Long-term vascular access for infants with moderate to severe osteogenesis imperfecta

Devin, Courtney L.; Sagalow, Emily S.; Penikis, Annalise; McGreal, Cristina M.; Bober, Michael B.; Berman, Loren

doi:10.1007/s00383-021-04975-2

Cited by 2 publications

(5 citation statements)

References 14 publications

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“…Again, the only other OI port‐a‐cath cohort in the literature reported similar postoperative complication rates. ( 29 ) It is reasonable to assume the same factors affecting port‐a‐cath longevity may correlate with this. An analysis of port‐a‐cath complication rate by OI type is not yet represented in the literature.…”

Section: Discussionmentioning

confidence: 99%

“…(15,16,19,(21)(22)(23)(24)(25)(26)(27)(28) However, at this time, only one other investigation examining port-a-cath outcomes in the OI population has been published to our knowledge. (29) Many studies consider port-a-cath longevity to be an outcome of interest. One multi-institution study compared outcomes for port-a-caths placed in children <10 kg or ≥10 kg.…”

Section: Discussionmentioning

confidence: 99%

“…( 15 , 16 , 19 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 ) However, at this time, only one other investigation examining port‐a‐cath outcomes in the OI population has been published to our knowledge. ( 29 )…”

Section: Discussionmentioning

confidence: 99%

“…( 15 , 16 , 19 , 21 , 23 , 24 , 26 , 27 , 28 ) However, there is a paucity of literature specific to OI patients. ( 29 ) The purpose of this study is to investigate the outcomes of port‐a‐cath placement in pediatric patients with OI for long‐term iv access at our center. We aim to facilitate evidence‐based decision‐making by surgeons and other medical providers caring for this population.…”

Section: Introductionmentioning

confidence: 99%

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Outcomes of Pediatric Osteogenesis Imperfecta Patients Requiring Port‐a‐Cath Placement for Long‐Term Vascular Access

et al. 2023

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Intravenous (iv) bisphosphonates are widely used to treat the skeletal manifestations of osteogenesis imperfecta (OI). Obtaining peripheral iv access in pediatric patients with OI is often difficult and traumatic. Although this may be mitigated with surgically placed iv ports (port-a-caths), surgeons may be hesitant to perform this procedure on these children because of the lack of safety data. This study aims to gain better insight into the safety and efficacy of port-a-cath use in this population and identify risk factors for port-acath complications. In the present study, we conducted a retrospective cohort analysis of patient characteristics and the incidence of port-a-cath-related complications in children with OI. Fifty-three port-a-caths were placed in 29 children (21 males and 8 females). Of the 29 patients, most are OI type III (n = 18), followed by type I (n = 4), type IV (n = 4), and type V (n = 3). At the time of initial port-acath placement, the median age was 52 months (10-191 months), and the median weight was 7.9 kg (5.1-41.1 kg). Most patients (n = 20) weighed less than 10 kg during initial placement. Weight correlated significantly with OI type (p = 0.048), sex (p = 0.03), and vessel used (p = 0.02). Median initial port-a-cath longevity was 43 months (1-113 months), and we found no significant difference in port-a-cath longevity between sexes, OI types, or vessels used. Most patients (n = 19) required multiple port-a-cath placements. There is a significant difference (p = 0.02) between the number of placements and OI type, with type IV having more than type III. Port-a-cath removal was almost always due to mechanical complications (n = 19) but also for infection (n = 1) and malposition (n = 1). Eight patients still had their initial port-a-caths in place at the conclusion of this study. These findings indicate that complications associated with port-a-cath placement are mild and can be used to safely deliver iv bisphosphonates to pediatric OI patients.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Outcomes of Pediatric Osteogenesis Imperfecta Patients Requiring Port‐a‐Cath Placement for Long‐Term Vascular Access

et al. 2023

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“…As well as pain and distress, there are specific risks related to holding infants with OI for insertion of a peripheral catheter such as fracture. Central venous access devices (CVADs) largely avoid these risks, although insertion itself carries risks and there is the risk of serious sepsis, albeit that this is probably low with proper care [ 11 •]. There have been cases of infants with OI experiencing frequent infections necessitating line removal, and it has been suggested that the impact of OI on soft tissues may predispose to problems with CVADs in small and severely affected infants.…”

Section: Early Postnatal Management Of the Infant With Severe Osteoge...mentioning

confidence: 99%

Early Life Management of Osteogenesis Imperfecta

Arundel,

Borg

2023

Curr Osteoporos Rep

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Purpose of Review This review aims to provide a review of the multidisciplinary management of infants with osteogenesis imperfecta (OI) during the first year of life, focusing on those with severe disease. The authors draw on published literature and direct experience of working in a large paediatric centre specialising in the management of rare bone disease. Recent Findings Whilst understanding of the pathophysiology of OI has grown over the past decade, the evidence base for management of infants remains limited. There has been a greater recognition of certain subjects of concern including pain management, cervical spine deformity, and neurocognitive development. Both international consensus guidelines on rehabilitation and disease-specific growth charts have been welcomed by clinical teams. Summary The early involvement of multidisciplinary specialist care is critical in ensuring optimal care for the infant with severe OI. A long-term perspective which focuses on the axial, craniofacial, and peripheral skeleton as well as on development more generally provides a framework which can guide the management of infants with severe OI.

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Long-term vascular access for infants with moderate to severe osteogenesis imperfecta

Cited by 2 publications

References 14 publications

Outcomes of Pediatric Osteogenesis Imperfecta Patients Requiring Port‐a‐Cath Placement for Long‐Term Vascular Access

Outcomes of Pediatric Osteogenesis Imperfecta Patients Requiring Port‐a‐Cath Placement for Long‐Term Vascular Access

Early Life Management of Osteogenesis Imperfecta

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