Anesthetic management of a rare case of Shprintzen–Goldberg craniosynostosis syndrome

Gupta, Arun Kumar; Divekar, D. S.; Shah, Bhawini; Dhulkhed, Vithal K

doi:10.1111/j.1460-9592.2010.03340.x

Cited by 4 publications

(4 citation statements)

References 3 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Minimal subcutaneous fat, abdominal wall defects, cryptorchidism in males, and myopia are also characteristic findings. 1 The exact etiology and mode of inheritance are unclear, but mutations in fibrillin-1 gene and other genes have been reported. 1 The diagnosis of this syndrome is suspected in individuals with the clinical and radiological features.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Double-valve surgery in Shprintzen-Goldberg syndrome

Elmistekawy

Hudson

Williams

et al. 2013

Asian Cardiovasc Thorac Ann

View full text Add to dashboard Cite

We describe the challenging case of a 28-year-old Jehovah's Witness patient who presented with symptomatic mitral and tricuspid valve disease and Shprintzen-Goldberg syndrome. This is the first reported double-valve surgery in such a patient who, apart from chest deformity, had a small body size, severe lung disease, difficult airway and vascular access, and to add to the complexity, refused blood and blood product use. The patient underwent a successful mitral valve replacement and tricuspid valve repair through a right thoracotomy. Apart from atrial fibrillation, he had a smooth hospital course and was discharged home on postoperative day 9.

show abstract

Section: Introductionmentioning

confidence: 99%

“…1 The exact etiology and mode of inheritance are unclear, but mutations in fibrillin-1 gene and other genes have been reported. 1 The diagnosis of this syndrome is suspected in individuals with the clinical and radiological features. 2 Few cases have been reported since it was described in 1982.…”

Section: Introductionmentioning

confidence: 99%

Double-valve surgery in Shprintzen-Goldberg syndrome

Elmistekawy

Hudson

Williams

et al. 2013

Asian Cardiovasc Thorac Ann

View full text Add to dashboard Cite

show abstract

“…S ir —We read with interest the recent article by Gupta et al. (1). Even though the authors have mentioned in detail about the anesthetic implications related to marfanoid craniosynostosis syndrome, we have some concerns regarding the management of the child.…”

mentioning

confidence: 99%

Response to Anesthetic management of a rare case of Shprintzen-Goldberg craniosynostosis syndrome

Gupta

Bathla

Saini

et al. 2010

Pediatric Anesthesia

View full text Add to dashboard Cite

pulmonary hypertension secondary to left atrial hypertension to have developed. Frequently, such pulmonary hypertension gradually settles following decompression of the left heart by the LVAD, but right ventricular function may be impaired, and sudden changes in pulmonary vascular resistance are extremely poorly tolerated. An apparently stable patient receiving LVAD support alone may acutely decompensate because of inadvertent elevation in pulmonary vascular resistance. Effective analgesia and normocapnoea minimize the risk of increases in pulmonary vascular resistance. Inhaled nitric oxide and vasoactive therapy (usually epinephrine and milrinone) must be to hand. Right ventricular failure is the likeliest cause of decompensation in the LVAD patient during the course of anesthesia. It is not safe to assume that passive pulmonary blood flow will always suffice.Third, Pratap and Wilmhurst counsel against external cardiac compressions. We carried out external cardiac compressions for a prolonged period in a child with hypertrophic cardiomyopathy associated with a malignant dysrhythmia; the left ventricular apical cannula had become obstructed as the left ventricle decompressed following VAD insertion with subsequent loss of output. This child had effective cardiac compression until cardiopulmonary bypass was instituted and the cannula repositioned following resection of left ventricular muscle. The cannulae and anastomoses remained intact during the period of cardiac compressions.

show abstract

“…Cardiovascular complications are the most common cause of death. 3,4 The craniofacial alterations in patients with SGS make airway management difficult. A high incidence of "can't intubate, can't ventilate" situations make general anesthesia a high-risk procedure.…”

mentioning

confidence: 99%

Regional Anesthesia in a Patient With Shprintzen-Goldberg Syndrome: A Case Report

et al. 2023

View full text Add to dashboard Cite

Shprintzen–Goldberg syndrome (SGS) is a rare condition characterized by craniofacial, cardiac, and neurologic alterations that can challenge an anesthesiologist. There are a few case reports of pediatric patients with SGS receiving general anesthesia but none about other techniques. A patient with SGS and insufficient dura mater was once reported, and this has caused some anesthesiologists to be wary of regional anesthesia. However, the link between SGS and insufficient dura mater remains unclear. We report the case of a 19-year-old patient with SGS who safely underwent an open cholecystectomy with regional anesthesia.

show abstract

Anesthetic management of a rare case of Shprintzen–Goldberg craniosynostosis syndrome

Cited by 4 publications

References 3 publications

Double-valve surgery in Shprintzen-Goldberg syndrome

Double-valve surgery in Shprintzen-Goldberg syndrome

Response to Anesthetic management of a rare case of Shprintzen-Goldberg craniosynostosis syndrome

Regional Anesthesia in a Patient With Shprintzen-Goldberg Syndrome: A Case Report

Contact Info

Product

Resources

About