Gregor Castrillon-Oberndorfer

Value of preoperative imaging in the diagnostics of isolated metopic suture synostosis: A risk–benefit analysis

Radiographic evaluation including plain radiographies and computed tomographic (CT) scans are considered as a necessary tool for diagnosis of craniosynostosis. As recently concerns about harmful effects of ionising radiation in children have been raised, some authors have suggested the use of magnetic resonance imaging (MRI) as a helpful alternative in preoperative imaging of patients with isolated metopic synostosis. Besides confirming the diagnosis of trigonocephaly, MRI is the superior technique for the evaluation of underlying brain anomalies. However, if the benefit of preoperative imaging justifies possible side effects is still discussed controversially. Hence, this study investigated the value of preoperative imaging for the diagnosis of isolated synostosis of the metopic suture compared to a sole clinical examination. In a series of 63 cases with isolated metopic craniosynostosis operated at the Department of Oral and Maxillofacial Surgery, 48 (76.2%) patients received additional radiography or MRI investigation, while in 15 (23.8%) patients the diagnosis was based on clinical examinations only. In all patients, diagnosis was confirmed intra-operatively by a fused metopic suture. CT scans with three-dimensional reconstruction (12.5%) or plain radiographs (39.6%) did not provide any additional benefit for the diagnosis or the surgical treatment. In 23 patients (47.9%), MRI showed the typical soft-tissue alterations like triangular brain deformation in the frontal area. Besides these findings, no brain or other underlying anomalies were diagnosed which had required any additional treatment. The incidence of underlying brain abnormalities in isolated metopic synostosis seemed not to be different from that of the general population. As the characteristic clinical manifestations were sufficient for an accurate diagnosis of isolated metopic synostosis, and with respect to the biological effects of ionising radiation and risks of sedation especially in infants, preoperative imaging should be reduced to a minimum.

Surgical outcome after using a modified technique of the pi-procedure for posterior sagittal suture closure

Fusion of the sagittal suture is the most prevalent form of craniosynostosis. Due to the variety of deformities of scaphocephaly depending on the location of the fused sagittal suture, the surgical procedure has to be adjusted to the individual case. In this study, 38 patients with a predominantly posterior sagittal suture closure were treated with a modified technique of the pi-procedure and the surgical outcome has been evaluated with respect to complications, morphological and aesthetic outcome. The improvement of the cephalic index in our series in the follow-up examination (mean 60.1 months) after surgery was significant (p<0.0001). According to the classification of Whitaker, 31 patients had a Class 1 outcome, with excellent surgical results. Aesthetic outcomes were excellent in 29, good in 5, and poor in 4 of cases, as judged by both the families and the craniofacial team. No severe complications have been observed. Posterior sagittal suture with marked occipital bulging can be successfully treated with this modified posterior procedure with a low complication rate, significant improvement of the cephalic index and a good aesthetic outcome. In all cases of sagittal synostosis, the operative procedure should be tailored to the nature and severity of the deformity.

GAPO syndrome associated with craniofacial vascular malformation

GAPO Syndrome Associated With Craniofacial Vascular Malformation Gregor Castrillon-Oberndorfer, Robin Seeberger, Claire Bacon, Michael Engel, Friedrich Ebinger, and Oliver Christian Thiele* Department of Oral and Maxillofacial Surgery, University Hospital Heidelberg, Heidelberg, Germany Molecular Division, Department of Human Genetics, University Hospital Heidelberg, Heidelberg, Germany Department of Pediatric Neurology, University Hospital Heidelberg, Heidelberg, Germany

The value of three-dimensional printing modelling for surgical correction of orbital hypertelorism

Orbital hypertelorism is defined as an increased distance between both medial and lateral sides of the orbits. Most common causes are frontonasal malformations, craniofacial fissures, encephalocele and a miscellaneous group of various syndromic or chromosomal disorders. Surgical correction of orbital hypertelorism is still challenging. The present report describes a case of severe orbital hypertelorism of an 11-year-old boy, where surgical correction was planned using three-dimensional printing modelling. This approach allowed reducing time of surgery, accurately planning the location of the osteotomies and precontouring the osteosynthesis material. Three-dimensional models are very helpful tools in planning complex craniofacial operative procedures.

Subtotal cranial vault remodelling in anterior sagittal suture closure: impact of age on surgical outcome

Isolated fusion of the sagittal suture is usually treated before 1 year of age, but some patients present at a later age. The aim of this study was to evaluate the impact of childrens age on the surgical outcome. The authors investigated 46 patients with isolated nonsyndromic sagittal craniosynostosis limited to the anterior two-thirds of the cranial vault. All patients underwent subtotal cranial vault remodelling, 36 patients (78.3%) before the age of 12 months (mean 8.92 months) and 10 patients after the age of 12 months (mean 15.77 months). Perioperative parameters and measurements of the cephalic index, preoperatively and postoperatively, were evaluated. All 46 patients showed improved head shape independent of their age. In patients younger than 12 months, mean cephalic indices improved from 65.99 to 74.49 (p<0.0001) and in patients older than 12 months from 66.38 to 74.38 (p<0.0004). There were no statistical differences in perioperative parameters including length of surgery, intraoperative blood loss and duration of hospital stay. In this study, patients showed no significant differences in surgical outcome that could have been related to the age at surgery. Surgical treatment should be performed early enough to benefit from the remodelling potential of the skull.

Solitary infantile myofibroma of the skull.

Infantile myofibromatosis is a proliferative disorder characterized by the development of single or multiple nodular lesions in the soft tissue, skeleton, and internal organs. These tumors can occur at any anatomic site, but in one third of the cases, the head and neck region is involved. Here, we report a case of an infantile myofibroma occurring in a 7-year-old girl presented as a solitary lesion in the head and neck area. The clinical heterogeneity and the misleading histopathologic appearances may render the diagnosis difficult. Usually, treatment of choice is surgical removal of the tumor; however, the low rate of recurrence and the possibility of spontaneous regression may lead to conservative surgery or therapeutic abstention.

The value of ultrasound-assisted pinned resorbable osteosynthesis for cranial vault remodelling in craniosynostosis

Resorbable osteosynthesis is a widespread tool in craniofacial surgery, however only a limited number of studies have focused on ultrasound-assisted pinned resorbable systems in the treatment of craniosynostosis. Thirty-eight children with various types of craniosynostosis including scaphocephaly, trigonocephaly, anterior and posterior plagiocephaly were treated using the Sonic Welding resorbable osteosynthesis system. All patients were evaluated for operation time, stability of the surgical results, rate of local infections and visibility or palpability of the osteosynthesis material in the follow-up ranging from 15 to 21 month. Mean operation time was not significantly higher compared to conventional osteosynthesis material and all remodelled cranial vaults showed immediate stability. Only one patient showed signs of an inflammatory skin reaction, which recovered spontaneously. The number of palpable or visible plates, respectively, increased during the first months with a maximum at 12 months (34 (89%) plates palpable, 26 (68%) plates visible). After this time point, the number decreased continuously until the end of the follow-up period at 21 months when 3 (20%) plates were palpable, 0 (0%) plates were visible). Ultrasound-assisted pinned resorbable systems seem to be a promising tool in craniofacial surgery providing a timesaving and stable osteosynthesis. An initial swelling of the plates during the first 12 months before the complete degradation might result in a palpable and visible bulge.

Bilateral coronal and sagittal synostosis in X-linked hypophosphatemic rickets: A case report

Craniosynostosis can be gene-linked, or caused by metabolic diseases, such as rickets, which results from a deficiency or impaired metabolism of vitamin D, magnesium, phosphorus or calcium leading to hypomineralization of the bone. X-linked dominant hypophosphatemic rickets (XLHR) is the most prevalent genetic type of hypophosphatemic rickets and is caused by germ line mutations in the PHEX-gene. In XLHR, only few case reports of craniosynostosis were described. Here, we present a clinical report of an 18 months old child with XLHR and bilateral coronal and sagittal synostosis who was treated by subtotal cranial vault remodelling with fronto-orbital advancement and right-angled Z-osteotomies. As a consequence of the childs diminished bone regeneration capacity, surgery that is performed after the age of 1 year requires more extensive craniectomy, multiple osteotomies and rigid fixation for calvarial vault remodelling to prevent extensive bone defects.

Long-term incidence of sensory disturbance in the forehead after fronto-orbital advancement in isolated nonsyndromic craniosynostosis

In the majority of the craniofacial literature, preservation of the supraorbital nerve during fronto-orbital advancement (FOA) is recommended. However, only a few studies have evaluated the incidence of sensory disturbance in the forehead after FOA during long-term follow-up. 57 children who underwent FOA in their first year of life because of isolated nonsyndromic craniosynostosis including trigonocephaly, anterior plagiocephaly or oxycephaly, were evaluated for sensory disturbance in the frontal region with a minimum follow-up of 27 months. An objective and repeatable measurement using the Semmes-Weinstein test was possible in 36 children older than 5 years at last follow-up. We revealed no sensory deficits in all patients, even in 3 patients, where one of the supraorbital nerves was transected during FOA. As previous reports have suggested a full recovery of sensation after transection of the supraorbital nerve during FOA I seen, the need to preserve the nerve has to be evaluated. However, as release of the nerve from the supraorbital rim is possible, we generally recommend preserving this structure, to minimize the risk of sensory deficits in the forehead region.