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.

[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.

[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.

[Risk factors for the formation of congenital hyperplasia of blood vessels (infantile hemangioma)]. / Faktory riska obrazovaniya vrozhdennoi giperplazii krovenosnykh sosudov (infantil'noi gemangiomy).

OBJECTIVE: The aim the study. To identify correlations between the development of blood vessel hyperplasia (GCS) and risk factors in pregnant women. To identify correlations between the development of blood vessel hyperplasia (GCS) and risk factors in pregnant women. MATERIALS AND METHODS: A selective retrospective analysis of 173 case histories and outpatient records of patients of the Clinic of Pediatric Maxillofacial Surgery and Dentistry of the Central Research Institute of Dentistry and Maxillofacial Surgery for 2011-2021 was carried out. The obstetric history of the mother, chronic diseases of mothers during pregnancy and bad habits were studied. The interrelation of the influencing unfavorable factor on the isolation, prevalence and vastness of foci of infantile hemangioma was determined. RESULTS AND DISCUSSION: There was no statistically significant relationship between the harmful habits of the mother and the number of lesions, as well as the isolation of the lesion of the mandibular-facial region (CHLO) and the prevalence of the process in the child. It was found that the prevalence of the process, the isolation of the lesion and the number of foci of CHLO did not have a reliable relationship with the complicated course of pregnancy in the mother. A reliable relationship was revealed between the number of lesions in the CHLO and chronic hypoxia, between the number of defects of the cardiovascular system and the prevalence of the process. But there was no reliable relationship between the number of CCC lesions and the number of lesions. 24 patients out of 173 were premature. In these patients, a statistical severity to the occurrence of GCS was revealed. There was no reliable relationship between the genetic predisposition on the line of both parents and the prevalence of the process, with the isolation of the lesion of CHLO and with the number of foci of CHLO lesions. CONCLUSION: Prematurity, chronic hypoxia, multiple malformations of the fetal cardiovascular system are risk factors for the development of vascular hyperplasia in children.

[Complex treatment of children with vascular head and neck malformations]. / Kompleksnoe lechenie detei s razlichnymi porazheniyami krovenosnykh sosudov golovy i shei.

AIM: The article analyzes the results of treatment of children affected with lesions of blood vessels of the head and neck. The research is aimed at developing and implementing minimally invasive techniques to treat such children. MATERIAL AND METHODS: The study group comprised 4416 patients with hyperplasia of blood vessels (the so-called infantile and congenital hemangiomas) and 397 patients with blood vessel malformations, examined and treated from 1991 to 2020. RESULTS: The paper summarizes developed indications and the results of implementation of effective minimally invasive methods for the treatment of children with vascular lesions: radiofrequency thermal ablation and pulsed dye laser treatment of children with severe forms of arterio-venous malformation in their maxillofacial area and. The treatment is currently the golden standard that provides stable aesthetic results. CONCLUSION: The most optimal and preferred methods of vascular lesions treatment proved to be minimally invasive techniques that include: radiofrequency thermal ablation, interstitial laser coagulation, pulse phototherapy, sclerotherapy and systemic treatment of beta-blockers.

Recurring gnathodiaphyseal dysplasia in two Russian brothers.

Two Russian brothers presented with recurring benign facial bone tumors and progressive limb bowing. The association of fibro-osseous jawbone lesions and long-bone bowing with cortical thickening suggested the diagnosis of gnathodiaphyseal dysplasia, in the absence of arguments in favor of fibrous dysplasia. Gnathodiaphyseal dysplasia is a rare autonomic dominant syndrome due to a mutation of the TMEM16E gene. The extreme and recurring phenotype of these two patients illustrates the variable expressivity of this disease. Differential diagnosis with other benign facial bone tumors is discussed.

Familial cherubism: the experience of the Moscow Central Institute for Stomatology and Maxillo-Facial Surgery.

The Central Institute for Stomatology and Maxillo-facial Surgery in Moscow is one the main centers for the treatment of pediatric head and neck tumors in the territory of the former Soviet Union. A series of 33 patients presenting with cherubism (24 children and 9 of their parents) is presented. The authors discuss the atypical clinical presentations, the relevant associated anomalies and the different treatment strategies. They report the first case of cherubism associated with gingival hypertrophy without neurological signs.