The Relationship of High Septal Deviation, the Depth of Olfactory Fossa, and Gera Angle: Is High Septal Deviation Associated With Any Anatomic Abnormalities in the Anterior Skull Base?

OBJECTIVE: To investigate the association of high septal deviation with the olfactory fossa depth and Gera angle. METHODS: Fifty-four computed tomography scans of patients with high septal deviation were evaluated. The olfactory fossa depth and Gera angle were measured. The values of the deviated and nondeviated sides were compared. RESULTS: No association between high septal deviation and the olfactory fossa depth and Gera angle was found. CONCLUSION: High septal deviation does not affect the olfactory fossa depth and Gera angle. In other words, the olfactory fossa depth and Gera angle have no association with the high septal deviation.

Asymmetric Meckel Cave Enlargement: A Potential Marker of PHACES Syndrome.

BACKGROUND AND PURPOSE: PHACES syndrome is a complex of morphologic abnormalities of unknown cause and includes posterior fossa abnormalities; head and neck infantile hemangiomas; arterial, cardiac, and eye anomalies; and sternal or abdominal wall defects. Accurate identification of the syndrome is important for optimal treatment. The purpose of this study was to investigate the incidence of asymmetric Meckel cave enlargement, a potential novel imaging marker, in a population of patients referred for evaluation of possible PHACES syndrome. MATERIALS AND METHODS: Eighty-five patients referred for neuroimaging evaluation of possible PHACES syndrome were identified and stratified on the basis of their ultimate clinical PHACES diagnosis categorization into PHACES, possible PHACES, or not PHACES. MR imaging studies were subsequently reviewed for the presence or absence of unilateral Meckel cave enlargement, with the reviewer blinded to the ultimate PHACES syndrome categorization. RESULTS: Twenty-five of 85 patients (29%) were ultimately categorized as having PHACES or possible PHACES according to consensus guidelines. Asymmetric Meckel cave enlargement was present in 76% (19/25) of these patients and in 82% (19/23) of only those patients with definite PHACES. This finding was present in none of the 60 patients determined not to have PHACES syndrome. In 7/19 patients (37%) with this finding, subtle MR imaging abnormalities consistent with PHACES were missed on the initial MR imaging interpretation. CONCLUSIONS: Asymmetric Meckel cave enlargement was a common feature of patients with PHACES in our cohort and may serve as a novel imaging marker. Increased awareness of this imaging feature has the potential to increase the diagnostic accuracy of PHACES.

Rhombencephalosynapsis: embryopathology and management strategies of associated neurosurgical conditions with a review of the literature.

OBJECT: Rhombencephalosynapsis (RS) is a rare congenital posterior fossa malformation characterized by dorsal fusion of the cerebellar hemispheres, hypogenesis or agenesis of the vermis, and fusion of the dentate nuclei and superior cerebellar peduncles. The objective of this institutional study is to review the clinical conditions associated with RS and analyze the varied biological profile of this unique condition. METHODS: The study data were collected retrospectively from the medical records of patients at Rainbow Babies and Children's Hospital. After required institutional review board approval, the authors obtained information regarding the cases of RS reviewed by the Departments of Radiology, Genetics, and/or Pediatric Neurology. Medical charts were systematically reviewed, and 9 patients were analyzed in detail. RESULTS: The authors describe 6 cases of RS and 3 cases of partial RS. This case series demonstrates an association between RS and symptomatic hydrocephalus (7 of 9 patients) and RS and Chiari malformation (5 of 9 patients). Patients with symptomatic hydrocephalus underwent endoscopic third ventriculostomy or ventriculoperitoneal shunt insertion. One of the patients with an associated Chiari malformation underwent foramen magnum decompression. CONCLUSIONS: The authors present a large case series of RS. Patients with RS often had hydrocephalus and/or a Chiari Type I or II hindbrain malformation. Neuroimaging findings of RS are presented along with hypotheses to explain the embryopathology of this unusual condition.

Treatment of anterior skull base defects by a transnasal endoscopic approach in children.

OBJECT: The object of this study was to assess the efficacy and complications of endoscopic management of anterior skull base defects. METHOD: The authors reviewed the medical records of 28 children (20 boys and 8 girls) undergoing endoscopic repair of anterior skull base defects in their tertiary referral center between 2001 and 2008; 18 cases were congenital and 10 cases posttraumatic. During the endoscopic procedure, rigid telescopes--2.7 or 4 mm in diameter, with 0° or 30° lenses--were used. In 23 patients the anterior skull base defect was sealed with fragments of middle turbinate (bone and mucosa). In the remaining 5 patients it was sealed with cartilage harvested from the nasal septum (3 cases) or from the auricle (2 cases), fibrin glue, and oxidized cellulose. A combined external and endoscopic approach was required in 3 cases because of the size and extensions of the encephalocele. Outcome was primarily assessed by means of clinical examination, nasal fibroscopy, and imaging. RESULTS: The mean duration of follow-up was 26.7 months (range 9-57 months). One patient treated by a combined approach died of meningitis 2 years after surgery. In the remaining 27 patients, there was no recurrence of CSF leak, meningitis, or encephalocele. An iatrogenic frontal or ethmoidal mucocele was observed in 4 cases. CONCLUSIONS: The endoscopic approach is a minimally invasive, safe, and efficient technique for removing nasal encephaloceles in children.

Nasal dermoid sinus cyst.

Midline congenital abnormalities of the skin may be related to subjacent visceral developmental defects. We report on three unrelated children presenting a small midline pit on the bridge of the nose from which a small tuft of hair was emerging. This presentation is the hallmark of a nasal dermoid sinus cyst. In one of the patients the sinus tract was connected to the anterior cerebral fossa and required neurosurgical management. Although midline cutaneous abnormalities may look trivial, dermatologists should keep a high index of suspicion for possible association with more severe underlying defect. The diagnosis of nasal dermoid sinus cyst requires appropriate imaging studies to determine the extension of the sinus tract thus allowing proper management.

Trigonocephaly: surgical considerations and long term evaluation.

Trigonocephaly accounts for approximately 10% of all craniosynostosis. Severe trigonocephaly results in a triangular-shaped forehead, superior-lateral orbital depression, hypotelorism, and compensatory occipital-parietal calvarial changes. Radiographic findings include ovoid orbits with parallel medial borders, thickened keel-shaped frontal bone, small ethmoid sinuses, and a short anterior cranial fossa with pitched sphenoid wings. Our experience with 50 infantile cases of severe nonsyndromic trigonocephaly patients treated from 1987 to 2005 is clinically reviewed to assess long-term growth based on a standardized operative technique. The average age of the patients at surgery was 6 months and the mean follow-up was 12 years. Our complication rate was 2%; the reoperative rate was 12%. The use of resorbable rigid plate fixation combined with alloplastic augmentation has improved the cosmetic outcome in patients treated since 1996 and reduced the reoperative rate.

Pseudoclosure of anterior fontanelle by wormian bone in isolated sagittal craniosynostosis.

AIMS AND OBJECTIVES: Although syndromic craniosynostosis is one of the causes for early closure of the anterior fontanelle, there is no literature on the incidence and causes of fontanelle closure in isolated single-suture craniosynostosis. The objective of this study was to review the incidence of fontanelle closure by a wormian bone in isolated, nonsyndromic sagittal craniosynostosis. MATERIALS AND METHODS: Intraoperative records of 100 consecutive children under 1 year of age, operated for isolated sagittal synostosis over a 14-year period (1987- 2000), were reviewed to identify the presence of a wormian bone closing the anterior fontanelle. RESULTS: The median age at surgery was 4.2 months with a range of 1.9-11.7 months. Intraoperatively, a wormian bone was seen replacing the anterior fontanelle in 4 cases giving an incidence of 4%. CONCLUSIONS: A wormian bone can occupy the anterior fontanelle in children with isolated sagittal craniosynostosis giving the appearance of a 'closed fontanelle'.