Tesis

Parent’s Opinion-Based Treatment Strategy and The Outcome Analysis for Apert Syndrome: an In-Depth Interview = Strategi dan Analisis Luaran Tatalaksana Sindrom Apert Berbasis Opini Orang Tua: Sebuah Wawancara Mendalam

This descriptive evaluation and in-depth interview aims to evaluate the treatment management for Apert syndrome patients in our center and take parents’ opinion into account on the treatment outcome and treatment orders. Three Apert patients that have undergone craniofacial surgery(s) and hand surgery(s) during January 2012-November 2018 were observed and recorded during their play to observe the hand function, craniofacial appearance and general appearance. The video recordings was then shown to another group consisted of 12 parents of Apert children to be evaluated by a guided in-depth interviews. Parents were also interviewed regarding the treatment decision for their children born with Apert. Nine out of 12 parents prioritized the hand surgery over the craniofacial surgery while the 3 parents prioritized the craniofacial surgery over the hand surgery. Ten subjects did not plan to have feet syndactyly release for their children unless it interferes with the walking. Only 2 subjects considered to have mid-face advancement surgery. Eight subjects found the children’s facial appearance in the videos are satisfactory but the midface still have some place for reconstruction. All parents agree that five-digits hand looked very well and the hand functions are acceptable. The clinodactyly was not a priority to be reconstructed for most of the parents.

Keywords: acrocephalosyndactylia; craniosynostoses; exophthalmos; malocclusion; syndactyly


Studi deskriptif dengan wawancara mendalam ini bertujuan untuk mengevaluasi
tatalaksana pasien sindrom Apert dengan memperhitungkan pendapat orang tua pasien
sindrom Apert untuk evaluasi luaran dan urutan intervensi bedah. Tiga pasien dengan
sindrom Apert yang sudah menjalani pembedahan kraniofasial dan tangan pada periode
Januari 2012-November 2018 diobservasi pada saat mereka sedang bermain untuk
dinilai fungsi tangan, tampilan kraniofasial dan juga penampilan secara umum. Video
rekaman kemudian dipertontonkan terhadap kelompok subjek kedua yang merupakan
12 orang tua pasien Apert untuk dievaluasi melalui wawancara mendalam. Wawancara
meliputi penentuan keputusan tatalaksana pada pasien Apert. Sebanyak 9 dari 12 subjek
menyatakan lebih memrioritaskan pembedahan tangan daripada pembedahan
kraniofasial, sementara 3 subjek mengutamakan pembedahan kraniofasial dilakukan
sebelum pembedahan tangan. Sepuluh subjek tidak berencana melakukan operasi
pembedahan sindaktili pada kaki anak mereka kecuali bila mengganggu proses berjalan.
Hanya 2 subjek mempertimbangkan pembedahan mid-face advancement. Delapan
subjek berpendapat penampilan wajah pasien di video cukup memuaskan namun area
sepertiga tengah wajah masih mungkin membutuhkan koreksi. Semua orang tua setuju
bahwa tangan dengan lima jari tampak sangat baik dan memiliki fungsi baik.
Rekonstruksi klinodaktili bukan merupakan hal yang diprioritaskan bagi sebagian besar
orang tua.

Kata kunci: acrocephalosyndactylia; craniosynostoses; exophthalmos; malocclusion;
syndactyly