has been suggested, but their degree of exposure to latex as well as the route and chronology for sensitization can be different from those of other children with multiple surgeries. The aim was to study the influence of the type of operation on the development of latex sensitization in children with myelomeningocele.

Methods

In 90 children with myelomeningocele operated with latex, data were collected about age, sex, family, and personal history of allergy, serum total IgE, presence of a ventricular-peritoneal (v-p) shunt, and number and duration of operations, classified as urological, orthopedic, on the v-p shunt, other neurosurgical, and others. A multivariate logistic regression analysis was performed, using the presence of latex sensitization as dependent variable.

Results

Serum total IgE, the number of urological and of orthopedic operations were synergistic variables to predict latex sensitization, together with the presence of a v-p shunt, but not the number of operations performed on this device. This seems to play an adjuvant role in the process of sensitization.

Conclusions

Not only the number, but also the type of operations, namely, of urological and orthopedic nature, is important in the development of latex sensitization in children with myelomeningocele.     

Late-onset inferior vena cava obstruction in a shunted patient--a unique cause of rebleeding in children with portal hypertension

Background

Rebleeding in the presence of an adequate patent portosystemic shunt in a patient with portal hypertension (PHT) is uncommon. Inferior vena cava (IVC) obstruction as the cause of rebleeding in this situation has not been reported in the literature.

Methods

Records from a pediatric tertiary care center were reviewed over a 15-year period. Portosystemic shunt procedures for bleeding esophageal varices were done in 39 children. Patients who, after a shunt surgery for PHT, developed a rebleed because of IVC obstruction in the presence of a patent shunt were identified.

Results and Conclusions

Late IVC obstruction in the presence of a patent shunt was identified in 2 patients. The etiology included adhesions, caudate lobe hypertrophy, and macronodular cirrhosis. Diagnosis was by angiography, and treatment included angioplasty and liver transplantation. Awareness of this condition helps direct treatment appropriately in the clinical scenario of a rebleed in a shunted patient with PHT.     

Portal vascular anomalies in Down syndrome: spectrum of clinical presentation and management approach

Purpose

The occurrence of portal vascular anomalies in Down syndrome has been sporadically reported in the literature. These rare disorders have a wide spectrum of anatomical and clinical presentations. The aim of this communication was to describe the clinical course, imaging features, and management approaches in patients with this association.

Methods

We conducted a comprehensive search of the databases of the Vascular Anomalies Center and the Department of Radiology at Children's Hospital Boston for patients with Down syndrome and portal vascular anomalies. Medical records and imaging studies of varying modalities were reviewed.

Results

Three children with Down syndrome and portal anomalies (portosystemic shunt, simple arterioportal shunt, complex arterioportal shunt) were managed at our institution. The portosystemic shunt was clinically insignificant and resolved without any intervention. The simple arterioportal shunt was successfully treated with embolization. The complex arterioportal shunt was associated with major congenital cardiac defects and the child ultimately expired despite a decrease in the arterioportal shunting after embolization.

Conclusions

Three is a wide spectrum of clinical and anatomical features of portal vascular shunts in Down syndrome. The management approach should be tailored based on the severity of symptoms. Percutaneous embolization can offer a safe, effective, and minimally invasive alternative to the surgical approach in selective cases.     

Measurement of the ductal L-R shunt during extracorporeal membrane oxygenation in the lamb

Objective

In neonates, initially a ductal shunt is often observed during veno-arterial extracorporeal membrane oxygenation (ECMO). Depending on the degree of pulmonary hypertension in these patients, the ductal shunt will be right to left (R-L), left to right (L-R), or bidirectional. A ductal L-R shunt will possibly lead to pulmonary hyperperfusion and interact with ECMO weaning. The aim of this study was to give more insight in this ductal L-R shunt during ECMO by quantification of this shunt in relation to cardiac output and ECMO flow.

Methods

In 7 lambs, closure of the duct was prevented by infiltration of the ductal wall with 10% formaline. This patent duct could be closed using a vesselloop around the duct. Ultrasound flowprobes were installed around the pulmonary artery, ascending aorta, and around the ECMO circulation tube. Right and left ventricular output and ECMO flow were measured. Ductus flow was defined as ductal left to right shunt (Qduct L-R) = flow in ascending aorta (Qao) − flow in central pulmonary artery (Qpa) and Qduct R-L = Qpa = Qao.

Results

In 6 of 7 lambs a ductal L-R shunt was observed with a mean shunt of 44% (range, 11 to 79) of left ventricular output (Qduct L-R/Qao). Comparison with ECMO flow (Qduct L-R/Qecmo) showed a mean shunt of 76% (range 15 to 230). When compared with the total systemic circulating volume (Qpa + flow in the ECMO circuit [Qecmo]), the mean ductal L-R shunt showed a percentage of 51% (range, 7% to 142%).

Conclusions

During ECMO, mostly a ductal L-R shunt is observed in this lamb model. This ductal shunt is hemodynamically important. The percentages of this shunt in comparison with left ventricular output, and total circulating volume will support the idea that a ductal L-R shunt during ECMO could be another deteriorating factor in the often critical circulation of the neonate on veno-arterial ECMO.     

Infectious risk to ventriculo-peritoneal shunts from gastrointestinal surgery in the pediatric population

Purpose

The infectious implication of abdominal surgeries on ventriculo-peritoneal (VP) shunts, including simultaneous shunt placement and management of shunt infections, has been ill defined in children.

Methods

We conducted a 9-year retrospective review of pediatric patients with VP shunts who underwent abdominal surgeries.

Results

Forty-two patients fit criteria. The median age at shunt placement was 1.75 years, and the median time between shunt placement and abdominal surgery was 24 days. The most common procedures included gastrostomy (17), fundoplication (15), and appendectomy (3). Seven patients had simultaneous abdominal surgery and shunt placement. All patients received preoperative antibiotics. Two children developed shunt infections, both occurred after appendectomy. Both were treated with antibiotics, with one requiring shunt removal. Median length of stay was 24 days but 28 days for those with infections. Thirty-eight patients were discharged home, 3 to chronic care facilities, and 1 died.

Conclusions

Infections did not occur in children with VP shunts undergoing elective abdominal procedures or procedures simultaneously with shunt insertion. Infections were seen only with emergent appendectomies, suggesting that performing gastrointestinal procedures at the time of VP shunt insertion is safe. Children with VP shunts undergoing emergent surgery for peritoneal infection warrant close observation for shunt infection.     

Prenatal and postnatal ciliated hepatic foregut cysts in infants

Purpose

Ciliated Hepatic Foregut Cyst (CHFC) is a rare congenital lesion arising from the embryonic foregut. Since squamous cell carcinomas arising from CHFC have been reported in adults, complete resection should be considered. We report our experience with CHFC.

Methods

We reviewed the charts of 2 patients who had surgery after prenatal detection of a CHFC and 2 patients with postnatal diagnosis.

Results

Two patients had antenatally detected liver cyst. Postnatal ultrasonography showed a cyst in segment IV, with wall calcifications and sediments. Bile ducts were encased in the wall of the cyst. They underwent central hepatectomy with double biliary diversion and uneventful post operative course. The two other patients underwent non anatomical resection of a cyst on the left lobe and in segment IV, found prior or during liver surgery.Pathology examination showed cysts filled with mucinous fluid, surrounded by an epithelium composed of ciliated cells. One case had a squamous metaplasia.

Conclusion

In infants, CHFC are found antenatally or incidentally. A solitary uni or mutilocular cyst with wall calcifications, sediments, located in the central liver segments should raise the diagnosis. Resection of large cysts in the central segments of the liver is challenging and biliary diversion should be considered.     

Is congenital cystic adenomatoid malformation a premalignant lesion for pleuropulmonary blastoma?

Background

The relationship between congenital cystic adenomatoid malformation (CCAM) and pleuropulmonary blastoma (PPB), whether causal, correlational, or coincidental, remains controversial. There is a lack of consensus as to the optimal treatment of patients with asymptomatic CCAM.

Method

We reviewed all cases of CCAM and PPB seen at our institution from 1999 to 2008. Institutional Research Ethics Board approval was obtained. The incidence of CCAM and PPB, respectively, was calculated based on birth numbers during the study period.

Results

Seventy-four CCAMs were resected over the study period in 129 children diagnosed with CCAM. Five PPBs were diagnosed during the study period. Three of the 5 PPB cases were initially diagnosed as CCAMs. These PPBs were not clinically or radiologically distinguishable from CCAMs. In our referral area, the incidence of CCAM was 1 in 12,000; and the incidence of PPB was 1 in 250,000 live births. The mortality rate for PPB in this cohort was 20%.

Conclusion

Asymptomatic cystic lung malformations represent a therapeutic dilemma. In this cohort, the incidence of PPB among apparently benign lung lesions was 4%. No clinical or radiological markers differentiated benign CCAMs from PPBs. Our experience provides further justification for resection of all CCAMs. This should be discussed with parents until CCAMs and PPBs can be clearly distinguished preoperatively.     

Can a pressure-limited vesico-amniotic shunt tube preserve normal bladder function?

Introduction

We have previously shown that a vesico-amniotic shunt (V-A shunt) produces fibrotic bladders with poor compliance in normal fetal lambs. We hypothesized that using a ventriculo-peritoneal shunt (V-P shunt) as a V-A shunt in normal bladders may preserve the filling/emptying cycle and normal bladder development.

Materials and methods

The V-A shunting in normal fetal lambs was performed at 74 days of gestation using a V-P shunt (group A) and a free-draining shunt tube (group B). Sham-operated lambs were used as controls (group C). They were all delivered at term (145 days), and the pressure-volume curve, bladder volume, and histologic features of the bladder wall were compared.

Result

The mean bladder volume in group B (n = 5), 5 ± 2.4 mL, was significantly smaller (P < .01) than that in group A (n = 6), 53 ± 14 mL, and group C (n = 10), 57.3 ± 12 mL. The bladder wall thickness in group A was 338 + 94.2 μm; group B, 741 ± 128 μm; and group C, 374 ± 120 μm. Group B bladders had very poor compliance with thick bladder wall (P < .01). Histologically, group B bladders showed prominent submucosal fibrotic change, but group A bladders were similar to controls.

Conclusion

This study shows that a pressure-limited shunt tube for V-A shunting preserves the normal fetal bladder development.     

Clinical study on intracranial arachnoid cyst: with reference to the middle cranial fossa

Thirty-two cases of congenital arachnoid cyst (AC), 26 cases of supratentorial, and 6 cases of infratentorial cyst are reported. They were encountered over a period of 9 years. The 26 supratentorial cysts consisted of 21 cases located in the middle fossa, 2 in the frontal, and 3 in the suprasellar. Patients' ages ranged from 7 months to 67 years (mean: 19.3 years), with a male to female ratio of 23:9. The most common symptoms were headache, epileptic seizure, and cranial enlargement. Based on observations between neuroradiological examinations including analysis of the cyst content, and operation, typical AC might be defined as "duplicated arachnoid and its splitting", and it may be said that "there is continuous existence of cerebral structure such as cortex and white matter lying adjacent to AC on MRI". Out of 32 cases, 15 received surgical treatment because of mass effect of AC itself, abnormal dynamics of the AC fluid, and/or increasing sign of ICP. 13 cases had resection of AC outer membrane with craniotomy, and the remaining two had cystoperitoneal shunt. One of the shunting cases complicated subdural hematoma. Surgical treatment was discussed and radical craniotomy rather than a shunt procedure was preferred. Prognosis of all cases was favorable and uneventful.     

The prevalence of shunt-treated hydrocephalus: a mathematical model

Background

The aim of the study was to create a mathematical model that reproduces the prevalence of shunt-treated hydrocephalus in the United States over the years and predicts trends in the near future.

Methods

A structured search was performed of the English language literature for case series reporting rates of shunt insertion and revision, shunt removal, and patient mortality. A meta-analytic model was constructed to pool data from multiple studies and to calculate these rates at various time intervals. Separate Markov models were used to predict numbers of shunts at any one time for children (<17 years old) and adults. The models analyze the number of shunts inserted every year since 1955 and follow the likely fates of those patients as time passes.

Results

Prevalence rates predicted by the model agree closely with those reported in the literature. However, the model's structure creates considerable variability around point estimates.

Conclusions

The model gives a comprehensive view of the prevalence of shunt-treated hydrocephalus in both children and adults from 1955 to the present. This model may prove useful in predicting resource use and needs for patients with hydrocephalus.     

Effectiveness of Rex shunt in the treatment of portal hypertension

Background

Children with portal venous thrombosis often have severe symptoms secondary to portal hypertension including recurrent upper gastrointestinal (UGI) bleeds and hypersplenism. We report results of the use of the mesenterico-left portal bypass (Rex shunt) in 5 consecutive patients.

Methods

A retrospective chart review of all patients with portal venous thrombosis who underwent Rex shunt procedure was performed. Children were evaluated preoperatively with magnetic resonance angiogram, Doppler ultrasound, and mesenteric angiogram. Postoperative ultrasounds were performed in follow-up.

Results

All patients had evidence of portal hypertension and hypersplenism. The average age of the patients was 13.2 ± 4.9 (7-19) years. The patient had an average of 2.6 ± 1.7 UGI bleeds requiring banding and 3.4 ± 4.2 U of blood transfused before undergoing the shunt. The mean operative time was 383 ± 46 minutes, and length of stay was 10.4 ± 7.1 days. In follow-up of 18.8 ± 5.2 months (11-24 months), all but 1 patient had a patent shunt. One narrowed shunt was successfully dilated by percutaneous angioplasty. Thrombocytopenia improved significantly in patients with functioning shunts but did not correlate with a significant decrease in splenic size.

Conclusions

The Rex shunt reestablishes normal hepatopedal portal flow, and in patients with functioning shunts, no recurrent UGI bleeds or transfusions were required or evidence of encephalopathy were noted.