A Surgical Approach to the Treatment of Fetal Hydrocephalus

Clewell, William H.; Johnson, Michael L.; Meier, Paul; Newkirk, J. B.; Zide, Sheldon L.; Hendee, Robert W.; Bowes, Watson A.; Hecht, Frederick; O’Keeffe, Daniel; Henry, George P.; Shikes, Robert H.

doi:10.1056/nejm198206033062202

Cited by 195 publications

(57 citation statements)

References 5 publications

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“…Options at the time of an in utero diagnosis of severe hydrocephalus are currently limited to termination of the pregnancy or expectant management. Fetal shunt placement has been attempted, but technical issues with the shunt and mixed patient outcomes resulted in a moratorium on fetal percutaneous shunting in the United States since 1985 (4,5).…”

mentioning

confidence: 99%

Repeated autologous umbilical cord blood infusions are feasible and had no acute safety issues in young babies with congenital hydrocephalus

et al. 2015

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Background: Babies with congenital hydrocephalus often experience developmental disabilities due to brain injury associated with prolonged increased pressure on the developing brain parenchyma. Umbilical cord blood (CB) infusion has favorable effects in animal models of brain hypoxia and stroke and is being investigated in clinical trials of brain injury in both children and adults. We sought to establish the safety and feasibility of repeated intravenous infusions of autologous CB in young babies with congenital hydrocephalus. Methods: Infants with severe congenital hydrocephalus and an available qualified autologous CB unit traveled to Duke for evaluation and CB infusion. When possible, the CB unit was utilized for multiple infusions. Patient and CB data were obtained at the time of infusion and analyzed retrospectively. results: From October 2006 to August 2014, 76 patients with congenital hydrocephalus received 143 autologous CB infusions. Most babies received repeated doses, for a total of two (n = 45), three (n = 18), or four (n = 4) infusions. There were no infusion-related adverse events. As expected, all babies experienced developmental delays. conclusion: Cryopreserved CB products may be effectively manipulated to provide multiple CB doses. Repeated intravenous infusion of autologous CB is safe and feasible in young babies with congenital hydrocephalus.h ydrocephalus results from an excessive accumulation of cerebral spinal fluid (CSF) within the ventricular system of the brain resulting in a progressive increase in ventricular volume and intracranial pressure. It may be caused by a blockage within the ventricular system, overproduction of cerebral spinal fluid, or decreased absorption of cerebral spinal fluid.The incidence of congenital hydrocephalus has been estimated as 0.3 to 1.5 per 1,000 live births (1,2). Congenital hydrocephalus may occur in isolation or as a result of neural dysgenesis, such as spina bifida or a Dandy Walker malformation. Though many patients with congenital hydrocephalus are suspected to have a genetic cause, a causal mutation is identified in only a small portion of patients, most commonly X-linked hydrocephalus due to a mutation in L1CAM (3). Various other chromosomal abnormalities have been described in babies with hydrocephalus with additional associated somatic defects. Such genetic cases can usually be identified before or at birth.Most cases of congenital hydrocephalus are diagnosed in utero on routine prenatal, screening ultrasonography when macrocephaly and/or ventriculomegaly are seen. Typically signs of hydrocephalus are first recognized between 15-10 wk of gestation. Options at the time of an in utero diagnosis of severe hydrocephalus are currently limited to termination of the pregnancy or expectant management. Fetal shunt placement has been attempted, but technical issues with the shunt and mixed patient outcomes resulted in a moratorium on fetal percutaneous shunting in the United States since 1985 (4,5).Current standard management of a baby with severe congeni...

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Repeated autologous umbilical cord blood infusions are feasible and had no acute safety issues in young babies with congenital hydrocephalus

et al. 2015

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“…The team from the Fetal Treatment Center team did not get discouraged by this outcome: the majority of patients with LUTO could be safely and effectively helped by shunt placement (Harrison et al, 1981a,b). At that time these shunts were also proposed for in utero treatment of hydrocephalus until experimental work in fetal sheep and monkeys showed that such a treatment was ineffective (Clewell et al, 1982). This is a good example of how, despite great initial enthusiasm, critical evaluation of results prompted reconsideration.…”

Section: Luto and Early Applications Of Shuntingmentioning

confidence: 99%

The making of fetal surgery

et al. 2010

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Fetal diagnosis prompts the question for fetal therapy in highly selected cases. Some conditions are suitable for in utero surgical intervention. This paper reviews historically important steps in the development of fetal surgery. The first invasive fetal intervention in 1963 was an intra-uterine blood transfusion. It took another 20 years to understand the pathophysiology of other candidate fetal conditions and to develop safe anaesthetic and surgical techniques before the team at the University of California at San Francisco performed its first urinary diversion through hysterotomy. This procedure would be abandoned as renal and pulmonary function could be just as effectively salvaged by ultrasound-guided insertion of a bladder shunt. Fetoscopy is another method for direct access to the feto-placental unit. It was historically used for fetal visualisation to guide biopsies or for vascular access but was also abandoned following the introduction of high-resolution ultrasound. Miniaturisation revived fetoscopy in the 1990s, since when it has been successfully used to operate on the placenta and umbilical cord. Today, it is also used in fetuses with congenital diaphragmatic hernia (CDH), in whom lung growth is triggered by percutaneous tracheal occlusion. It can also be used to diagnose and treat urinary obstruction. Many fetal interventions remain investigational but for a number of conditions randomised trials have established the role of in utero surgery, making fetal surgery a clinical reality in a number of fetal therapy programmes. The safety of fetal surgery is such that even non-lethal conditions, such as myelomeningocoele repair, are at this moment considered a potential indication. This, as well as fetal intervention for CDH, is currently being investigated in randomised trials.

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“…Because of poor postnatal neurologic outcomes, efforts were made in the 1980s to intervene prenatally in order to bypass the obstructed aqueduct by means of serial cephalocentesis [10] and ventriculoamniotic shunting [11,12]. Ventriculoperitoneal shunting in newborns dramatically improved survival [13].…”

Section: Fetal Intervention For Severe Hydrocephalusmentioning

confidence: 99%

Fetal Therapy for Isolated Aqueductal Stenosis

Emery¹,

Greene

Hogge³

2015

Fetal Diagn Ther

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Aqueductal stenosis (AS) is a form of noncommunicating hydrocephalus, which causes increased intracranial pressure secondary to obstruction of the aqueduct of Sylvius. Relief of intracranial pressure in the fetus by ventriculoamniotic shunting may diminish or even prevent permanent neurologic injury. Shunting was attempted in the 1980s but was abandoned due to technical difficulties. Given the advances in prenatal diagnosis and fetal intervention over the last 3 decades, we believe that an evidence-based reevaluation of the option is timely and appropriate. The aim of this review article is to discuss the clinical significance of the diagnosis of AS, current management strategies, current diagnostic capabilities, new shunt technology, and barriers to progress. Finally, we will advance a research agenda that will provide evidence-based management recommendations.

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A Surgical Approach to the Treatment of Fetal Hydrocephalus

Cited by 195 publications

References 5 publications

Repeated autologous umbilical cord blood infusions are feasible and had no acute safety issues in young babies with congenital hydrocephalus

Repeated autologous umbilical cord blood infusions are feasible and had no acute safety issues in young babies with congenital hydrocephalus

The making of fetal surgery

Fetal Therapy for Isolated Aqueductal Stenosis

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