Long-term ophthalmological outcomes in patients with anterior plagiocephaly managed with a fronto-orbital advancement procedure.

BACKGROUND: Anterior plagiocephaly results from early fusion of a unilateral coronal cranial suture, which affects 1/10,000 infants at birth and can be associated with ophthalmological complications. The study aimed to evaluate the frequency of oculomotor disorders and refractive errors in children with non-syndromic anterior plagiocephaly. METHODS: Patients with anterior plagiocephaly undergoing surgery using a fronto-orbital advancement technique were included in this retrospective study between 2011 and 2017. The following data were collected: cycloplegic refraction in diopters (D), best-corrected visual acuity, manifest strabismus in primary position, ocular motility, head tilt in primary position, slit-lamp and fundus examination. The refractive errors were determined by autorefraction after cycloplegia with cyclopentolate. RESULTS: Among the sixteen patients included, hyperopia >2.5D was found in 10 patients, astigmatism >1D in 10 patients and anisometropia >1D in 7 patients. Astigmatism was contralateral to the synostosis in 7 cases. In total, significant refractive errors were found in 92.9%. Amblyopia was found in 33.3% patients. Strabismus was found in 11 patients, most frequently combined horizontal and vertical, of which 4 required strabismus surgery. The most frequent vertical deviation was ipsilateral hypertropia in 54.5%. We found superior oblique muscle limitation in 3 patients. Optic disc pallor was found in 2 patients. CONCLUSION: In the years following fronto-orbital advancement for non-syndromic anterior plagiocephaly, refractive errors and oculomotor disorders are frequently encountered, mainly contralateral astigmatism and strabismus with both horizontal deviation and ispilateral hypertropia.

Is reverse frontal cranioplasty eligible for the correction of both the forehead deformities and the intracranial hypertension in craniosynostosis? Comparison of the preoperative and postoperative intracranial volumes.

BACKGROUND: This study aimed to describe the surgical technique of reverse frontal cranioplasty (RFC), the aesthetical modification as well as the modification of intracranial volume (ICV) to assess its potential eligibility for the treatment of the intracranial hypertension (IH). MATERIALS AND METHODS: A retrospective monocentric study included the patients with a history of craniosynostosis with a forehead deformity who underwent RFC. A subjective outcome questionnaire (SOQ) was conducted with each patient or their parent to determine their level of satisfaction after RFC. Pre- and postoperative computed tomography (CT) scans were analyzed and compared to investigate the ICV change and fronto-nasal angle. RESULTS: Eleven patients were included in the study (6 female and 5 male) with a mean age of 10.9 years old (range 3-23 years) and an average follow-up of 4.5 years (1-11 years). All patients responded to the questionnaire with a high level of overall satisfaction (mean 9.1/10). The mean preoperative FNA was 134° ± 5° while the mean postoperative angle was 126.4° ± 6, corresponding to an average decrease of 7.6° (95% CI, 4.0-11.2°; p < 0.001). One patient with preoperative IH had a clinical recurrence during the follow-up. The ICV was significantly higher after the surgery (p < 0.0001), with an average increase of 3.2% (95% CI, 2.3-4.1%). CONCLUSION: Reverse (RFC) is a useful technique for the correction of the frontal malformations related to craniosynostosis, such as a sloping forehead and/or a lack of the supraorbital projection. Regarding the limited gain of intracranial volume (ICV), it should not be used alone as primary cranial expansion surgery for craniosynostosis with intracranial hypertension (IH).

Management of isolated and complex craniosynostosis residual deformities: What are the maxillofacial tools?

Treatment of craniosynostosis is complex and has greatly progressed in recent decades. From the early stages in the 1950s to today's most recent techniques, surgeons have faced the challenge of overcoming the deformities often caused by such invasive, complex surgeries. In the most recent years, new techniques have been developed that address surgical sequelae, including those of surgery performed in childhood. After a general introduction on craniosynostosis, the present paper describes the various types of deformity that may result from complex surgery and offers an overview of the various tools available to surgeons. An explanation of each indication and procedure is given.

Virtual 3D planning of osteotomies for craniosynostoses and complex craniofacial malformations.

INTRODUCTION AND OBJECTIVES: Computer-assisted surgery has been more and more widely used in craniofacial surgery in recent years. It is useful in many situations: stereolithographic models, surgical simulations of osteotomies and bone repositioning, and cutting guides and customized implants. The present paper argues that computer-assisted surgery is particularly useful in complex cases such as rare malformations, or to address the sequelae of previous surgeries. The various advantages of the technique are emphasized from a surgical and from a teaching standpoint. MATERIALS AND METHODS: Forty cases of various computer-assisted surgeries were analyzed, allowing a comprehensive review of outcomes in cases such as craniosynostosis, complex craniosynostosis, hypertelorism, craniosynostosis sequelae and cranio-facial and orbital trauma. RESULTS: Results were promising in all of the cases reviewed, except in a few cases for which computer-assisted surgery with cutting guides may not be necessary. In these specific cases, the pedagogical input is nevertheless interesting for residents and students. CONCLUSION: Computer-assisted surgery is revolutionizing the surgical approach to complex craniofacial malformations, as well as easing management of less complex ones. It is likely that in the years to come this technique will supersede previous ones. However, using this technique implies being willing to rely on a non-human device. We need to consider computer-assisted surgery as a tool that can change surgical practices. The surgeon can rely on it, yet nothing will replace his/her eye and experience. It is the combination of both this experience and the appropriate use of computer-assisted surgery that, ultimately, leads to successful surgery.

Addressing hypertelorism: Indications and techniques.

From its first descriptions in the early 1920s to today's use of cutting guides via computer-assisted surgery, surgical techniques to address hypertelorism have progressed. The present article aims to provide historical background and an overview of the development of surgical techniques during the late 20th century and in recent years. First, a historical overview identifies the most important surgical advances leading to the present state of the art. Each major surgical innovation is described, to explain the changes in this surgical field, according to the type of approach. Then, a precise description of today's most recent practices is provided, with particular emphasis on the spectacular advances deriving from computer-assisted surgery. A thorough description of the use of cutting guides throughout the surgical phase is given.

Posterior cranial vault distraction osteogenesis in craniofacial surgery: Technical note.

INTRODUCTION: Posterior cranial vault distraction is a treatment for intracranial hypertension in certain cases of craniosynostosis and faciocraniosynostosis. It allows the harmonization of the skull back and prevents turricephaly. This study presents the surgical technique. TECHNICAL NOTE: Posterior cranial vault distraction osteogenesis consists of a circular osteotomy of the skull back. Four distractors are placed on the cranial flap without detachment of the dura mater. The distraction is carried out over nearly 1 month with an objective of 20mm. The technique's effectiveness is judged on the correction of the skull back shape and the disappearance of clinical and radiological signs of intracranial hypertension. DISCUSSION: Posterior cranial vault distraction osteogenesis enables a large increase in cranial volume and a correction of the dysmorphic skull back. This easy surgery and the low rate of complications make this technique a main surgical approach in the management of faciocraniosynostosis.

Neurogenic stuttering: further corroboration of site of lesion.

Cases of neurogenic stuttering have been reported in the literature throughout the past century. Site(s) of lesion(s) have been documented usually by association of symptoms, EEG studies and occasionally by computed tomography (CT). The authors present three cases in which the site(s) of lesion(s) are documented by CT, magnetic resonance imaging (MRI) and SPECT. This study supports previous findings of neurogenic stuttering following either bilateral diffuse lesions or a unilateral lesion. In at least one case, the actual site of the lesion would have been missed without the use of SPECT testing. EEG studies were not helpful in identifying the site of the lesion.

[Therapeutic strategies in somatotroph adenomas with extrasellar extension: role of the medical approach, a consensus study of the French Acromegaly Registry]. / Stratégies thérapeutiques dans les adénomes somatotropes avec extension extrasellaire. Place du traitement médical. Etude consensus du Répetoire français de l'Acromégalie.

UNLABELLED: From the first 198 patient files included into the French Acromegaly Registry, we analyzed 68 patients harboring a somatotroph adenoma with extrasellar extension, after exclusion of those treated by stereotactic or conventional radiotherapy. In these patients (including 37 women), aged 21-77 yr. (45.7 +/- 13.3), GH concentrations ranged from 2-260 microg/L (38.6 +/- 44.3), and IGF I from 86-967% of age-matched upper limit of normal (303 +/- 164). Maximal diameter of the adenoma at MRI was 11-36.5 mm (20.4 +/- 6.5), with cavernous sinus involvement in 68% of cases. Three subgroups were defined: 20 patients treated by long-acting somatostatin analogs only (group M), for a mean duration of 3 yr. (extremes 1-7 yr.), 48 patients initially treated by transsphenoidal surgery (group C), of whom 21 were secondarily treated by long-acting somatostatin analogs (group CM) for a mean duration of 1.2 yr. (extremes 0.2-2 yr.). All 3 groups were not statistically different in terms of tumor mass and initial levels of GH and IGF-1. Patients from group M were significantly older than those of the other groups (p<0.05). RESULTS: 46% of patients from group C after surgery vs. 45% of patients from group M had a mean GH below 2.5 microg/L. Biochemical remission (GH<2.5 microg/L and normal IGF1 normal) was obtained in 31% of cases in group C, vs. 25% in group M. In this group, a decrease of the largest tumor diameter was observed in 10 patients (71.5%), ranging from 10-25% in 7 (50%) and exceeded 50% in 3 (21.5%). In group CM, the biochemical remission rate (42%) and final GH or IGF1 values were not significantly different from group M. In conclusion, these data suggest that surgery or long-acting somatostatin analogs have a comparable efficacy in terms of remission rates in somatotroph macroadenomas with extrasellar extensions.