U The authors describe a simple and fast technique for removal of deeply situated broken pedicle screw fragments. The screw fragments are removed using a fine-serrated, conically cored bit with a light rough inner surface that is mounted on a common slow-speed drill capable of rotating clockwise and counterclockwise. The cored head of the bit is pressed and engaged on the cut surface of the broken screw, and the drill is made to turn in counterclockwise rotation; this, by means of friction, causes the two surfaces to interlock, and consequently the broken screw fragment backs out. This technique was used to retrieve both broken titanium and stainless steel screws, and satisfying results were obtained. There were no complications associated with the application of the technique, and the pedicle as well as the screw hole were always preserved, offering, in the event that the vertebral instability continued, the possibility of applying a new screw of slightly larger diameter at the same screw hole. This technical application offers the opportunity of removing deeply situated screw fragments by using a simple technique while maintaining the vertebral pedicle and screw-hole integrity.

Clinical manifestations of COVID-19 are generally milder in children than adults. Severe acute respiratory distress syndrome is rarely reported, even though cardiovascular diseases, diabetes and chronic lung diseases have been associated with severe outcomes. 1 Children Other 36 (26%) Abbreviations: NICU, neonatal intensive care unit; OBI, brief intensive observation; PICU, paediatric intensive care unit; PED, paediatric emergency department. a Respiratory support: oxygen, high-flow nasal cannula, continuous positive airway pressure.

A 9-month-old infant was referred for evaluation of an extensive skin bruise of the hypogastric region that appeared after minor trauma (Figure 1). Abuse-related trauma was suspected; however, findings of the physical examination showed moderate hypotonia with normal reflexes, hyperelastic skin, severe kyphoscoliosis (Figure 2), pectus excavatum, and sternal retractions while breathing.The history was remarkable for parental consanguinity, severe hypotonia since birth, a right-side clubfoot, and sensorineural hearing loss. During the first months of life, enteral nutrition, due to poor suction capability, was required, and autonomous oral feeding started at the age of 6 months. Muscular tone progressively improved, and at the age of 7 months, the child achieved head control, and at 8 months, he could maintain the sitting position. Previous investigations had ruled out spinal muscular atrophy and Prader-Willi syndrome, whereas creatine-phosphokinase levels and general blood tests were all normal.Due to the history of easy bruising, self-improving hypotonia, early-onset progressive kyphoscoliosis, and hyperelastic skin, a genetic connective tissue disorder was suspected.Considering parental inbreeding, a single-nucleotide polymorphism array to detect homozygosity areas was performed. In an analysis of the homozygous areas, the FKBP14 gene, which encodes the protein family involved in the assembly of the extracellular matrix, 1-3 was sequenced, resulting in a homozygous mutation (c.362dup;

This study aimed to determine how common are specific red flags of life-threatening headache (LTH) among children with complaints of headache in the emergency department. A retrospective study was conducted over five years, including all patients aged < 18 years who presented for a headache to a Pediatric Emergency Department. We identified patients with life-threatening headaches and compared the recurrence of the main red flags (occipital location, vomit, nocturnal wake-up, presence of neurological signs, and family history of primary headache) to the remaining sample. Two-thousand-fifty-one children (51% female, 49% male) were included. Seven patients (0.3%) were diagnosed with a life-threatening headache. In the analysis of red flags, only the presence of abnormal neurological evaluation and vomiting was found to be more common in the LTH sample. No statistically significant difference was found for nocturnal awakening or occipital localization of pain. Urgent neuroradiological examinations were performed in 72 patients (3.5% of cases). The most common discharge diagnosis was infection-related headache (42.4%), followed by primary headaches (39.7%). This large retrospective study confirms the most recent literature suggesting that night awakenings and occipital pain are common symptoms also associated with not-LTH. Therefore, if isolated, they should not be considered red flags.

Background Ehlers-Danlos syndrome (EDS) represents a group of connective tissue disorders characterized by the fragility of the soft connective tissues resulting in widespread skin, ligament, joint, blood vessel and internal organ involvement. The clinical spectrum is highly variable in terms of clinical features, complications, severity, biochemical characteristics and genes mutations. The kyphoscoliotic type EDS (EDS VIA) is a rare variant of the disease, with an incidence of 1:100.000 live births. EDS VIA presents at birth as severe muscular hypotonia, early onset of progressive kyphoscoliosis, marked hyperelasticity and fragility of the skin with abnormal scarring, severe joint hypermobility, luxations and osteopenia without a tendency to fractures. This condition is due to a mutation in the PLOD1 gene, and less commonly in FKBP14 gene, which results in the erroneous development of collagen molecules with consequent mechanical instability of the affected tissue. Case presentation A female newborn, found to be floppy at birth, presented a remarkable physical examination for joint hypermobility, muscle weakness, hyperelastic skin, a slight curve of the spine, the absence of the inferior labial and lingual frenulum. Due to severe hypotonia, neuromuscular disorders such as Spinal Muscular Atrophy (SMA), genetic diseases such as Prader Willi syndrome (PWS), myopathies and connective tissue disorders were considered in the differential diagnosis. Targeted gene sequencing were performed for SMN1, PLOD1, FKBP14, COL6A1, COL6A2, COL6A3. The urinary lysyl and hydroxy-lysyl pyridinoline ratio was diagnostic before discovering the homozygous duplication in the PLOD1 gene, which confirmed kyphoscoliotic EDS diagnosis. Conclusion In front of a floppy infant, a large variety of disorders should be considered, including some connective diseases. The presence at the birth of kyphoscoliosis, associated with joint hypermobility and the absence of the lingual and lower lip frenulum, should suggest an EDS.