Multi-Level Fibrotomy for Pediatric Patients with Cerebral Palsy: A Cohort Study

Muscle retraction in Cerebral Palsy (CP) often requires surgical treatment. Multilevel procedures (using open or percutaneous techniques) are commonly performed in the ambulant patient with CP. The necessity to find new surgical techniques, reduce postoperative discomfort, and accelerate the healing process and rehabilitation is mandatory for these patients. A retrospective cohort study with 189 pediatric patients with CP was performed. The multilevel gradual fibrotomy of Ulzibat was modified using an ophthalmic knife. No significant complications were reported using our technique. Opioid drugs were not necessary, and casting time was reduced at the first 24 h. A significant Range of Motion recovery was assessed post-operatory and maintained at the last follow-up. Mean days of hospitalization were 2.2. The mean follow-up was 39 months (6–64 months). The modified multilevel fibrotomy reduces postoperative pain with easier patient management, resulting in a faster discharge from the hospital. However, the retrospective nature and the lack of a control group of the present study did not allow the authors to report significant results. Further studies with longer follow-up are in progress to obtain more certain data that confirm our preliminary results.

Respiratory Dysfunction in Becker Muscular Dystrophy Patients: A Case Series and Autopsy Report

Background: Few studies have examined respiratory dysfunction in patients with Becker muscular dystrophy (BMD). Objective: This study aimed to examine the characteristics of respiratory dysfunction in patients with BMD. Methods: The present retrospective study assessed respiratory parameters of adult BMD patients using medical records and compared these parameters with various patient characteristics to identify correlations. BMD patients aged 17 years and older who had been diagnosed genetically and/or pathologically were included in the analysis. Results: Of the source population of 133 patients, respiratory function was assessed in 85. Two of these patients had no symptoms, and eight had died. Mean % forced vital capacity (% FVC) was 94.2+/–21.7% (median, 96.1%; range, 5.1–134.1%). In 16 (19%) of the 85 patients, % FVC was <80%. Of these, seven were non-ambulant. Age, ambulation, and cardiac function did not significantly differ between patients with or without respiratory dysfunction, whereas age at onset was significantly lower in patients with respiratory dysfunction (7.7+/–4.7 years vs. 14.4+/–11.9 years; p = 0.001). One non-ambulant patient was a continuous NPPV user, and one patient had been recommended NPPV use but refused. Autopsy of one patient revealed that the diaphragm and intercostal muscles were less affected than proximal skeletal muscles. Conclusion: BMD patients are at risk of developing respiratory dysfunction due to dystrophic changes in respiratory muscles. Respiratory function should be carefully and periodically monitored in these patients.

P574Accuracy of wrist-worn heart rate monitors for chronotropic assessment in atrial fibrillation

Introduction Wrist-worn fitness and heart rate (HR) monitors are increasingly popular. Previous studies in healthy participants with sinus rhythm (SR) have yielded variable results depending on HR, activity levels and device tested. A paucity of data exists on their accuracy in atrial fibrillation (AF) in ambulatory patients. Purpose We sought to assess the HR accuracy of 2 commercially available smart watches (Fitbit Charge HR [FB] and Apple Watch Series 3 [AW]) compared with Holter monitoring in an ambulant patient cohort. Methods Patients aged >18 years referred for 24-hour Holter monitoring were prospectively recruited. The Holter monitor was the criterion measure. Each patient was randomly allocated to either a FB or AW along with their Holter monitor. The study protocol was approved by the institutional review board. Statistical analysis: Pearson (r) correlation coefficients and Bland-Altman comparison with 95% limits of agreement (LoA) were evaluated to assess criterion validity and agreement between the smart watch and Holter ECG-HR. Bias was the calculated mean difference between the smart watch and ECG-HR. A ± 10-beat different between Holter-HR and SW-HR was used as a clinically relevant range to establish the accuracy of HR estimation by SW. Results Across all devices, 53,288 hear rate values were recorded from 32 patients. Twenty six patients were in persistent AF and six were in SR. Twelve patients wore the FB while 20 wore the AW. In the FB arm, nice patient were in persistent AF and three in SR. In the AW arm, persistent AF was the rhythm in seventeen and SR in three. Patients in SR demonstrated overall strong agreement compared to Holter monitoring (Mean Bias <1 beat, LoA −11 to 11 beats) and a correlation coefficient of 0.87 (p<0.001). In AF, both devices underestimated HR measurements (Overall Bias −9 beats, LoA −41 to 23, r=0.60, p<0.001). The AW had lower bias and narrower LoA compared to FB (−5 beats vs −13 beats, LoA −31 to 21 beats vs −50 to 22 beats). Using a ± 10-beat range against ECG-HR for clinical accuracy, both the AW and FB performed satisfactorily in SR with 95.2% of AW and 92.2% of FB HR readings considered valid. In AF, however, the AW-HR readings were within the ± 10-beat threshold in 76.5% of the time compared with only 56.1% of FB readings. Conclusion In ambulatory patients, smart watches were accurate in HR estimation when compared to Holter monitor in SR; however tended to underestimate HR in AF. Further improvements in device technology are needed before the widespread consumer adoption of this nascent technology for chronotropic assessment in arrhythmias.

Osteogenesis Imperfecta type II with the variant c.4237G>A (p.Asp1413Asn) in COL1A1 in a dichorionic, diamniotic twin pregnancy

We present the case of a 34-year-old woman with a prenatally diagnosed osteogenesis imperfecta type II of one fetus of a diamniotic-dichorionic twin pregnancy at 28 weeks and 2 days of gestation. The diagnosis was suspected after a routine ultrasound examination, specified by 3D-ultrasound and confirmed with moleculargenetic analyses of COL1A1 with DNA of fetal cells obtained after amniotic drainage. Since a premature rupture of membranes occurred a cesarean section was performed at 36 weeks of gestation. Both newborns received primary medical care by neonatologists. The affected child received further treatment initially stationary, later as an ambulant patient. Even though ultrasound is a powerful tool to identify clinical features of osteogenesis imperfecta, the condition can finally only be confirmed by collagen or DNA analyses. We discuss the possibilities and limits of prenatal diagnosis, treatment options as well as issues that are relevant for genetic counseling.

Edwin Smith Papyrus Case 8: a reappraisal

There are 3 translations of the Edwin Smith Papyrus: Breasted's (1930), Allen's (2005), and Sanchez and Meltzer's (2012). Case 8 is similarly presented in all 3 translations, although with increasing detail in the later works. The patient in Case 8 had a comminuted skull fracture under intact skin. There were palpable pulsations at the fracture site. There was deviation of the eye on the side of the injury and an ipsilateral spastic hemiparesis in an ambulant patient with bleeding from the nose and ear. Explanations of the paralysis have included a contrecoup lesion and compression of the contralateral cerebral peduncle against the tentorial incisura. Brainstem compression due to herniation is accompanied by loss of consciousness. Extensive contrecoup lesions consistent with the extent of the described paresis would also be associated with probable coma. The paralysis was spastic, but spasticity takes weeks to develop after trauma. Yet this patient's trauma was fresh, as there was still bleeding from the nose and the ear. It is suggested the paresis antedated the trauma, which was not its cause. The reasons for this suggestion are presented in this paper.

An Arm Elevating Dressing

An essential aspect of management of upper limb trauma (including burns), infection, and surgery, is elevation to reduce tissue swelling and to rest the injured part. Surgeons dealing with upper limb pathology each have their own method of elevating the arm. I wish to describe a type of arm elevating support which is used primarily for elevation of the upper limb postoperatively, but can be used as a sling in the ambulant patient.