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Osteofibrous dysplasia (OFD) is a rare, noncancerous tumor that does not spread to other parts of the body. It typically develops during childhood. Many cases are treated conservatively with careful observation over time. So, what should we do for pediatric patient with cranial malformation caused by OFD?
Let’s learn from Ya, a four-year and six-month old patient’s case.
Three years ago, a hard immobile lump sized 1.5*1.0 cm was found on Ya’s right upper side of the skull (spanning parietal bone and frontal bone), but she did not feel pain or uncomfortable. She got her first diagnosis in the local hospital: the lump might be due to hematoma caused by injury, and careful observation was suggested.
As time went by, the lump grew up along with Ya. Unfortunately, two years ago, another lump was found near the previous one. With the ongoing “unexplained” lump, Ya stepped on her long journey for treatment.
Eventually, Ya was diagnosed with OFD after a series of examination.
OFD makes up less than 1% of all tumors that originate in bone. Almost all OFD tumors occur in the long middle part of the tibia, with rare reports of occurrences in the fibula, as well as the long bones in the arm (humerus, radius, and ulna). OFD tumor grows within the hard, cortical bone that makes up the outer portion of these bones.
Following physical examination, the surgeon decided to remove Ya's lesion and repair her skull defect in one stage operation.
To remove the diseased portion of the bone, first the area of lesion was identified. Then, the area of bone resection was planned on Ya’s 3D reconstructed CT image. With this information, a customized skull substitute made of polyetherether ketone (PEEK) was created for Ya.
To make sure the diseased portion of bone will be removed accurately as designed, Medprin, the manufacturer of Recranio™, suggested to use a customized 3D printing cutting guide for assistance.
Using the cutting guide and PEEK customized implant, neurosurgeon successfully completed the craniectomy and reconstruction surgery for Ya, as shown in the post-operative CT scan.
With “disappeared” lumps, Ya steps on another new journey towards new life.
The PEEK substitute chosen for Ya is Recranio™ Customized Cranio-Maxillofacial Repair System. Medprin team has extensive design experience that Recranio™ can be applied for maxillofacial defect, cranial defect as well as plastic surgery in different cases.
Feel free to contact us if you have some trouble in complicated cranioplasty cases.