scholarly journals Hydrocephalus and craniosynostosis

1997 ◽  
Vol 3 (6) ◽  
pp. E1 ◽  
Author(s):  
Giuseppe Cinalli ◽  
Christian Sainte-Rose ◽  
Eve Marie Kollar ◽  
Michel Zerah ◽  
Francis Brunelle ◽  
...  
Keyword(s):  
Tonsillar Herniation ◽  
Ventricular Dilation ◽  
Syndromic Craniosynostosis ◽  
Hydrodynamic Disturbance ◽  
Csf Hydrodynamics ◽  
Constant Feature ◽  
Apert's Syndrome ◽  
Subarachnoid Spaces ◽  
Crouzon's Syndrome

Object A retrospective study of 1727 cases of craniosynostosis was undertaken to determine the interrelationship between abnormal cerebrospinal fluid (CSF) hydrodynamics and craniosynostosis. Methods The patients were divided intwo two groups: nonsyndromic craniosynostosis and syndromic craniosynostosis. Cases of occipital plagiocephaly without suture synostosis and cases of shunt-induced craniosynostosis were excluded from the study. The majority of patients (1297) were treated surgically for their cranial deformity; 95% of these patients had a postoperative follow-up review lasting 5 years. Clinical and radiographic charts covering the time from presentation through the follow-up period were reviewed. Conclusions Abnormal intracranial CSF hydrodynamics was found in 8.1% of the patients (3.4% of whom had received shunts and 4.5% of whom had not). Three types of CSF hydrodynamic disturbance were observed: progressive hydrocephalus with ventricular dilation, nonprogressive ventriculomegaly, and dilation of the subarachnoid spaces. Hydrocephalus occurred much more frequently in patients with syndromic craniosynostosis (12.1%) than in those with isolated craniosynostosis (0.3%). In fact, patients with kleeblattschädel exhibited hydrocephalus as a constant feature and patients with Crouzon's syndrome were far more likely to have hydrocephalus than those with other syndromes. In Apert's syndrome, ventricular dilation occurred very frequently, but it was almost always nonprogressive in nature. In most cases of syndromic craniosynostosis, venous sinus obstruction and/or chronic tonsillar herniation were found. Their role in the pathophysiology of hydrocephalus in craniosynostosis is discussed.

Hydrocephalus and craniosynostosis

1998 ◽  
Vol 88 (2) ◽  
pp. 209-214 ◽  
Author(s):  
Giuseppe Cinalli ◽  
Christian Sainte-Rose ◽  
Eve Marie Kollar ◽  
Michel Zerah ◽  
Francis Brunelle ◽  
...  
Keyword(s):  
Ventricular Dilation ◽  
Content Type ◽  
Syndromic Craniosynostosis ◽  
Review Period ◽  
Hydrodynamic Disturbance ◽  
Csf Hydrodynamics ◽  
Constant Feature ◽  
Apert's Syndrome ◽  
Fine Print

Object. A retrospective study of 1727 cases of craniosynostosis was undertaken to determine the interrelationship between abnormal cerebrospinal fluid (CSF) hydrodynamics and craniosynostosis. Methods. The patients were divided into two groups: nonsyndromic craniosynostosis and syndromic craniosynostosis. Cases of occipital plagiocephaly without suture synostosis and cases of shunt-induced craniosynostosis were excluded from the study. The majority of patients (1297) were treated surgically for their cranial deformity; 95% of these patients had a postoperative follow-up review period lasting 5 years. Clinical and radiographic charts covering the time from presentation through the follow-up period were reviewed. Conclusions. Abnormal intracranial CSF hydrodynamics was found in 8.1% of the patients (3.4% of whom had received shunts and 4.5% of whom had not). Three types of CSF hydrodynamic disturbance were observed: progressive hydrocephalus with ventricular dilation, nonprogressive ventriculomegaly, and dilation of the subarachnoid spaces. Hydrocephalus occurred much more frequently in patients with syndromic craniosynostosis (12.1%) than in those with isolated craniosynostosis (0.3%). In fact, patients with kleeblattschädel exhibited hydrocephalus as a constant feature and patients with Crouzon's syndrome were far more likely to have hydrocephalus than those with other syndromes. In Apert's syndrome, ventricular dilation occurred very frequently, but it was almost always nonprogressive in nature. In most cases of syndromic craniosynostosis, venous sinus obstruction and/or chronic tonsillar herniation were found. Their role in the pathophysiology of hydrocephalus in craniosynostosis is discussed.

Chronic tonsillar herniation in Crouzon's and Apert's syndromes: the role of premature synostosis of the lambdoid suture

1995 ◽  
Vol 83 (4) ◽  
pp. 575-582 ◽  
Author(s):  
Giuseppe Cinalli ◽  
Dominique Renier ◽  
Guy Sebag ◽  
Christian Sainte-Rose ◽  
Eric Arnaud ◽  
...  
Keyword(s):  
Resonance Imaging ◽  
Tonsillar Herniation ◽  
Content Type ◽  
Lambdoid Suture ◽  
High Incidence ◽  
Apert's Syndrome ◽  
Suture Closure ◽  
Crouzon's Syndrome ◽  
Fine Print

✓ The incidence of chronic tonsillar herniation (CTH) was evaluated with magnetic resonance imaging in 44 patients with Crouzon's syndrome and 51 with Apert's syndrome; the incidence was 72.7% in Crouzon's syndrome and 1.9% in Apert's syndrome. All the patients with Crouzon's syndrome and progressive hydrocephalus had CTH, but of 32 individuals with Crouzon's syndrome and CTH, only 15 had progressive hydrocephalus. Five patients with Apert's syndrome were treated for progressive hydrocephalus; none had CTH. The patterns of suture closure in these two groups of patients were studied, and significant differences in coronal, sagittal, and lambdoid sutures were found between patients with Crouzon's and Apert's syndromes. In Crouzon's syndrome, significant differences in the pattern of lambdoid suture closure were found between the groups with and without CTH; in the group with CTH, the lambdoid closure appeared earlier. The authors propose that the high incidence of individuals with CTH who have Crouzon's syndrome is related to the premature synostosis of the lambdoid suture in the first 24 months of age.

The Use of Split Thickness Skin Grafts in the Correction of Apert’s Syndactyly

2001 ◽  
Vol 26 (1) ◽  
pp. 8-10 ◽  
Author(s):  
M. M. AL-QATTAN
Keyword(s):  
Skin Graft ◽  
Joint Contracture ◽  
Skin Grafts ◽  
Thickness Skin ◽  
Constant Feature ◽  
Apert's Syndrome ◽  
Web Space ◽  
Skin Graft Contraction ◽  
The Web

Stiffness of the interphalangeal joints of the fingers is a constant feature of Apert’s syndrome. Because of this stiffness, the author has used split-thickness skin grafts when correcting Apert’s syndactyly, thinking that contraction of such grafts post-operatively would not cause any joint contracture or finger deviation. This paper reports the results of eight patients whose average age at first surgery was 6 months. Separation of all digits was accomplished before the age of 2 years. A dorsal rectangular flap and interposing triangular digital flaps were utilised to create the web space and partially cover the skin defects in the fingers. The remaining digital defects were covered with thin split-thickness skin grafts which took fully in all cases. At final follow-up (1–6 years), the areas covered by skin grafts have reduced in size significantly because of skin graft contraction. However, this did not result joint contracture or digital deviation.

scholarly journals Ophthalmological findings in children with non-syndromic craniosynostosis: preoperatively and postoperatively up to 12 months after surgery

2021 ◽  
Vol 6 (1) ◽  
pp. e000677
Author(s):  
Evangelia Ntoula ◽  
Daniel Nowinski ◽  
Gerd Holmstrom ◽  
Eva Larsson
Keyword(s):  
Visual Acuity ◽  
University Hospital ◽  
Cranial Sutures ◽  
Sagittal Craniosynostosis ◽  
Syndromic Craniosynostosis ◽  
Disc Oedema ◽  
Before And After ◽  
Congenital Condition ◽  
Skull Deformity

AimsCraniosynostosis is a congenital condition characterised by premature fusion of one or more cranial sutures. The aim of this study was to analyse ophthalmic function before and after cranial surgery, in children with various types of non-syndromic craniosynostosis.MethodsChildren referred to Uppsala University Hospital for surgery of non-syndromic craniosynostosis were examined preoperatively. Visual acuity was measured with Preferential Looking tests or observation of fixation and following. Strabismus and eye motility were noted. Refraction was measured in cycloplegia and funduscopy was performed. Follow-up examinations were performed 6–12 months postoperatively at the children’s local hospitals.ResultsOne hundred twenty-two children with mean age 6.2 months were examined preoperatively. Refractive values were similar between the different subtypes of craniosynostosis, except for astigmatism anisometropia which was more common in unicoronal craniosynostosis. Strabismus was found in seven children, of which four had unicoronal craniosynostosis.Postoperatively, 113 children were examined, at mean age 15.9 months. The refractive values decreased, except for astigmatism and anisometropia in unicoronal craniosynostosis. Strabismus remained in unicoronal craniosynostosis. Two new cases with strabismus developed in unicoronal craniosynostosis and one in metopic, all operated with fronto-orbital techniques. No child had disc oedema or pale discs preoperatively or postoperatively.ConclusionOphthalmic dysfunctions were not frequent in children with sagittal craniosynostosis and preoperative ophthalmological evaluation may not be imperative. Children with unicoronal craniosynostosis had the highest prevalence of strabismus and anisometropia. Fronto-orbital techniques used to address skull deformity may be related to a higher prevalence of strabismus postoperatively.

Development of secondary unilateral coronal suture synostosis with a sagittal suture synostosis in a nonsyndromic patient

2012 ◽  
Vol 9 (2) ◽  
pp. 116-118 ◽  
Author(s):  
Leonardo Rangel-Castilla ◽  
Steven W. Hwang ◽  
Andrew Jea ◽  
William E. Whitehead ◽  
Daniel J. Curry ◽  
...  
Keyword(s):  
Surgical Intervention ◽  
Early Stage ◽  
Sagittal Synostosis ◽  
Clinical Appearance ◽  
Syndromic Craniosynostosis ◽  
Intervention Failure ◽  
Frontoorbital Advancement ◽  
Coronal Synostosis ◽  
Second Procedure

Multiple-suture synostosis is typically associated with syndromic craniosynostosis but has been occasionally reported in large series of nonsyndromic children. The diagnosis of multiple fused sutures usually occurs at the same time, but rarely has the chronological development of a secondary suture synostosis been noted. The development of secondary bicoronal suture synostosis requiring surgical intervention has only been reported, to date, after surgical intervention and is hypothesized to arise from a disruption of inhibitory factors from the dura. The disinhibition of these factors permits the sutures to then fuse at an early stage. The authors report on a patient who developed secondary unilateral coronal synostosis after the diagnosis of an isolated sagittal synostosis. The secondary synostosis was identified at the time of the initial surgical intervention and ultimately required a second procedure of a frontoorbital advancement. The clinical appearance of this phenomenon may be subtle, and surgeons should monitor for the presence of secondary synostosis during surgery as it may require intervention. Failure to identify the secondary synostosis may necessitate another surgery or result in a poor cosmetic outcome. The authors recommend close clinical follow-up for the short term in patients with isolated sagittal synostosis.

scholarly journals Outcomes of endoscopic suturectomy with postoperative helmet therapy in bilateral coronal craniosynostosis

2016 ◽  
Vol 18 (3) ◽  
pp. 281-286 ◽  
Author(s):  
S. Alex Rottgers ◽  
Subash Lohani ◽  
Mark R. Proctor
Keyword(s):  
Hospital Stay ◽  
Head Circumference ◽  
Major Complication ◽  
Csf Leak ◽  
Perioperative Morbidity ◽  
Syndromic Craniosynostosis ◽  
Duration Of Surgery ◽  
Coronal Synostosis ◽  
The Mean

OBJECTIVE Historically, bilateral frontoorbital advancement (FOA) has been the keystone for treatment of turribrachycephaly caused by bilateral coronal synostosis. Early endoscopic suturectomy has become a popular technique for treatment of single-suture synostosis, with acceptable results and minimal perioperative morbidity. Boston Children's Hospital has adopted this method of treating early-presenting cases of bilateral coronal synostosis. METHODS A retrospective review of patients with bilateral coronal craniosynostosis who were treated with endoscopic suturectomy between 2005 and 2012 was completed. Patients were operated on between 1 and 4 months of age. Hospital records were reviewed for perioperative morbidity, length of stay, head circumference and cephalic indices, and the need for further surgery. RESULTS Eighteen patients were identified, 8 males and 10 females, with a mean age at surgery of 2.6 months (range 1–4 months). Nine patients had syndromic craniosynostosis. The mean duration of surgery was 73.3 minutes (range 50–93 minutes). The mean blood loss was 40 ml (range 20–100 ml), and 2 patients needed a blood transfusion. The mean duration of hospital stay was 1.2 days (range 1–2 days). There was 1 major complication in the form of a CSF leak. The mean follow-up was 37 months (range 6–102 months). Eleven percent of nonsyndromic patients required a subsequent FOA; 55.6% of syndromic patients underwent FOA. The head circumference percentiles and cephalic indices improved significantly. CONCLUSIONS Early endoscopic suturectomy successfully treats the majority of patients with bilateral coronal synostosis, and affords a short procedure time, a brief hospital stay, and an expedited recovery. Close follow-up is needed to detect patients who will require a secondary FOA due to progressive suture fusion or resynostosis of the released coronal sutures.

Morphometric analysis of posterior fossa after in utero myelomeningocele repair

2011 ◽  
Vol 7 (4) ◽  
pp. 362-368 ◽  
Author(s):  
Ryan A. Grant ◽  
Gregory G. Heuer ◽  
Geneive M. Carrión ◽  
N. Scott Adzick ◽  
Erin S. Schwartz ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Surgical Repair ◽  
Correlation Coefficients ◽  
In Utero ◽  
Clinical Effects ◽  
Type Ii ◽  
Tonsillar Herniation ◽  
Myelomeningocele Repair ◽  
Repair Group

Object Myelomeningocele (MMC) is characterized by a defect in caudal neurulation and appears at birth with a constellation of neuroanatomical abnormalities, including Chiari malformation Type II. The authors investigated the effects of antenatal versus postnatal repair of MMC through a quantitative analysis of morphometric changes in the posterior fossa (PF). Methods The authors retrospectively reviewed the records of 29 patients who underwent in utero MMC repair, 24 patients who underwent postnatal repair, and 114 fetal and pediatric controls. Tonsillar displacement, cerebellum length, pons length, clivus-supraocciput (CSO) angle, and PF area were compared in antenatal and postnatal MMC repair groups as well as in controls without neural tube defects by using t-tests and correlation coefficients. Results Initially, the in utero CSO angle was significantly more acute in all patients with MMC—prenatally and postnatally repaired—as compared with controls (57.8° vs 75.4°, p < 0.001); however, the angle rapidly changed and became similar to that in controls between 30 and 31 weeks' gestation to approximately 80°, with antenatal repair having little effect. Postnatally, the CSO angle decreased in controls (R = −0.58) and in the antenatal repair group (R = −0.17). The cerebellum and pons length demonstrated no significant differences in any group. Overall, tonsil descent was corrected in the antenatal repair group as compared with postnatal repair (p < 0.001), and the PF area increased in all 3 groups in utero. Growth was less rapid in patients with MMC compared with controls, but this was corrected by antenatal repair (p = 0.015). Conclusions Myelomeningocele was associated with tonsillar herniation and a smaller PF than in control fetuses. Antenatal surgical repair corrected both abnormalities. The CSO angle began significantly more acutely in patients with MMC, but normalized with development regardless of when surgery was performed. Determining the clinical effects of antenatal repair requires further follow-up.

scholarly journals Resolution of neonatal posthemorrhagic ventricular dilation coincident with patent ductus arteriosus ligation: case report

2020 ◽  
Vol 26 (3) ◽  
pp. 255-261 ◽  
Author(s):  
Erik B. Vanstrum ◽  
Matthew T. Borzage ◽  
Jason K. Chu ◽  
Shuo Wang ◽  
Nolan Rea ◽  
...  
Keyword(s):  
Patent Ductus Arteriosus ◽  
Intraventricular Hemorrhage ◽  
Ductus Arteriosus ◽  
Medical Intervention ◽  
Cranial Ultrasound ◽  
Ventricular Dilation ◽  
Venous Flow ◽  
Systemic Hemodynamic ◽  
Patent Ductus

Preterm infants commonly present with a hemodynamically significant patent ductus arteriosus (hsPDA). The authors describe the case of a preterm infant with posthemorrhagic ventricular dilation, which resolved in a temporally coincident fashion to repair of hsPDA. The presence of a PDA with left-to-right shunting was confirmed at birth on echocardiogram and was unresponsive to repeated medical intervention. Initial cranial ultrasound revealed periventricular-intraventricular hemorrhage. Follow-up serial ultrasound showed resolving intraventricular hemorrhage and progressive bilateral hydrocephalus. At 5 weeks, the ductus was ligated with the goal of improving hemodynamic stability prior to CSF diversion. However, neurosurgical intervention was not required due to improvement of ventriculomegaly occurring immediately after PDA ligation. No further ventricular dilation was observed at the 6-month follow-up.Systemic venous flow disruption and abnormal patterns of cerebral blood circulation have been previously associated with hsPDA. Systemic hemodynamic change has been reported to follow hsPDA ligation, although association with ventricular normalization has not. This case suggests that the unstable hemodynamic environment due to left-to-right shunting may also impede CSF outflow and contribute to ventriculomegaly. The authors review the literature surrounding pressure transmission between a PDA and the cerebral vessels and present a mechanism by which PDA may contribute to posthemorrhagic ventricular dilation.

Midterm Follow-Up of Midface Distraction for Syndromic Craniosynostosis: A Clinical and Cephalometric Study

2007 ◽  
Vol 120 (6) ◽  
pp. 1621-1632 ◽  
Author(s):  
Pradip R. Shetye ◽  
Sean Boutros ◽  
Barry H. Grayson ◽  
Joseph G. McCarthy
Keyword(s):  
Syndromic Craniosynostosis ◽  
Cephalometric Study

Presence of lymphocyte aggregates in the synovium of patients with early arthritis in relationship to diagnosis and outcome: is it a constant feature over time?

2010 ◽  
Vol 70 (4) ◽  
pp. 700-703 ◽  
Author(s):  
Marleen G H van de Sande ◽  
Rogier M Thurlings ◽  
Maria J H Boumans ◽  
Carla A Wijbrandts ◽  
Maria Grazia Modesti ◽  
...  
Keyword(s):  
Clinical Outcome ◽  
Definitive Diagnosis ◽  
Early Arthritis ◽  
Follicular Dendritic Cells ◽  
Constant Feature ◽  
Drug Naïve ◽  
N 93 ◽  
Over Time ◽  
Stable Feature

ObjectivesTo evaluate the presence of lymphocyte aggregates in synovial tissue of patients with early arthritis in relationship to clinical outcome and to determine whether this is a stable feature over time.MethodsArthroscopic synovial biopsy samples were collected in a prospective cohort of disease-modifying antirheumatic drug-naïve patients with early arthritis (<1 year's disease duration) at baseline (n=93) and, if rheumatoid arthritis was suspected, after 6 months of follow-up (n=17). After 2 years of follow-up, definitive diagnosis and clinical outcome were assessed. Size of synovial lymphocyte aggregates was graded (score 1–3). Lymphoid neogenesis (LN) was defined by the presence of grade ≥2 aggregates and subclassified based on the presence of follicular dendritic cells (FDCs).ResultsLN was present in 36% of all patients and FDCs in 15% of patients with LN. Presence of lymphocyte aggregates differed over time. LN was associated with the degree of synovial inflammation. There was no relationship between the presence of lymphocyte aggregates at baseline and definitive diagnosis or clinical outcome after follow-up.ConclusionsPresence of lymphocyte aggregates is a dynamic phenomenon related to the degree of synovitis and can be detected in different forms of early arthritis. This feature does not appear to be related to clinical outcome.

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