scholarly journals Intravenous Sedation in Arnold-Chiari Malformation With Respiratory Failure

2019 ◽  
Vol 66 (1) ◽  
pp. 37-41 ◽  
Author(s):  
Yoshiki Shionoya ◽  
Eishi Nakamura ◽  
Takahiro Goi ◽  
Kiminari Nakamura ◽  
Katsuhisa Sunada
Keyword(s):  
Respiratory Failure ◽  
Respiratory Depression ◽  
Chiari Malformation ◽  
Dental Treatment ◽  
Anesthetic Management ◽  
Cranial Nerves ◽  
Intravenous Sedation ◽  
Type Ii ◽  
Neck Extension ◽  
Arnold Chiari Malformation

Type II Arnold-Chiari malformation (ACM) is an abnormality in which the cerebellum, pons, and medulla oblongata are displaced downward into the spinal cord. Type II ACM is often complicated by respiratory depression, sleep-disordered breathing, and deglutition disorder as a result of medullary dysfunction and impairment of the lower cranial nerves. Bending and stretching of the neck is restricted, and anesthetic management is problematic in patients with the disorder. We performed dental treatment twice under intravenous sedation in a patient with intellectual disability with type II ACM complicated by hypercapnic respiratory failure. Propofol was used for the first sedation procedure. Repeated bouts of respiratory depression occurred on that occasion, so the airway was managed manually by lifting the jaw. However, aspiration pneumonitis occurred postoperatively. A combination of dexmedetomidine and midazolam was used for sedation on the second occasion, and the intervention was completed uneventfully without any respiratory depression. Our experience with this patient highlights the need for selection of an agent for intravenous sedation that does not require neck extension and has minimal effect on respiration in patients with type II ACM, who are at high risk of respiratory depression and pulmonary aspiration.

Anesthetic management of parturients with Arnold Chiari malformation-I: a multicenter retrospective study

2019 ◽  
Vol 37 ◽  
pp. 52-56 ◽  
Author(s):  
T.R. Gruffi ◽  
F.M. Peralta ◽  
M.S. Thakkar ◽  
A. Arif ◽  
R.F. Anderson ◽  
...  
Keyword(s):  
Retrospective Study ◽  
Chiari Malformation ◽  
Anesthetic Management ◽  
Arnold Chiari Malformation

scholarly journals Anesthetic Management of a Patient With Hereditary Angioedema for Oral Surgery

2019 ◽  
Vol 66 (1) ◽  
pp. 30-32
Author(s):  
Rumiko Hosokawa ◽  
Masanori Tsukamoto ◽  
Saki Nagano ◽  
Takeshi Yokoyama
Keyword(s):  
Hereditary Angioedema ◽  
Tooth Extraction ◽  
Dental Treatment ◽  
Oral Surgery ◽  
Anesthetic Management ◽  
Treatment Options ◽  
Third Molar ◽  
Intravenous Sedation ◽  
C1 Inhibitor ◽  
Impacted Third Molar

Hereditary angioedema (HAE) is a rare genetic disease that results from deficiency or dysfunction of C1 inhibitor (C1-INH). This disease is characterized by sudden attacks of angioedema. When edema occurs in the pharynx or larynx, it can lead to serious airway compromise, including death. Physical and/or psychological stress can trigger an attack. Dental treatment, including tooth extraction, is also a recognized trigger. We report a case of a 20-year-old male with HAE who required impacted third molar extractions. C1-INH concentrate was administered 1 hour before surgery, which was completed under deep intravenous sedation. This report describes the anesthetic management of a patient with HAE and reviews treatment options and concerns.

Respiratory Failure in Arnold-Chiari Malformation

Respiration ◽  
2002 ◽  
Vol 69 (1) ◽  
pp. 86-86
Author(s):  
T.L. Hattingh ◽  
J.T. Butler ◽  
A.L. Biebuyck ◽  
C.T. Bolliger
Keyword(s):  
Respiratory Failure ◽  
Chiari Malformation ◽  
Arnold Chiari Malformation

Mesencephalic spur (beaking deformity of the tectum) in Arnold-Chiari malformation

1976 ◽  
Vol 45 (3) ◽  
pp. 315-320 ◽  
Author(s):  
Adelola Adeloye
Keyword(s):  
Chiari Malformation ◽  
Type Ii ◽  
Content Type ◽  
Arnold Chiari Malformation ◽  
Degrees Of Severity ◽  
Fine Print

✓ The beaking deformity of the tectum, referred to in this paper as the mesencephalic spur, is regularly present in the Type II variety of the Arnold-Chiari malformation in varying degrees of severity. When the mesencephalic spur is related to associated myelomeningocele, it was found that the more extensive the myelomeningocele, and the older the affected children, the more prominent was the mesencephalic spur.

scholarly journals Anesthetic Management of a Patient With Systemic Sclerosis and Microstomia

2020 ◽  
Vol 67 (1) ◽  
pp. 28-34
Author(s):  
Yoshiki Shionoya ◽  
Hatsuko Kamiga ◽  
Gentarou Tsujimoto ◽  
Eishi Nakamura ◽  
Kiminari Nakamura ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Respiratory Depression ◽  
Dental Treatment ◽  
Anesthetic Management ◽  
Moderate Sedation ◽  
Emergency Airway ◽  
Analgesic Effects ◽  
Anesthetic Agents ◽  
History Of ◽  
Skin Blood Vessels

Systemic sclerosis (SSc) is an autoimmune disease that can cause fibrosis in vital organs, often resulting in damage to the skin, blood vessels, gastrointestinal system, lungs, heart, and/or kidneys. Patients with SSc are also likely to develop microstomia, which can render dental treatment difficult and painful, thereby necessitating advanced anesthetic management. This is a case report of a 61-year-old woman with a history of SSc with microstomia, interstitial pneumonia, and gastroesophageal reflux disease in whom intravenous moderate sedation was performed using a combination of dexmedetomidine and ketamine for dental extractions. Both anesthetic agents are known to have analgesic effects while minimizing respiratory depression. Consequently, the increased discomfort caused by opening the patient's mouth and stretching the buccal mucosa was sufficiently managed, permitting an increase in maximum interincisal opening and completion of treatment without complications. Patients with SSc present with serious comorbidities that can negatively impact anesthetic management, so the implementation of an anesthetic plan that takes such risks into account is required. Furthermore, emergency airway management is likely to be difficult in patients with microstomia. For intravenous moderate sedation, combined use of dexmedetomidine and ketamine, which have analgesic effects while minimizing respiratory depression, may be particularly effective in patients with SSc and microstomia.

scholarly journals Type I Arnold-Chiari malformation with bronchiectasis, respiratory failure, and sleep disordered breathing: a case report

2013 ◽  
Vol 8 ◽  
Author(s):  
Raffaele Campisi ◽  
Nicola Ciancio ◽  
Laura Bivona ◽  
Annalisa Di Maria ◽  
Giuseppe Di Maria
Keyword(s):  
Respiratory Failure ◽  
Chiari Malformation ◽  
Sleep Disordered Breathing ◽  
Clinical Manifestations ◽  
Low Flow ◽  
Cervical Canal ◽  
Type I ◽  
Arnold Chiari Malformation ◽  
Pulmonary Manifestations ◽  
Disordered Breathing

Arnold Chiari Malformation (ACM) is defined as a condition where part of the cerebellar tissue herniates into the cervical canal toward the medulla and spinal cord resulting in a number of clinical manifestations. Type I ACM consists of variable displacement of the medulla throughout the formamen magnum into the cervical canal, with prominent cerebellar herniation. Type I ACM is characterized by symptoms related to the compression of craniovertebral junction, including ataxia, dysphagia, nistagmus, headache, dizziness, and sleep disordered breathing. We report a case of a life-long nonsmoker, 54 years old woman who presented these symptoms associated with bronchiectasis secondary to recurrent inhalation pneumonia, hypercapnic respiratory failure, and central sleep apnea (CSA). CSA was first unsuccessfully treated with nocturnal c-PAP. The subsequent treatment with low flow oxygen led to breathing pattern stabilization with resolution of CSA and related clinical symptoms during sleep. We suggest that in patients with type I ACM the presence of pulmonary manifestations aggravating other respiratory disturbances including sleep disordered breathing (SDB) should be actively investigated. The early diagnosis is desirable in order to avoid serious and/or poorly reversible damages.

scholarly journals Arnold-Chiari Malformation Type II and CYP1B1 Congenital Glaucoma: A Possible Association

2021 ◽  
Vol 2021 ◽  
pp. 1-3
Author(s):  
Shaikha Aldossari ◽  
Amani Al Bakri ◽  
Yumna Kamal
Keyword(s):  
Genetic Testing ◽  
Chiari Malformation ◽  
Primary Diagnosis ◽  
Congenital Glaucoma ◽  
Type Ii ◽  
Nasal Bridge ◽  
Arnold Chiari Malformation ◽  
Ocular Symptoms ◽  
Peripheral Hospital ◽  
Eye Examination

Background. We describe a case of an infant with Arnold-Chiari Malformation Type II (ACM-II) who was born with lumbosacral myelomeningocele, hydrocephalus, and primary congenital glaucoma (PCG) together with dysmorphic features (scaphocephaly, frontal bossing, hypotelorism, entropion, and flat nasal bridge), which according to our knowledge, is a combination that has yet to be described in literature. Primary diagnosis. A 2-year-old female who is known to have ACM-II was referred due to abnormal eye examination done in a peripheral hospital that suggested infantile glaucoma in both eyes. Findings. During her last physical exam (postop), she was vitally stable, conscious with good feeding. Ophthalmic assessment revealed buphthalmia, superior paracentral scar, deep anterior chambers (AC), and round pupils with positive red reflex, clear lens, and an IOP of 16, 14 mm Hg, respectively. Neurological exam showed paraparesis and moving upper extremities and has axial hypotonia. Genetic testing showed CYP1B1 gene mutation. Conclusion. The aim of reporting this case is to share the findings in this infant as it may be a new association. The main learning message here is that ACM-II patients may present with certain ocular symptoms, including glaucoma-related ones that may mimic neurological disorders. This report brings information that could alert general practitioners, neurologists, and neurosurgeons. A deeper understanding of this rare disorder may aid the diagnosis of cases with similar characteristic physical findings by referring them to an ophthalmology clinic for further evaluation. Case presentation. A 2-year-old female who is known to have Arnold Chiari Malformation Type II (ACM- II) was referred due to abnormal eye examination done in a peripheral hospital that suggested infantile glaucoma in both eyes. MRI at 3 months of age showed lumbosacral myelomeningocele and hydrocephalus. Genetic testing confirmed a CYP1B1 mutation. These combinations of symptoms were never described in the literature before.

scholarly journals Anesthetic Management of a Patient With Citrullinemia Type I During Dental Treatment

2021 ◽  
Vol 68 (3) ◽  
pp. 158-162
Author(s):  
Makiko Shibuya ◽  
Rie Iwamoto ◽  
Yukifumi Kimura ◽  
Nobuhito Kamekura DDS ◽  
Toshiaki Fujisawa
Keyword(s):  
Dental Treatment ◽  
Genetic Disorder ◽  
Anesthetic Management ◽  
Third Molar ◽  
Intravenous Sedation ◽  
Type I ◽  
Oral Medications ◽  
Gag Reflex ◽  
Serum Ammonia ◽  
Citrullinemia Type I

We report a case involving intravenous sedation for third molar extractions in a 32-year-old man with citrullinemia type I (CTLN1), a genetic disorder that affects the urea cycle. The patient was diagnosed with CTLN1 after he exhibited seizures soon after birth and was intellectually disabled because of persistent hyperammonemia, although his recent serum ammonia levels were fairly well controlled. We planned to minimize his preoperative fasting, continue his routine oral medications, and monitor his serum ammonia levels at least twice. Sedation with midazolam and a propofol infusion was planned to suppress his gag reflex and reduce protein hypercatabolism due to stress. Epinephrine-containing local anesthetics, which enhance protein catabolism, were avoided, replaced by plain lidocaine for blocks and prilocaine with felypressin for infiltration anesthesia. No significant elevation in ammonia levels was observed. In patients with CTLN1, sedation can be useful for preventing hyperammonemia. Patients who develop symptomatic hyperammonemia may require urgent/emergent treatment involving other medical specialists. Therefore, preoperative endocrinology consultation, perioperative monitoring of serum ammonia levels, and preemptively coordinating for appropriate care in the event hyperammonemia occurs should all be considered.

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