Minimally invasive endoscopically assisted remodelation (MEAR) of sagittal craniosynostosis: an alternative technique to open and endoscopic procedures with cranial orthosis time span reduction.

INTRODUCTION: Sagittal craniosynostosis represents the most frequent simplex skull suture pathology. There are currently several operative approaches to this defect. Minimally invasive techniques are preferred for young infants. Since July 2017, we have employed endoscopically assisted craniectomies followed by cranial orthosis. Gradually, we have developed our modified technique, the minimally invasive endoscopically assisted remodelation (MEAR). SURGICAL TECHNIQUE: MEAR is a combination of principles gained from classical cranial vault remodeling techniques and minimal invasive approaches. The long and wider lateral osteoectomies performed in the parietal and occipital bones along with loosening of the periosteum and dura adhesions at the lambdoid sutures lead to early correction of parieto-occipital dimensions. RESULTS: Thirty-one consecutive patients with scaphocephaly underwent MEAR. The median preoperative cephalic index of 67 units (P25:63.3, P75:70) was improved to a median postoperative cephalic index of 77 units (P25:75, P75: 81). Sufficient correction was achieved in all patients. Cranial orthosis was needed for a median of 1.5 months (P25:1, P75:2). We had no major surgical complications in this pilot series. CONCLUSIONS: With MEAR, we have achieved good cosmetic results. Duration of cranial orthosis was significantly shortened compared to conventional endoscopic-assisted procedures.

Sagittal craniosynostosis associated with midline cephalhematoma or vice versa, case report and a review of the literature.

INTRODUCTION: Sagittal craniosynostosis associated with midline cephalhematoma is a rare finding. Despite the controversy regarding its etiopathogenesis, this condition represents a clear indication for surgery. CASE REPORT: We present a case of a 10-week-old boy with an ossified midline vertex cephalhematoma and sagittal craniosynostosis. The child underwent a cephalhematoma excision and minimally invasive non-endoscopic narrow vertex craniectomy, with calvarial vault remodeling followed by 2 weeks use of a cranial orthosis. On 5-month follow-up, mesocephaly was achieved. CONCLUSION: Our case is well documented with native CT, 3D CT, intraoperative pictures, and 3D head scan imaging. We described our minimally invasive non-endoscopic technique that led to a rapid cranial vault remodeling with reduction of cranial orthosis need. A review of literature focused on surgical techniques is included.

[Dual X-ray absorptiometry (DEXA) in children with chronic diseases]. / Zastosowanie metody absorcjometrii promieniowania rentgenowskiego o podwójnej energii (DEXA) u dzieci z chorobami przewleklymi.

Dual energy X-ray absorptiometry (DEXA) can be used for the measurement of bone density at the level of lumbar spine, whole body scan, and also for the measurement of bone mass content (BMC), lean body mass (LBM), and fat body mass (FBM). Although this method has been originally developed for the diagnosis and monitoring of osteoporosis in adults, it is used in children with chronic diseases like chronic renal failure, chronic gastrointestinal and rheumatological diseases. However, children with chronic disease often demonstrate statural growth disturbances and decreased growth velocity. Therefore, their actual height does not correspond to the actual chronological age. Normal values of DEXA for a given age are based on data from children with normal height. Interpretation of DEXA regardless of the actual height, which is usually stunted, may lead to false conclusions and wrong therapeutic attitudes. Thus, when interpreting results obtained in such patients a few transformations and calculations should be done.

Lymphocyte subsets in renal transplant recipients during growth hormone therapy.

OBJECTIVE: To evaluate the effect of growth hormone treatment on growth, levels of insulin-like growth factor I (IGF-I) and lymphocyte subsets in immunosuppressed renal allograft recipients. METHODS: 18 children (aged 8.0-16.6 years) received growth hormone 1 IU/Kg/week daily for two years. Height, IGF-I levels and in 11/18 patients, lymphocyte subsets were evaluated serially. RESULTS: Standardized growth velocity increased from -1.0+1.5 to +1.2+2.2 and standardized IGF-I levels from +0.8+1.5 to +3.1+1.1 (1 year) and to +1.4+1.7 (2 years). The total lymphocyte count and the number of T lymphocytes (CD3+) decreased. The decrease was more marked in CD8+ (from 1.5+0.3 x10(9)/L to 0.9+0.3 x10(9)/L, 1 year and to 0.8+0.1 x10(9)/L, 2 years) compared to CD4+ (from 1.5+0.3 x10(9)/L to 1.0+0.2 x10(9)/L, 1 year and to 1.3+0.2 x10(9)/L, 2 years), resulting in an increment of the CD4+/CD8+ index. CONCLUSIONS: The differential effect of growth hormone treatment on CD4+ and CD8+ lymphocytes might be explained by different expression of the IGF-I receptor in these distinct subsets.

Body composition in children receiving recombinant human growth hormone after renal transplantation.

BACKGROUND: Recombinant human growth hormone (rhGH) is an anabolic hormone promoting protein synthesis in various tissues. Therefore, changes in body composition may be expected during rhGH treatment. METHODS: We studied changes in body composition during two years of rhGH treatment in 21 children after at least one year with a functioning renal transplant. The mean +/- SD age was 12.9+/-2.5 years at the start of rhGH therapy. A whole body, dual energy X-ray absorptiometry (DEXA) exam was performed before the initiation of rhGH therapy (T0), and was repeated at one and two year intervals after initiation of the therapy (T1 and T2, respectively). RESULTS: Lean body mass increased by a median of 0.48 SDS during the first year of treatment (P = 0.022), and the median increase during two years of therapy was 0.36 SDS (P = 0.061). On the contrary, the median fat body mass decreased by 2.17 SDS during the T0 to T1 period (P = 0.04) and by 1.99 SDS during the T0 to T2 period (P = 0.055). The index for fat body mass/lean body mass (FBM/LBM) decreased by a median of 5.3% during T0 to T1 (P < 0.001), however, a slower but still significant decrease by a median of 4.2% was noted at T2 (P < 0.05). Bone mass content did not change significantly during rhGH treatment. The medians in caloric and protein intakes were stable during rhGH treatment. CONCLUSION: A significant increase of lean body mass and a decrease of fat body mass was noted during rhGH therapy in children after renal transplantation.

Longitudinal follow-up of children after surgical treatment of scaphocephaly.

78 patients underwent surgery for scaphocephaly at the mean age of 1.2 (0.2-14.7) years in Faculty Hospital Motol in Prague. All operated children ("free bone flap" technique) have been longitudinally followed-up by the same neurosurgeon and by the same anthropologist. Non-invasive direct cephalometry has been an inevitable part of diagnostics, surgical treatment indication, postoperative follow-up and evaluation of the outcome of the treatment. Better cosmetic results in children operated before one year of age (the change of index cephalicus 6.5 units) in comparison with those treated later (3.7 units) is demonstrated (p = 0.02).

[Growth hormone therapy in prepubertal children with chronic renal insufficiency]. / Liecba rastovým hormónom u prepubertálnych detí v chronickej renálnej insuficiencii.

BACKGROUND: Growth retardation is a serious problem in children with chronic renal failure (CRF) despite normal endogenous growth hormone (GH) secretion. Intensive medical care and dialysis do not improve height velocity. The aim of the study was to evaluate the efficacy of GH therapy in children with growth retardation secondary to CRF. METHODS AND RESULTS: Biosynthetic growth hormone was given to 7 prepubertal children (five boys and 2 girls, age with a range 3.5-14.5 years) with severe growth retardation and CRF during 1 year. The dosage of GH was 1 IU/kg/per week. GH was given daily, sc. The patients had a full examination every 3 months. Six children completed the study. Height velocity improved with GH therapy in 5 children. Renal function deterioration was accelerated in 2 children. The serum alkaline phosphatase concentration increased and the serum calcium concentration decreased during GH treatment in all children. Glucose, thyroid gland hormone concentration and lipid concentrations remained constant. CONCLUSIONS: The study showed clearly that treatment with biosynthetic GH gave an impressive improvement of height velocity in growth retarded prepubertal children with CRF.

[Complete isosexual precocious puberty (true, LHRH-dependent)-- personal experience]. / Kompletní predcasná puberta isosexuální (pravá, závislá na LHRH)--nase zkusenosti.

The authors examined a group of 10 girls with true sexual precocity. The age of the patients was within the range of 11 months and seven and a half years. Height acceleration was on average almost one year, skeletal age acceleration was on average almost one and a half years; when BA was evaluated according to tables of visualization methods of the Motol Clinic and the TW2 and C method, a marked acceleration of BA was observed in the RUS method. To differentiate incomplete puberty-thelarche-in all girls the stimulation test, using Relefact LHRH (Hoechst), was performed, the diagnosis of true puberty was suggested by LH serum levels elevated above 12 IU/l. In incomplete puberty only FSH was elevated. In all patients oestrogenization of the vaginal epithelium, breast development (M2-3) and pubic hair was present, in three patients menarche. For treatment Decapeptyl-Depot (Ferring) was administered, the longest period of treatment was eight months. Therefore the change of HA and BA was not evaluated, but even during this brief period of treatment regression of pubertal sex signs occurred.

[Clinical and anthropologic aspects of form and size of the child's head]. / Klinische und anthropologische Aspekte der Form und Grösse des kindlichen Kopfes.

The author recommends the classic direct cephalometry for measurements in clinical anthropology during childhood. It is referred a trend of debrachycephalization in the Czech child population. The presented results prove a decreasing cephalic index from birth up to 3 years of life and a relationship between positioning of the child and the shape and size of the child's neurocranium.