Congenital Brevicollis (Klippel-Feil Syndrome) and Cardiovascular Anomalies

Morrison, Stanley G.

doi:10.1001/archpedi.1968.02100010616015

Cited by 23 publications

(13 citation statements)

References 26 publications

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“…Many radiological reports of KFA mention SBO in the abnormal cervical vertebrae. However, there are also reports of open neural tube defects, including cervical or occipital meningocele [Latto, 1942;Peters, 1962;Cohney, 1963;Morrison et al, 1968;Primrose, 1970;Jarvis and Sellars, 1974;Pizzutillo et al, 1994] and cervical meningomyelocele [Nora et al, 1961]. The sibling of a child with Type 1 KFA was reported with hydrocephalus, [Gunderson et al, 1967] although it was not known if cervical fusion was present.…”

Section: Discussionmentioning

confidence: 99%

Klippel–Feil anomaly and neural tube defects

McGaughran

2004

American J of Med Genetics Pt A

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Section: Discussionmentioning

confidence: 99%

Klippel–Feil anomaly and neural tube defects

McGaughran

2004

American J of Med Genetics Pt A

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“…Associated renal malforma tions occur in up to 64% of patients; unilateral renal agenesis is the most commonly reported anomaly, al though horseshoe kidneys, double collecting systems, and renal ectopia have all been described [77, 122. 177], Uni lateral pulmonary agenesis has also been reported [77,125]. Cardiac anomalies have been described in 4.5% of patients; most have a ventriculoseptal defect with or without other associated cardiac anomalies [118.…”

Section: Klippel-feil Syndrome and Iniencepludymentioning

confidence: 99%

The Embryogenesis of Complex Dysraphic Malformations: A Disorder of Gastrulation?

1992

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“…Second, cardiovascular congenital anomalies have been recognized in these patients, with a prevalence of 4.2%-14% [12,13]. Several lesions may occur, the commonest being ventricular septal defects, but also patent ductus arteriosus [12,14].…”

Section: Discussionmentioning

confidence: 99%

“…These patients may further develop cardiac failure preoperatively. Other lesions reported are mitral valve prolapse, bicuspid aortic valve, and coarctation of the aorta [13]. Preoperative evaluation to rule out such conditions should be mandatory.…”

Section: Discussionmentioning

confidence: 99%

Anesthetic management of a patient with Klippel-Feil syndrome

Mitra

Gombar

Sharma

et al. 2001

Journal of Anesthesia

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nasal septum with malocclusion of teeth. The thumb of his right hand was absent, and he had Sprengel's deformity (elevated and small scapula) (Fig. 2). He had restricted neck movements with minimal extension at the cervical vertebrae. He had fusion of the cervical vertebrae at the level of C2-3 and C5-6. The patient had a severe degree of kyphoscoliosis (Cobb angle was 40°) and could only lie down with two pillows below his occiput. On auscultation, he had minimally decreased air entry of the right lung. His higher function examination suggested poor mentation, and this was further corroborated by IQ testing, which revealed an IQ rating of 73. He had no muscle weakness or other neurological symptoms. His cardiovascular system was unaffected, with normal two-dimensional echocardiography. Ultrasound echography of his abdomen showed absence of the right kidney. He was unable to perform pulmonary function tests because of his cleft palate. However, hematological, coagulation, and biochemical investigations were unremarkable. His chest X-ray did not reveal any significant changes.The preoperative evaluation of airway (temporomandibular joint function) revealed a mouth opening of more than 40 mm. Mallampati classification was not possible because of the associated cleft palate. His extension of head was more than two-thirds restricted. The patient had dental malocclusion. However, radiological examination for estimation of mandibular space was not done.After informed consent from his parents, the child was premedicated with 75 mg ranitidine orally on the night before and on the morning of surgery and received 0.1 mg glycopyrrolate intramuscularly 1 h before operation.In the operating theater, he was connected to a multichannel monitor (Datex-AS-3 Light, Helsinki, Finland), and his heart rate, noninvasively measured blood pressure, electrocardiogram, arterial oxygen saturation (SpO 2 ), and end-tidal carbon dioxide (ETCO 2 ) were

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Congenital Brevicollis (Klippel-Feil Syndrome) and Cardiovascular Anomalies

Cited by 23 publications

References 26 publications

Klippel–Feil anomaly and neural tube defects

Klippel–Feil anomaly and neural tube defects

The Embryogenesis of Complex Dysraphic Malformations: A Disorder of Gastrulation?

Anesthetic management of a patient with Klippel-Feil syndrome

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