[Prospective analysis of inflammatory markers and perioperative clinical data in children with craniosynostosis undergoing reconstructive surgery]. / Prospektivnyi analiz dinamiki laboratornykh markerov vospaleniya i klinicheskoi kartiny perioperatsionnogo perioda pri rekonstruktivnykh operatsiyakh u detei s kraniosinostozami.

BACKGROUND: Craniosynostosis (CS) is a group of skull malformations manifested by congenital absence or premature closure of cranial sutures. Reconstructive surgery in the second half of life is traditional approach for CS. The issues of surgical stress response after reconstructive surgery for CS in children are still unclear. OBJECTIVE: To evaluate clinical and laboratory parameters in children undergoing traumatic reconstructive surgery for CS. MATERIAL AND METHODS: Inclusion criteria were CS, reconstructive surgery, age <24 months, no comorbidities and available laboratory diagnostic protocol including complete blood count, biochemical blood test with analysis of C-reactive protein, procalcitonin, ferritin and presepsin. The study included 32 patients (24 (75%) boys and 8 (25%) girls) aged 10.29±4.99 months after surgery between October 2021 and June 2022. Non-syndromic and syndromic forms of CS were observed in 25 (78.1%) and 7 (21.9%) cases, respectively. RESULTS: There were no infectious complications. We analyzed postoperative clinical data, fever, clinical and biochemical markers of inflammation. CONCLUSION: Early postoperative period after reconstructive surgery for CS in children is accompanied by significant increase of inflammatory markers (C-reactive protein, procalcitonin, ferritin). However, these findings do not indicate infectious complications. This is a manifestation of nonspecific systemic reaction. Severity of systemic inflammatory response syndrome with increase in acute phase proteins indicates highly traumatic reconstructive surgery for CS in children. Analysis of serum presepsin allows for differential diagnosis between infectious complication and uncomplicated course of early postoperative period.

Normative reference data for intracranial volume in children: The results of CT volumetry.

BACKGROUND: Intracranial volume (ICV) is an important indicator of the development of the brain and skull in children. At present, there is a lack of ICV growth standards based on large infant and children samples. Our aim was to assess the normal range of the ICV variation in Russian children using a modern automatic system for constructing the endocranial cavity (Endex) and to provide growth standards of the ICV for clinical practice. METHODS: High-resolution head CT scans were obtained from 673 apparently healthy children (380 boys and 293 girls) aged 0-17 years and transformed into the ICV estimates using the Endex software. The open-source software RefCurv utilizing R and the GAMLSS add-on package with the LMS method was then used for the construction of smooth centile growth references for ICV according to age and sex. RESULTS: We demonstrated that the ICVs estimates calculated using the Endex software are perfectly comparable with those obtained by a conventional technique (i.e. seed feeling). Sex-specific pediatric growth charts for ICV were constructed. CONCLUSIONS: This study makes available for use in clinical practice ICV growth charts for the age from 0 to 17 based on a sample of 673 high-resolution CT images.

[Difficulties in differential diagnosis of sagittal synostosis (scaphocephaly)]. / Trudnosti differentsial'noi diagnostiki sagittal'nogo sinostoza (skafotsefalii).

Craniosynostosis is characterized by congenital absence or premature closure of skull sutures. The most common form of craniosynostosis is synostosis of sagittal suture followed by scaphocephaly. There are some head deformities similar to scaphocephaly such as positional and constitutional dolichocephaly, etc. These patients have no sagittal suture synostosis. However, there are difficulties in differential diagnosis between these deformities and scaphocephaly. OBJECTIVE: To develop differential diagnostic criteria between dolichocephalic head deformities and true scaphocephaly following sagittal synostosis. MATERIAL AND METHODS: The study included 33 patients with dolichocephaly (25 (75.8%) boys and 8 (24.2%) girls) between December 2013 and August 2022. The inclusion criterion was available CT or ultrasound data confirming or excluding sagittal synostosis. Age of patients was 8.62±7.71 (1.77-36) months. We analyzed anamnestic, clinical and radiological data. Radiological data was compared with diagnostic findings in 20 patients with scaphocephaly. Both groups were comparable in age, gender and cranial index. RESULTS: We present clinical and radiological signs, as well as algorithm for differential diagnosis between scaphocephaly and dolichocephaly. CONCLUSION: There are objective difficulties in differential diagnosis between scaphocephaly following sagittal synostosis and dolichocephalic head deformities. In most cases, we cannot establish the cause of congenital forms of dolichocephaly. The most likely causes may be pre- and postnatal compressive and positional effects. Ultrasound of skull sutures is preferable for differential diagnosis between these abnormalities. Correction of dolichocephaly can be carried out according to aesthetic indications with individual cranial orthoses.

[Transsphenoid extirpation of pituitary adenomas in patients with McCune-Albright syndrome (two cases from practice and literature review)]. / Transsfenoidal'noe udalenie adenom gipofiza u patsientov s sindromom MakK'iuna-Olbraita (dva sluchaia iz praktiki i obzor literatury).

INTRODUCTION: McCune-Albright Syndrome is a rare genetic disease characterized by the formation of fibrous osteodysplasia foci of various localization, including the bones of skull base. Having a gross lesion of the main bone body and the simultaneous formation of the pituitary adenoma, its transnasal removal becomes very difficult. MATERIAL AND METHODS: Two clinical observations are presented where at patients with the classic manifestation of McCune-Albright syndrome we were able to successfully remove somatotropinomas with endoextrasellar growth. In both cases the presence of visual disorder was the indication for surgery. RESULTS: Despite pronounced deviations in the anatomy of the skull base bones in both cases we managed to access the Turkish saddle. Removal of the pituitary tumor did not differ from standard surgery. Improved vision after surgery was observed in two patients. Tumors were removed subtotally and clinical and biochemical remission of acromegaly was not achieved. In both cases, the tumors had morphological signs of atypia. Patients continued to receive therapy with somatostatin analogues and radiation. CONCLUSION: The possibility of performing transnasal surgery in this category of patients using specific instruments and intraoperative navigation is shown.

[Nasal schwannoma with skull base destruction in a child (a case report and literature review)]. / Nazal'naia shvannoma s destruktsiei osnovaniia cherepa u rebenka (sluchai iz praktiki i obzor literatury).

Schwannomas (neurinomas) are benign, encapsulated, and slow-growing neoplasms developing from any peripheral, cranial, or vegetative nerve that has the Schwann sheath. Schwannomas of the nasal cavity and paranasal sinuses in adults occur rarely, less than 4% of cases. The world literature lacks similar data for the pediatric age group because these tumors in children are unique and extremely rare. The most common clinical symptoms of sinonasal schwannoma are nasal breathing disorders, hyposmia or anosmia, and snoring at night. Other symptoms are associated with intranasal schwannoma invasion of neighboring organs and tissues and include exophthalmos, epiphora (lacrimation), headaches, and anesthesia in the facial area. In this article, we describe a rare case of diagnosis and successful surgical treatment of a child with intranasal schwannoma destructing the skull base.

[Modern diagnosis and treatment in children with congenital basal encephalocele]. / Sovremennye metody diagnostiki i lecheniia detei s vrozhdennymi bazal'nymi cherepno-mozgovymi gryzhami.

Basal encephalocele is a rare disease that predominantly occurs in children. Its most common symptoms include nasal liquorrhea, difficulty in nasal breathing, and deformity of the naso-orbital region. MATERIAL AND METHODS: The study group included 19 patients with basal encephalocele, aged 2 months to 18 years. Ten (59%) patients were operated on through a transnasal endoscopic approach; 3 (17.5%) patients were operated on through a transcranial approach; 4 (23.5%) patients were operated on using a combined approach: the patients underwent simultaneous elimination of a cranio-orbital region deformity using the basal transcranial approach as well as hernial sac resection and hernioplasty using the transnasal endoscopic approach. Two children had no surgery due to minimal symptoms and a lack of cerebrospinal fluid leak. RESULTS: Application of the algorithms for diagnosis and treatment of encephalocele, suggested by the authors, enabled making the timely diagnose, defining the optimal surgical tactics, and achieving good treatment results. CONCLUSION: A differentiated approach to the choice of a surgical technique for basal encephalocele, the use of auto-tissues for skull base reconstruction, intraoperative and postoperative lumbar drainage, and simultaneous elimination of deformity of the fronto-naso-orbital region enable avoiding complications and achieving good functional and aesthetic results.

[Endoscopic endonasal diagnosis and treatment of skull base meningoencephalocele]. / Éndoskopicheskaia éndonazal'naia diagnostika i lechenie meningoéntsefalotsele osnovaniia cherepa.

PURPOSE: The study purpose was to determine the successful outcome rate of endoscopic endonasal reconstruction of skull base CSF fistulas with removal of meningocele as well as analyze disease relapses. The second purpose of the study was to describe the treatment outcomes, depending on the type, location, and size of meningocele as well as on a technique used to reconstruct the skull base defect. MATERIAL AND METHODS: Three hundred and eleven patients diagnosed with cerebrospinal fluid (CSF) rhinorrhea who underwent surgery at the Burdenko Neurosurgical Institute in the period between 2007 and 2014 were retrospectively analyzed. Meningocele was detected in 141 (45.3%) of all patients with CSF rhinorrhea. The diagnosis was made before surgery and verified during endoscopic endonasal reconstructive closure of skull base defects. Therefore, the analysis group included all cases of meningocele and meningoencephalocele verified during surgery. The study analyzed the gender, age of surgery, follow-up period, comorbidities, use of lumbar drainage, etiology of the defect, localization, type of reconstructive material, complications, and disease relapses. We defined the efficacy of endoscopic endonasal meningocele resection with fistula reconstruction as the absence of signs of CSF rhinorrhea and a hernia sac based on the data of control CT and endoscopy performed in the late postoperative period. The follow-up period ranged from 1 month to 5 years. RESULTS: All patients underwent endoscopic endonasal reconstruction of the skull base defect. The treatment efficacy was 110 (78%) patients for primary surgery and 26 (84%) patients for repeated surgery. CONCLUSION: Endoscopic endonasal surgery is a safe, effective, and minimally invasive technique for treatment of meningocele and meningoencephalocele, which enables resection of a hernia sac and reconstruction of dura mater and skull base defects.

[A giant hyperostotic parasagittal meningioma in a child with neurofibromatosis type II (a case report and literature review)]. / Gigantskaya giperostoticheskaya parasagittal'naya meningioma u rebenka s neirofibromatozom II tipa (sluchai iz praktiki i obzor literatury).

Large parasagittal meningiomas, in particular hyperostotic ones, in children are rare and problematic in the differential diagnosis. The literature reports only single clinical cases related to this issue; opinions about the indications, surgical treatment options, and prognosis are contradictory. This paper presents a clinical case of a hyperostotic parasagittal meningioma with intra-extracranial growth in a 10-year-old boy with neurofibromatosis type II significantly worsening the prognosis. We discuss the epidemiological and clinical features of childhood meningiomas and issues of their diagnosis, treatment, and prognosis.

[Compression-distraction osteosynthesis in management of children with craniosynostoses].

Le Fort III midfacial distraction using internal and external devices is a generally accepted procedure for midfacial retrusion in patients with craniosynostoses. Many novel techniques for correction of cranial deformities in craniosynostoses are being introduced today. The authors described 14 consecutive cases of Le Fort III midfacial distraction using internal and external distraction devices. The paper discusses advantages and limitations of these methods.