The Apert Hand—Angiographic Planning of a Single-Stage, 5-Digit Release for All Classes of Deformity

Harvey, Isaac; Brown, Scott M.; Ayres, Oliver; Proudman, Timothy W.

doi:10.1016/j.jhsa.2011.10.017

Cited by 18 publications

(8 citation statements)

References 16 publications

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“…A type 3 deformity consists of complex syndactyly of digits 1 through 5, with complex syndactyly of the thumb; it has been described as the rosebud hand. 16 In our case, there was a type 2 syndactyly. …”

Section: Dsjuogmentioning

confidence: 49%

Apert Syndrome in the Era of Prenatal Diagnosis

Stamatian¹,

Caracostea²,

Kovacs³

et al. 2014

Donald School Journal of Ultrasound in Obstetrics and Gynecology

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Apert syndrome is a rare autosomal dominant disorder charac terized by severe craniosynostosis, hypoplastic mediofacial structures and symmetric syndactyly. The syn dactyly involves both cutaneous and osseous structures of the upper and lower limbs. Apart from skeletal anomalies, affected fetuses may often present central nervous system malformations and various visceral malformations (congenital heart defects, genitourinary ano malies, choanal stenosis, tracheal abnormalities, dia phrag matic hernia and omphalocele). Mental retardation is frequently encountered, as are a large variety of craniofacial and neurocognitive phenotypes. The incidence of Apert syndrome is approximately one in 65,000 newborns, representing 4.5% of all cranio synostosiscases.ThefirstcasesofApertsyndromewere diagnosed by ultrasound and fetoscopy in the third trimester of pregnancy. With the introduction of threedimensional (3D) ultrasonography,identificationoffetalchangescharacteristicof Apert syndrome is possible in the second trimester of pregnancy. DiagnosisconfirmationrequiresfetalDNAanalysisbyfibroblast growthfactorreceptor2geneamplification(FGFR2)followed bysequencinganddigestionwithrestrictionenzymes.Recently, noninvasive prenatal diagnosis was reported using free fetal DNA analysis with cell-free fetal DNA (cffDNA) in maternal plasma by Polymerase Chain Reaction (PCR) assay and enzymatic digestion with restriction enzymes. As the syndrome hasrarelybeenreportedinRomania,wepresentthecaseofa fetus diagnosed with Apert syndrome in the second trimester of pregnancy. Early ultrasound detection of suggestive signs for this syndrome (craniosynostosis, syndactyly) was followed by confirmationofthediagnosisbyprenatalfetalDNAanalysis.

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Section: Dsjuogmentioning

confidence: 49%

Apert Syndrome in the Era of Prenatal Diagnosis

Stamatian¹,

Caracostea²,

Kovacs³

et al. 2014

Donald School Journal of Ultrasound in Obstetrics and Gynecology

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“…3-D MDCT allows syndactyly release of adjacent fingers in a single-stage procedure (Harvey et al., 2012). Even central digits can be released at the same time as border digits, and the number of operations needed in these patients can be decreased (Fearon, 2003; Zucker et al., 1991), which is particularly helpful in children with associated malformations requiring surgical procedures for correction (Piza-Katzer et al., 2008).…”

Section: Discussionmentioning

confidence: 99%

“…Usually, the patient is supine with the arms positioned above the head in order to keep the radiation dose at the maximum possible distance from the abdominal organs. Also, using only the arterial phase is sufficient in surgical planning, thus further decreasing the radiation dose (Harvey et al., 2012). In our experience, 3-D CT angiography is a fast and comprehensive technique for analysing soft tissues and neurovascular structures in children…”

Section: Discussionmentioning

confidence: 99%

Three-dimensional CT angiography for surgical planning in congenital hand malformations: a case series presentation

Morandi

Rieger

Baur

et al. 2020

J Hand Surg Eur Vol

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Between January 2000 and December 2019, three-dimensional computer tomographic (CT) angiography was used in a total of 140 hands (116 patients, mean age 6.8 years) with congenital hand malformation to assess the vascular and bony structures. Analysis showed overall satisfactory three-dimensional CT images for operative planning, including detailed abnormal vascular patterns and bony malformations. Among the 116 patients, six patients with typical findings of a few malformations are reported in detail. Pitfalls in interpretation of the images and the use of three-dimensional CT angiography in surgical planning are discussed. We conclude that three-dimensional CT angiography is useful for preoperative planning of complex congenital hand malformations. Level of evidence: IV

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“…Piza-Katzer et al cited the benefits of greater visualization of the bone and flexor tendon anatomy, in addition to the need to delineate the vascular anatomy for concomitant release of the second and third webspaces in their second stage. We reduced the radiation exposure below that described previously by Harvey et al (2012) by lowering the voltage to 80 kVp. Our dose length product values of 75–110 mGy-cm, using our final protocol, were lower than previous reported values of 450 mGy-cm.…”

Section: Discussionmentioning

confidence: 99%

“…A novel technique for single-stage, 5-digit release of the Apert hand has recently been described, using CT angiography to delineate the vascular anatomy of the hand and guide surgical planning (Harvey et al, 2012). Five patients underwent bilateral releases using this approach and there were no complications related to digital ischemia.…”

Section: Introductionmentioning

confidence: 99%