Investigation of umbilical venous vessels anatomy and diameters as a guideline for catheter placement in newborns.

Umbilical cord catheters (UCC) are important for the primary care of critically ill newborns. To analyze anatomical variations of the umbilical vein (UV) and its further course, we performed abdominal spiral-CT examinations on stillborns. The aim of the study was to explore the high incidence of mal-positioned UCCs and to improve their positioning. Eighteen stillborns were investigated (29.2 weeks ± 6.7 weeks (IQR)). CTs were performed using either air or contrast medium injection into the UV. We measured the diameter at the narrowest points of (i) the umbilical vein, (ii) the segmental portal vein, (iii) the left portal vein, (iv) the umbilical recess, and (v) the ductus venosus. The branching angles between (a) the umbilical vein and intrahepatic veins and (b) the ductus venosus and umbilical recess were measured. The diameter of the UV increases from 3.4 to 11 mm (median [IQR]:4.6 mm [4.2-6.9]: r2 = 0.64). The left portal vein has a larger diameter (3.6 mm [2.6-4.55]; r2 = 0.43) than the left segmental portal vein (2.3 mm [1.8-2.75]; r2 = 0.23). The diameter of the ductus venosus (2.5 mm [1.6-3.4]; r2 = 0.59) is half that of the umbilical recess (5.1 mm [3.3-6.2]; r2 = 0.43). The most obtuse angle is formed by the junction between the umbilical recess and ductus venosus (151° [133-159]; r2 = 0.001). The branch angle from the outgoing UV into the left portal vein is more obtuse (128° [123-144]; r2 = 0.0001) than that of the segmental portal vein (115° [105-119]; r2 = 0.0001). To avoid mal-positioning, our data suggest the use of a soft catheter. The UV and its extensions are wide enough to admit a 4 Fr. catheter without complete obstruction. Clin. Anat. 31:269-274, 2018. © 2017 Wiley Periodicals, Inc.

[Strabologic and neuro-ophthamologic aspects of childhood cancer]. / Strabologische und neuroophthalmologische Aspekte kindlicher Tumorerkrankungen.

Pediatric oncology has achieved major progress by continuous optimization of diagnostic and therapeutic approaches. In the interdisciplinary team, the ophthalmologist plays an important role. In the field of strabismus and neuro-ophthalmology clinical symptoms as strabismus, visual loss, anisocoria, visual field defects, and involuntary eye movements may be key indicators of childhood cancer. The appropriate diagnostic workup as well as the diagnostic and therapeutic steps during the course of the disease are important often requiring individually tailored approaches.

Introduction of a new standardized assessment score of spine morphology in osteogenesis imperfecta.

PURPOSE: Osteogenesis imperfecta (OI) is a rare hereditary disease leading to multiple bone deformities and fractures. In the absence of causal therapy, a symptomatic approach is based on treatment with bisphosphonates and physiotherapy. The clinical and radiological manifestations vary. Therefore, standardization and quantification for an objective comparison, especially during therapy, are required. In this paper, radiological changes of the spine are quantified according to their clinical relevance to define a scoring system that transfers the morphological changes into a single value representing the severity of the disease. MATERIALS AND METHODS: 268 lateral spine X-rays of 95 patients with OI (median age 5.6 years) were assessed. The findings were classified based on their clinical relevance. RESULTS: The three criteria, vertebral compression, thoracolumbar kyphosis and deformity type, were quantified in a new grading system. Based on this, a "severity classification" (1 to 5) was defined with implications for diagnostics and treatment. A mathematical formula that takes into account the three criteria and their correlations to clinical relevance, resulting in a "severity score", was developed. CONCLUSION: "Severity classification" and "severity score" introduce a new concept for a standardized evaluation of spine X-rays in patients with OI. For both scientific and routine purposes, it provides the user with a simple and easy-to-handle tool for assessing and comparing different stages of severity prior to and during therapy with detailed accuracy.

Reshaping of vertebrae during treatment with neridronate or pamidronate in children with osteogenesis imperfecta.

BACKGROUND/AIMS: Osteogenesis imperfecta (OI) is a hereditary disease causing increased bone fragility. Pamidronate (PAM), which has to be administered as a 3-day course according to the original protocol by Glorieux, is the most frequently used therapy. Other bisphosphonates like neridronate (NER), which can be infused during an outpatient visit, have also proven to be effective. This is the first analysis comparing the effect of PAM and NER using vertebral morphometry. METHODS: 28 patients with OI type III and IV were retrospectively analyzed by matched pairs. RESULTS: No differences were detected between patients treated with PAM or NER at the start of therapy: mean age 4.4 years (p = 0.730), mean height 86.8 cm/85.3 cm (p = 0.854), lumbar vertebral area 208.9 mm(2)/206.0 mm(2) (p = 0.555), and in all vertebral indices. After 1 year of treatment (mean 1.16 years; p = 0.854) both groups showed a significant increase in the vertebral area and improved vertebral indices. Again there were no differences between the groups in the vertebral area (p = 0.590). CONCLUSION: In this study there was no difference between patients treated with PAM or NER regarding vertebral morphometry during the first year of therapy. Because of the possibility of an outpatient setting, NER is convenient for these children.

[Standardized x-ray reports of the spine in osteogenesis imperfecta]. / Standard zur Befundung von Röntgenaufnahmen der Wirbelsäule bei Patienten mit Osteogenesis imperfecta.

PURPOSE: In this study we present a standard for radiological reports in patients with osteogenesis imperfecta (OI). The parameters can be used to describe X-rays of the lateral spine and give an impartial description of anatomical structures during a treatment with bisphosphonates. MATERIAL AND METHODS: In this retrospective analysis we included 48 patients with OI (31 female, 17 male [1.5 months - 19 years, mean age 9.0 years]). Lateral spine X-rays were analyzed by 2 radiologists before and during treatment. The parameters of the standardized report are degree of kyphoscoliosis, compression of single vertebrae, predominant type of vertebral deformities and extent of vertebral compression (score 1 - 5). RESULTS: There was no clear trend in the change of compression of single vertebrae. Some vertebrae with ventral compression showed an upgrowth to vertebrae with harmonic compression. Other deformities showed only marginal changes. In 26 patients the kyphoscoliosis improved (mean 10 degrees), in 36 patients the thoracic vertebrae compression increased and in 30 patients the vertebral height in the lumbar spine increased. The improvement of vertebral height was 1 point in the thoracic and lumbar spine. CONCLUSION: We propose a standardized report of X-rays of the lateral spine in patients with OI with quantitative and semiquantitative parameters using morphological criteria. These include compression of single vertebrae, degree of kyphoscoliosis, vertebral deformities and the severity of vertebral compression in the thoracic and lumbar spine.

[Piriform sinus fistula resulting in acute dyspnea in a newborn]. / Sinus-piriformis-Fistel als seltene Ursache von Dyspnoe bei einem Neugeborenen.

Piriform sinus fistulas are rare congenital deformities that may become symptomatic through cyst enlargement and inflammation. The fistula usually manifests as bacterial thyroiditis and is very uncommonly seen as a cause of acute dyspnea in newborns. We report the case of a newborn in whom a piriform sinus fistula led to acute breathing impairment. If the piriform sinus fistula and adherent cyst cannot be totally removed initially, the treatment must be followed later by a complete resection to prevent infection.

Successful renal transplantation in a child with thrombosis of the inferior vena cava and both iliac veins.

An 8-year-old girl who was born premature in the 24th gestational week suffered a septic venous thrombosis due to an indwelling central line during the early perinatal period. As a result the inferior vena cava including the intrahepatic segment and both iliac veins was obliterated. The right kidney was primarily dysplastic, and the left kidney developed a partial infarction. Renal function was compensated until the age of 6 years. Magnetic resonance angiography at that time showed a collateral system via the azygos vein. The venous pressure and its variation with breathing as measured invasively showed normal values. During pretransplant initiation of immunosuppressive therapy, the child developed cerebral convulsions after the third dose of cyclosporine. Therefore we utilized a regimen of rapamycin, mycophenolate mofetil, and steroids. The transplantation was performed using a living donor graft from the child's mother. The relatively long vein from the left kidney was used for anastomosis with a large presacral collateral vein. Twelve months after transplantation the kidney function is stable with a serum creatinine of 0.5 mg/dL. The recipient thrombosis of the caval and iliac veins is not a principal contraindication for successful renal transplantation. MR angiography and invasive pressure measurements facilitated evaluation of the collateral venous system. The living donation setting allowed the initiation of an immunosuppressive regimen that was tailored to the concomitant diseases of the child.