Manajemen Low Flow Anesthesia pada Pasien Kraniosinostosis dengan Hipertelorisme yang menjalani Four Box Wall Osteotomy, dan Eksisi Redundant Skin Fronto Nasal

Craniosynostosis is a case that diagnosed in the first year of life and can need surgical in young age. Craniosynostosis is a part of hypertelorism with incidence rate around 20%. Boy, 13 years old with hypertelorism had undergone multiple surgery for correction of hypertelorism before. Patient was planned to advance surgical correction of four box wall osteotomy which consist frontal part correction and part of it is release craniosynostosis in coronal suture. Risk of massive bleeding because patient already in teen age and length of surgery can be prolonged. Difficult airway management due to fascial deformity, use of low flow anesthesia to preserve temperature and reduce inhalation anesthesia usage, intraoperative fluid management in consideration maintenance and replacement blood loss and post operative pain management has become another consideration. Covid-19 as part of problems post operatively being known before extubation made the process is delayed. Massive bleeding needs massive transfusion protocol to speed up blood availability. Blood product such as PRC, FFP and TC should be available because coagulation factor is part of consideration. Anesthesia management in hypertelorism with four box wall osteotomy need good communication between anesthesiologist, neurosurgeon, plastic surgeon and pediatric intensivist to reduce perioperative risk including covid-19 in pandemic era.

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Clinical case of an infant with severe hemophilia A on emicizumab

Russian Journal of Pediatric Hematology and Oncology ◽

10.21682/2311-1267-2021-8-3-123-126 ◽

2021 ◽

Vol 8 (3) ◽

pp. 123-126

Author(s):

T. A. Andreeva ◽

I. A. Lavrichenko

Keyword(s):

Clinical Case ◽

Hemophilia A ◽

Coagulation Factor ◽

Primary Prophylaxis ◽

Intracranial Bleeding ◽

First Year ◽

Severe Hemophilia ◽

Traumatic Bleeding ◽

Post Traumatic ◽

First Year Of Life

Hemophilia A is an X-linked congenital bleeding disorder caused by a deficiency or absence of coagulation factor VIII. In children who are in the first year of life, bleeding into the head accounts for 12.8–17.7 % of cases, and up to 45.5 % of them are intracranial bleeding in contrast to adult patients, in whom joints are the most frequent localization of bleeding. The first 2 years of life are the most dangerous in relation tointracranial bleeding for a child with hemophilia and the provision of full preventive treatment is extremely important for this time.Aim of the study – present the first experience of using emicizumab as primary prophylaxis in a child of the first year of life with hemophilia A. A patient born in 2020 with a severe hemophilia A had two post-traumatic bleeding that required hospitalization and replacement therapy. We decided to start primary prophylaxis with emicizumab at the age of 10 months.There were not spontaneous bleedings during 8 months of emicizumab usage. Post-traumatic bleeding did not require hospitalization and additional therapy.The clinical case demonstrates that emicizumab is effective and safe in infant who have not previously received prophylactic treatment.

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The Development of Swallowing Respiratory Coordination

Perspectives on Swallowing and Swallowing Disorders (Dysphagia) ◽

10.1044/sasd18.1.19 ◽

2009 ◽

Vol 18 (1) ◽

pp. 19-24

Author(s):

Maggie-Lee Huckabee

Keyword(s):

Cortical Activation ◽

First Year ◽

Healthy Children ◽

Nasal Airflow ◽

Single Session ◽

Primary Finding ◽

Patterns Of Behavior ◽

Cortical Modulation ◽

First Year Of Life ◽

Swallowing Apnea

Abstract Research exists that evaluates the mechanics of swallowing respiratory coordination in healthy children and adults as well and individuals with swallowing impairment. The research program summarized in this article represents a systematic examination of swallowing respiratory coordination across the lifespan as a means of behaviorally investigating mechanisms of cortical modulation. Using time-locked recordings of submental surface electromyography, nasal airflow, and thyroid acoustics, three conditions of swallowing were evaluated in 20 adults in a single session and 10 infants in 10 sessions across the first year of life. The three swallowing conditions were selected to represent a continuum of volitional through nonvolitional swallowing control on the basis of a decreasing level of cortical activation. Our primary finding is that, across the lifespan, brainstem control strongly dictates the duration of swallowing apnea and is heavily involved in organizing the integration of swallowing and respiration, even in very early infancy. However, there is evidence that cortical modulation increases across the first 12 months of life to approximate more adult-like patterns of behavior. This modulation influences primarily conditions of volitional swallowing; sleep and naïve swallows appear to not be easily adapted by cortical regulation. Thus, it is attention, not arousal that engages cortical mechanisms.

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