Surgical Management of Hypertelorism: The 3D Animated Technical Note.

The hypertelorism surgery is a complex procedure requiring a long learning curve. Even though the box osteotomy technique is well described in literature, its representation is generally based on texts and illustrations that do not really give a 3-dimensional or a dynamic point of views. The authors present a 3-dimensional animated video, Supplemental Digital Content 1, http://links.lww.com/SCS/E561 showing the craniofacial osteotomies and focusing on the critical points to correct hypertelorism.

3D reappraisal of trepanations at St. Cosme priory between the 12th and the 15th centuries, France.

OBJECTIVE: This study aims to place trepanation in a medieval therapeutic context by addressing its medical use in neurological disorders and by testing the existence of particular dietary care for the sick. MATERIALS: Six cases of trepanation found at the St. Cosme priory (La Riche, France) dated from the 12th-15th centuries. METHODS: Neurological health was explored by geometric morphometrics by comparing the six cases to 68 skulls and 67 endocraniums belonging to individuals from the same period and geographical area. Trepanned diet was investigated by carbon and nitrogen isotopes and compared to 49 individuals from the same site. RESULTS: The study of shapes suggests a possible pathological state for four subjects. The diet of the trepanned is not different from the rest of the population. CONCLUSIONS: The treatment of neurological disorders emerges as the main therapeutic motivation in the corpus, contrary to the reports from the ancient surgical treatises. A specific diet for the sick is not highlighted. SIGNIFICANCE: Geometric morphometrics is rarely used in paleopathology and the results suggest a potential of this type of analysis in the identification of pathological cases. The results on therapeutic motivations and diet do not fit the descriptions from ancient medical sources. LIMITATIONS: The study of forms did not lead to definitive diagnosis. The isotopic study does not allow us to appreciate all the aspects of the diet. SUGGESTIONS FOR FURTHER RESEARCH: A geometric morphometric study of the skulls and endocraniums of individuals with a known neurological condition would allow a better appreciation of the link between shapes and pathologies.

Surgical treatment of orbital hypertelorism: Historical evolution and development prospects.

Orbital hypertelorism (OR.H) is defined as an abnormal increase in the distance between the two orbits secondary to a skeletal anomaly, and it occurs in association with numerous congenital craniofacial malformations. Since its description by Greig in 1924, OR.H and the associated corrective procedures have captivated many surgeons. Here we present a discussion of the historical evolution of surgery for OR.H and highlight its future prospects. In the mid-twentieth century, only cover-up techniques simulating approximation of the eyes via an optical illusion were used, such as frontonasal skin resection, epicanthal fold surgery, and rhinoplasty. Subsequently, numerous surgeons attempted to correct the deformation using orbitonasal osteotomies via an extracranial approach. However, the outcomes were largely inadequate. Finally, in 1967, Tessier developed an efficient two-stage technique for OR.H correction via an intracranial approach; this technique revolutionized the management of OR.H. In 1970, Converse refined Tessier's procedure by performing a one-stage surgery that preserved olfaction. In 1976, Van Der Meulen developed the facial bipartition technique, which simultaneously corrected maxillary and craniofacial deformities. Box osteotomies and facial bipartition are still used for the correction of OR.H. Using the technological advancements introduced in the early 2000s, several surgeons have attempted to improve these techniques with the use of three-dimensional (3D) surgical planning, preoperative 3D printing, augmented reality-based surgical navigation, and computer assisted surgery using cutting guides. These modern-day practices are rapidly developing and are expected to refine and standardize the surgical correction of OR.H in the future.

Is It Safe To Use Frontofacial Monobloc Advancement and Cutting Guides on Adult Patients with Crouzon Syndrome? Introducing 2 Cases on 41- and 56-Year-Old Patients.

BACKGROUND: Patients with Crouzon syndrome are mainly treated in childhood by frontofacial monobloc advancement to avoid ophthalmic, neurologic, and maxillary complications. There is no reported case of surgery on adult patients with Crouzon syndrome in the literature. However, when faced with 2 cases of adult patients showing severe quality of life deterioration, our team decided to make an attempt using monobloc advancement technique. CASE DESCRIPTION: Two women aged 41 and 56 presented with untreated Crouzon syndrome and suffered from exorbitism, intracranial hypertension with chronic headaches, and hypoplastic maxillary. We decided to perform frontofacial monobloc advancement with internal distraction despite their advanced age using planned surgery and cutting guides. Distraction began 7-10 days after surgery and was of 15 mm. Distractors were taken off at 6 months. Surgical treatment corrected chronic headaches, ocular symptoms due to exorbitism, and hypoplastic maxillary. Patients were satisfied with the functional and aesthetic results. We noticed that this heavy surgery was more difficult to bear by these adults than children. CONCLUSIONS: Adults with craniofacial malformations have a lower self-esteem, lower quality of life, and less satisfaction with their facial look as compared with individuals without facial malformations. There is also an increased risk of psychosocial problems. Despite postoperative difficulties and minor complications, our 2 patients were satisfied with the functional and aesthetic results. This led to the conclusion that surgically addressing adult patients with Crouzon syndrome via monobloc advancement is appropriate and secure when performed by a trained team.

Use of cutting guides during craniosynostosis sequelae surgery: A comparative study between computer-assisted planning and post-operative results.

BACKGROUND: The authors compared results of craniofacial reconstruction surgery using cutting guides with planned reconstruction on patients presenting craniosynostosis surgery sequelae. METHODS: This is a retrospective study performed on seven patients who had undergone craniofacial reconstructive surgery in University Hospital Center of Tours (France) in 2015. Patients had long-term sequelae of trigonocephaly and anterior plagiocephaly surgery. 3D computer model was constructed, based on CT scans and used for surgical planning. Cutting guides were realized to use patient autologous bone. Post-operative 3D cranioplasty was superimposed to the 3D pre-operative to determine the minimal distance between each point of the post-operative flap and its pre-operative point corresponding. RESULTS: Mean of minimal distances calculated per patient ranged from 0.89 mm to 1.85 mm. The best result for percentage of points having the minimal distance inferior to 1.8 mm was 98.2 percent; the worst result was 55 percent. This value ranged from 77.5 to 98.2 percent for trigonocephaly cases. This value ranged from 55 to 77.5 percent for plagiocephaly cases. No significant difference was found between pre and post-operative areas and volumes of each flap, p = 0.12 and p = 0.19 respectively. CONCLUSION: Using cutting guides facilitates complex craniofacia reconstructions with patient autologous bone and obtains precise and reproducible results.

Long-Term Assessment of Suturectomy in Trigonocephaly and Anterior Plagiocephaly.

INTRODUCTION: Techniques for treating trigonocephaly and anterior plagiocephaly have evolved from the initial suturectomy technique to frontal cranioplasty. The authors evaluated the suturectomy technique in adolescent patients with these craniosynostoses, by carrying out a retrospective, long-term assessment at the end of growth. METHODS: Patients with anterior plagiocephaly or trigonocephaly were included. All had undergone coronal or metopic suturectomy with bilateral orbitofrontal bandeau resection between 1997 and 2005. The primary endpoint was the Whitaker classification. The secondary endpoints were anthropometric measurement, assessments of the bone defects on computed tomography scan, and the comments of patients and their relatives concerning the final skull outcome. Patients with anterior plagiocephaly also attended an ophthalmological consultation. RESULTS: Seventeen patients were included in the study: 8 with anterior plagiocephaly and 9 with trigonocephaly. Mean age at the time of surgery was 6.91 months. Mean age at the time of craniofacial consultation was 14 years. Fourteen patients (82%) were classified as Whitaker Class III and IV, corresponding to poor esthetic results and persistent bone defects. Seven patients requested further surgery. CONCLUSION: This study shows that suturectomy seems to yield poor esthetic results in the long term and patients should be followed up throughout adolescence to correct any craniofacial deformities.

Intracranial Pressure Monitoring Demonstrates that Cerebral Edema Is Not Correlated to Hyperammonemia in a Child with Ornithine Transcarbamylase Deficiency.

BACKGROUND: Ornithine transcarbamylase deficiency (OTCD) is an inborn error of urea cycle resulting in increased plasma levels of ammonia and glutamine and cerebral edema. However, the underlying mechanism of brain cytotoxicity remains controversial. Our objective is to present an unusual acute hyperammonemic crisis suggesting a key role of brain glutamine to mediate ammonia neurotoxicity and the interest of intracerebral pressure (ICP) monitoring to maintain adequate cerebral perfusion pressure and to prevent neurological damages. PATIENT: A 6-year-old boy with OTCD was admitted for an acute hyperammonemic encephalopathy following viral infection. At admission, he presented vomiting, confusion, lethargy (Glasgow scale 7/15), and bilateral papilledema, suggesting cerebral edema. Plasma ammonia level was slightly increased (194 µmol/L, rr 25-50 µmol/L), contrasting with the severity of neurological deterioration and with high levels of glutamine in plasma (1,949 µmol/L, rr 335-666 µmol/L) and the brain (10-fold increase on in vivo MR spectroscopy). The patient was placed on neuroprotective treatments and respiratory support. MAIN RESULTS: With a hypercaloric protein-free diet and nitrogen scavenger drugs, plasma levels of ammonia and glutamine rapidly decreased without neurological improvement. Continuous ICP monitoring showed repetitive peaks of pressure up to 60 mmHg in the first four days and was helpful to manage neuroprotective treatments. After several days, the patient progressively recovered without cognitive or motor disability. CONCLUSION: This case report highlights the discrepancy between the severity of neurological impairment, presumably related to high level of brain glutamine, and plasma levels of ammonia or glutamine in a child with acute hyperammonemic encephalopathy related to OTCD. In this situation, continuous ICP monitoring was helpful to manage neuroprotective treatments and prevent brain damages.

Frontofacial Monobloc Advancement With Simultaneous Frontal Cranioplasty in Adolescents With Residual Apert Syndrome Deformations.

INTRODUCTION: The treatment of faciocraniosynostosis has steadily evolved since the introduction of craniofacial surgery in the 1950s. The aim of this study is to demonstrate the positive results obtained by frontofacial monobloc advancement with simultaneous frontal cranioplasty in adolescents with adult facial bones and residual Apert syndrome deformations. MATERIALS AND METHODS: Three adolescents underwent surgery between September 1, 2010 and March 31, 2011. All had faciocraniosynostosis in the context of Apert syndrome and had undergone brain decompression surgery during the first year of life. However, they presented intracranial hypertension. The authors carried out frontofacial monobloc advancement with internal distraction and frontal cranioplasty. RESULTS: The mean frontal advancement was 13.8 mm. The mean maxillary advancement was 16.3 mm. About exorbitism, 2 patients had grade III and 1 had grade I before surgery. After monobloc advancement, 2 patients had no exorbitism and 1 had grade I. About dental occlusion, 3 patients had class III before surgery and were overcorrected in class II after advancement. DISCUSSION: Frontofacial monobloc advancement yields satisfactory functional and esthetic results in these cases. In conclusion, simultaneous frontofacial monobloc advancement and cranioplasty appears to be a promising technique for the treatment of adolescents with residual craniofacial deformations.

Orbitofrontal monobloc advancement for Crouzon syndrome.

INTRODUCTION: Usually, patients suffering from Crouzon syndrome have synostosis of coronal sutures, exophthalmia, hypertelorism, and hypoplasia of the middle third of face. Sometimes maxillary retrusion is absent, so these patients have class I or II relationship. In these cases, frontofacial monobloc advancement, which is the gold standard, increases the maxillo-mandibular dysmorphia. Therefore we propose orbitofrontal monobloc advancement minus dental arch, without splits of the pterygoid plates. CASE REPORT: A 12-year-old girl with Crouzon syndrome had intracranial hypertension, exophthalmia, a middle third retrusion and a class II occlusion. We achieved orbitofrontal monobloc advancement which is frontofacial monobloc advancement minus maxillary dental arch. Four distractors KLS Martin were used. After 20 days of distraction, the final advancement was 10.2 mm for cranial distractors and 10.5 mm at fronto-zygomatic. Distractors were removed after 8 months. DISCUSSION: We offer patients suffering from Crouzon syndrome with class I or II relationship a change from the classic frontofacial monobloc advancement leaving the maxillary dental arch in place, thus avoiding the worsening of the maxillo-mandibular dysmorphia related to surgery. The idea of associating Le Fort I osteotomy with a frontofacial monobloc advancement or Le Fort III osteotomy has already been described, mainly by Tessier and Obwegeser, however they probably achieved a complete Le Fort I osteotomy while we don't split the pterygoid plates. The patient's morphology and his surgical history determine the choice between Le Fort III and monobloc advancement. Dental occlusion needs to be taken into account for surgical indication.

Maxillary surgical seeding of a clival chordoma.

Seeding on surgical pathway is a rare form of clival chordoma treatment failure. We report the case of a 42-year-old male with a clival chondroid chordoma removed by a sublabial transsphenoidal approach followed by proton beam radiotherapy, who developed a maxillary bone recurrence 3 years after surgery.

The interperiosteo-dural concept applied to the perisellar compartment: a microanatomical and electron microscopic study.

OBJECT: The dura mater has 2 dural layers: the endosteal layer (outer layer), which is firmly attached to the bone, and the meningeal layer (inner layer), which directly covers the brain. These 2 dural layers join together in the middle temporal fossa or the convexity and separate into the orbital, lateral sellar compartment (LSC), or spinal epidural space to form the extradural neural axis compartment (EDNAC). The aim of this work was to anatomically verify the concept of the EDNAC by using electron microscopy. METHODS: The authors studied the cadaveric heads obtained from 13 adults. Ten of the specimens (or 20 perisellar areas) were injected with colored latex and fixed in formalin. They carefully removed each brain to allow a superior approach to the perisellar area. The 3 other specimens were studied by microscopic and ultrastructural methods to describe the EDNAC in the perisellar area. Special attention was paid to the dural layers surrounding the perisellar area. The authors studied the anatomy of the meningeal architecture of the LSC, the petroclival venous confluence, the orbit, and the trigeminal cave. After dissection, the authors took photographs of the dural layers with the aid of optical magnification. The 3 remaining heads, obtained from fresh cadavers, were prepared for electron microscopic study. RESULTS: The EDNAC is limited by the endosteal layer and the meningeal layer and contains fat and/or venous blood. The endosteal layer and meningeal layer were not identical on electron microscopy; this finding can be readily related to the histology of the meninges. CONCLUSIONS: In this study, the authors demonstrated the existence of the EDNAC concept in the perisellar area by using dissected cadaveric heads and verified the reality of the concept of the meningeal layer with electron microscopy. These findings clearly demonstrated the existence of the EDNAC, a notion that has generally been accepted but never demonstrated microscopically.

Optic radiations: a microsurgical anatomical study.

OBJECT: In this study, the authors used a fiber-dissection technique to describe the optic radiation. They focused on the morphological characteristics (length and breadth) of this structure, its course, and its relationships with neighboring fasciculi and the lateral ventricle. METHODS: The authors dissected 10 previously frozen, formalin-fixed human brains with the aid of an operating microscope by following the fiber dissection technique described by Klingler in 1960. Lateral, inferior, and medial approaches were made. The optic radiation, also known as the Gratiolet radiation, extended from the lateral geniculate body to the calcarine fissure. The average distance from the tip of the anterior Meyer loop to the calcarine sulcus was 105 mm (range 95-114 mm). The breadth of the optic radiations, one on each side of the brain, averaged 17 mm at the level of the inferior horn (range 15-18 mm). This tract could be divided into three main segments: the anterior or Meyer loop, the body, and the end of the optic radiation. Adjacent anatomical structures included: laterally, the inferior longitudinal fasciculi; medially, the tapetum of the corpus callosum; and the ependyma of the inferior horn of the lateral ventricle. CONCLUSIONS: Various practical surgical approaches are discussed. The knowledge gained by studying this particular anatomy will help prevent injury to the optic radiations during neurosurgery.