Spring-Assisted Cranial Expansion for Multisuture Craniosynostosis: First Case Report from the Indian Subcontinent.

We present a first use case report from the Indian subcontinent of a 5-month-old child with multisuture craniosynostosis with raised intracranial pressure managed by spring-assisted cranial expansion followed by traditional fronto-orbital advancement and cranial vault remodeling. We emphasize the advantages of spring-assisted cranial expansion in extremely young infants with raised intracranial pressure over posterior vault distraction osteogenesis and open posterior vault remodeling.

External cranial expansion as treatment of intracranial hypertension. Technical note.

Intracranial hypertension may be idiopathic or due to multiple etiologies. Some bone dysplasias and chronic shunt overdrainage syndrome may sometimes lead to intracranial hypertension associated with craniocerebral disproportion due to thickening of cranial diploe. The internal cranial expansion procedure has been used for patients with intracranial hypertension, whether or not associated with craniocerebral disproportion. Its purpose is to increase the intracranial volume by drilling down the inner table. This technique enables the craniocerebral disproportion to be improved and intracranial hypertension reduced. In other etiologies of intracranial hypertension with non-thickened diploe, internal cranial expansion may not be enough to resolve the hypertension. For these cases we propose a modification of the technique by expanding the cranial vault outwards; external cranial expansion. We describe this technique as used in a pediatric patient who presented with chronic headache, tonsillar ectopia, and sleep apnea syndrome. This patient also had a multisuture craniosynostosis, a non-thickened diploe, and intracranial hypertension.

Conventional posterior cranial vault expansion: indications and results-review of the literature.

In the late 1980s, craniofacial surgery units reported suboptimal cosmetic results, cranial volume restriction, and intracranial hypertension after anterior cranial vault remodeling in bilateral coronal synostosis associated with severe brachyturricephaly. A possible explanation was a severe associated growth restriction of the posterior calvaria with radiological synostosis at the lambda sutures. "Conventional" or "fixed" posterior cranial vault expansion techniques were developed to address these limitations, sometimes as the first surgical step in a two-staged protocol of total calvarial reconstruction, combined with suboccipital decompression in cases of symptomatic cerebellar tonsillar herniation or, more easily, to resolve the characteristic occipital flattening of lambdoid synostosis. Various surgical approaches have been described; however, the indications for and timing of surgical treatment and postoperative evaluation of results still remain controversial. Although more invasive, conventional posterior cranial vault expansion has proven to be safe and offers a remodeled and protective bony vault immediately after surgery, but the underlying cranial base malformation remained untreated, with implications in the postoperative growth of the facial skeleton. Overcorrection, rigid stabilization, and grafting are also concerns to be addressed.

Extended experience in parieto-occipital expansion surgery by meander technique-clinical and radiological evaluation.

INTRODUCTION: Brachycephaly and anterior and posterior plagiocephaly appear as an isolated entity or manifest in syndromic conditions. In severe cases, possible treatment options currently comprise either cranioplasty or osteogenetic distraction. The aim of this paper is to retrospectively review the perioperative course of a series of children treated by posterior meander expansion technique at our institution with focus on the course of postoperative intracranial volume and eventual tonsillar descent evolution. METHODS: Forty-two children received a posterior cranial vault remodeling by means of a posterior meander technique during a 7-year period. Hospital records were reviewed, and pre- and postoperative MRIs were analyzed for intracranial volume, cephalic and asymmetry index, and tonsillar position over time. RESULTS: Median age at surgery was 11.5 months (range 17 days-10 years). Nineteen children had a symmetrical cranial deformity, twenty-three an asymmetrical synostosis. Half of the cohort showed a syndromic condition. Transfusions were administered in the majority (92.2%) of the cases. A significant postoperative increase of intracranial volume was present from 1188.9 ± 370.4 cm3 to 1324.8 ± 352.9 cm3 (p < 0.001). The asymmetry index showed a significant improvement postoperatively: 0.86 ± 0.06 versus 0.91 ± 0.05 (p < 0.001), while the cephalic index showed a non-statistical change (0.91 ± 0.11 versus 0.88 ± 0.08). Tonsillar herniation, bilateral or homolateral, showed no significant changes at early control, while a nonsignificant amelioration of tonsillar descent was seen among children older than 12 months at late imaging follow-up. CONCLUSION: Among the osteoplastic techniques, the posterior meander technique offers several advantages, such as early mobilization of the child, less bony defects, absence of implants, and a small complication rate. However, further comparative studies among different surgical techniques are needed.

Whether cranial expansion is indicated in selected cases with microcephaly?

PURPOSE: Some children with microcephaly experienced severe social emotional deficits during their 1-2 years of age. Cranial expansion is generally not used in these cases. Our aim is to assess whether such operation is effective to improve their social emotional status, and thereby boosting their functional development in selected cases with such condition. METHODS: A retrospective cohort review in microcephalic cases who had undergone cranial expansion in Shanghai Children's Hospital since Jun. 2016 to Jun. 2017 with at least 12 months follow-up was conducted. Inclusion and exclusion criteria were set for the selection of the target patients in the current study. Our study focused on the changes of social emotional status (evaluated by The Infant-Toddler Social and Emotional Assessment, ITSEA) and functional development (applying neurodevelopmental questionnaire) pre- and 12 months post-op in these cases. RESULTS: A total of 14 cases were included in the current study. On the basis of a 2-cm enlargement during surgery(1 cm advancement of fronto-orbital rim on each side), HC continued to increase at an average of 0.5 cm in one year after the procedure in these cases, making its corresponding standard deviation improve from -4.5 to -3.4. Among those 14 cases, 11 (78.6%) showed improvement in all of these four domains in ITSEA at 12 months post-op. Scores were significantly better at 12 months post-op with p < 0.01 compared with those in domains of internalizing behavior and dysregulation behavior pre-operatively. Improvement was observed with p < 0.05 in domains of externalizing behavior and social-emotional competence as well. With regard to functional development, 8 cases (57.1%) were revealed improvement in all of these three sections of motor, speech and cognitive function in the questionnaire, 5 (35.7%) concluded partially upgraded in 1 or 2 sections. Only one (7.1%) showed no change during the follow-up. No surgery-related complications were recorded in the current study. CONCLUSION: When progressive copper-beaten sign is revealed in their serial skull X-rays, microcephalic cases with social emotional deficits in their 1-2 years of age could benefit from cranial expansion surgery with regard to improving their social emotional status and functional development.

Cranial expansion and aqueductoplasty for combined isolated fourth ventricle and slit-ventricle syndrome: a surgical alternative.

INTRODUCTION: An isolated fourth ventricle (IFV) is a rare entity observed in shunted patients and its treatment is still uncertain. Endoscopic aqueductoplasty has shown good results for restoring CSF flux between the third and fourth ventricles. However, it needs some grade of ventricular dilation to be performed. Some patients affected by IFV show slit-ventricle morphology in CT/MRI. Usually, the rise of opening pressure or the shunt externalization gets enough ventricular dilation. However, the lack of intracranial compliance in some patients makes these options unsuitable and high-ICP symptoms are developed without ventricular dilation. METHODS: We present a two cases series affected by IFV with no ventricular dilation in radiological exams. ICP sensors were implanted, observing high-ICP and establishing the diagnosis of craniocerebral disproportion. A two-stage surgical plan based on a dynamic cranial expansion followed by a supratentorial endoscopic aqueductoplasty was performed. A physical and mathematical model explaining our approach was also provided. RESULTS: Chess-table cranial expansion technique was performed in both patients. Six/seven days after the first surgery, respectively, ventricular dilation was observed in CT. Endoscopic precoronal aqueductoplasty was then performed. No postoperative complications were described. IFV symptoms improved in both patients. Eighteen and 12 months after the two-stage surgical plan, the patients remain symptom-free and void of flow is still observed between the third and the fourth ventricles in MRI. CONCLUSION: The two-stage approach was a suitable option for the treatment of these complex patients affected by both craniocerebral disproportion and isolated fourth ventricle.

Dynamic Chess-Table Cranial Expansion for Treatment of Craniocerebral Disproportion: Technical Note and Volumetric Results.

OBJECTIVE: Craniocerebral disproportion (CCD) is a challenging disease, and several expansile procedures have been used for its treatment. This report describes the dynamic chess-table cranial expansion technique and reports on 9 patients with primary and secondary CCD treated with this procedure. METHODS: Nine patients affected by CCD were treated with chess-table cranial expansion and reviewed. Symptoms of increased intracranial pressure (ICP) and radiologic findings were analyzed. ICP was monitored using epidural or telemetric sensors. Intracranial volume was measured using computed tomography image processing tools before the surgery, 24 hours after the surgery, and 1 month later. A mathematical model was developed to explain the clinical and surgical results. RESULTS: Five patients had secondary CCD and 4 had primary CCD. The mean age for cranial expansion was 16.78 years. The most frequent symptoms were headaches, nausea/vomiting, and decreased consciousness. Slit ventricles and sutural sclerosis were observed in 33.3% and 55.6% of patients. The mean ICP before the procedure was 48.67 mm Hg. Progressive cranial expansion was seen in all patients after surgery. The mean ICP decreased to 11 mm Hg and mean intracranial volume expansion was 85.8 cm3 at 1 month after surgery. There were no serious complications after surgery. All patients improved their symptoms, and no patient needed additional cranial expansion procedures. CONCLUSIONS: Chess-table cranial expansion is a safe and effective procedure and may be used as an alternative treatment for CCD. Progressive expansion of the intracranial volume is related to a decrease in ICP recordings and an improvement of symptoms.

Transsutural distraction osteogenesis for 285 children with craniosynostosis: a single-institution experience.

OBJECT Although distraction osteogenesis (DO) requires a secondary procedure in the surgical correction of craniosynostosis, it is relatively simple, requires less transfusion, results in a shorter intensive care unit stay, and is quite safe. Because of these positive factors, various DO techniques have been developed. However, there is disagreement regarding the superiority of DO. The authors reported on a new DO technique, transsutural DO (TSDO), 6 years ago that was performed in 23 patients over a period of 6 months, and it continues to be used at the present time. In this paper the authors report the results of TSDO performed in 285 patients with craniosynostosis over a period of 6 years at a single institution. METHODS TSDO consists of a simple suturectomy of the pathological suture followed by direct distraction of the suturectomy site only. Types of TSDO conducted included sagittal TSDO in 95 patients, bicoronal in 14, unilateral coronal in 57, lambdoid in 26, metopic in 13, multiple in 19, syndromic in 33, and secondary in 28. The mean age (± SD) of the patients was 19.4 ± 23.0 months, and mean follow-up was 39.5 ± 21.0 months. RESULTS The mean operating time was 115 ± 43 minutes, and mean anesthesia time was 218 ± 56 minutes. The mean transfusion volume of red blood cell components was 48 ± 58 ml, and mean transfusion volume of fresh-frozen plasma was 19 ± 35 ml. Total transfusion volume was significantly less in infants younger than 12 months of age and in children with lower lumbar puncture pressures (p < 0.05). Complications included 1 (0.4%) death from postoperative acute pneumonia after a distractor removal operation and 23 (8%) surgical morbidities comprising 10 revisions (3.5%) and 13 early removals of distracters (4.6%). CONCLUSIONS TSDO is a simple, effective, and safe method to use for treating all types of craniosynostosis. Some morbidity was experienced in this study, but it may be attributed to the learning curve of the technique.

Spring-assisted cranial vault expansion in the setting of multisutural craniosynostosis and anomalous venous drainage: case report.

Patients with multisutural craniosynostosis can develop anomalous venous connections between the intracranial sinuses and cutaneous venous system through enlarged emissary veins. Cranial vault remodeling in this subset of patients carries the risk of massive intraoperative blood loss and/or occlusion of collateral draining veins leading to intracranial venous hypertension and raised intracranial pressure, increasing the morbidity of cranial expansion. The authors report the use of spring-mediated expansion as a technique for cranial reconstruction in which the collateral intracranial venous drainage system can be preserved. A patient with bilateral lambdoid, sagittal, and unicoronal synostosis presented for cranial reconstruction. A tracheostomy and ventriculoperitoneal shunt were placed prior to intervention. At the time of reconstruction, a Luckenschadel skull abnormality and Chiari malformation Type I were present. A preoperative CT venogram demonstrated large collateral superficial occipital veins, small bilateral internal jugular veins, and hypoplastic jugular foramina. Collateral flow from the transverse and sigmoid sinuses through large occipital emissary veins was seen. Spring-mediated cranial vault expansion was performed with care to preserve the large collateral veins at the occipital midline. Four springs were placed at each lambdoid and the posterior and anterior sagittal sutures following 1-cm strip suturectomies. Removal of the springs was performed 2 months postoperatively. Cranial vault expansion was performed without disturbing the aberrant intracranial/extracranial venous collateral system. Estimated blood loss was 150 ml. A CT scan obtained 3 months postoperatively showed resolution of the Luckenschadel deformity and a 40% volumetric increase in the skull compared with the preoperative CT. Patients with anomalous venous drainage patterns and multisutural synostosis can undergo spring-mediated cranial vault expansion while preserving the major emissary veins draining the intracranial sinuses. Risks of blood loss, intracranial venous hypertension, and increased intracranial pressure may be decreased compared with traditional techniques of repair.