Langerhans cell histiocytosis (LCH) is rarely encountered in ophthalmology practice. It is a spectrum of disorder characterized by accumulation of histiocytes in various tissues. Diagnosis is challenging as it may simulate periorbital hematoma, rhabdomyosarcoma, and neuroblastoma. We report a case of unifocal LCH with orbital extension. Diagnosis was obtained from incisional biopsy, and histopathological examination showed numerous histiocytes with eosinophilic infiltrations. The presence of Langerhans cells was confirmed by the presence of protein S-100, CD1a, and/or Langerin (CD207). Treatment depends on the degree of organ involvement. She responded well to cytotoxic drugs and steroids. This emphasized that prompt tissue diagnosis is crucial for early management.
The aim of this study was to compare optic canal parameters of syndromic craniosynostosis patients with those of normal patients to visit the possibility of optic nerve impingement as a cause of visual impairment. Computed tomography scan images were processed using the Materialise Interactive Medical Image Control System (MIMICS) Research 21.0 software (Materialise NV, Leuven, Belgium). Eleven optic canal parameters were measured: 1) height of optic canal on the cranial side, 2) height of optic canal on the orbital side 3) length of the medial wall of the optic canal, 4) length of the lateral canal wall of the optic canal, 5) diameter of the optic canal at five points (Q1-Q4 and mid canal), and 6) area and perimeter of optic canal. These measurements were obtained for both the right and left optic canals. The study sample comprised four Crouzon syndrome, five Apert syndrome, and three Pfeiffer syndrome patients. The age of these syndromic craniosynostosis patients ranged from 2 to 63 months. The height of the optic canal on the orbital side (p = 0.041), diameter of the mid canal (p = 0.040), and diameter between the mid-canal and the cranial opening (Q3) (p = 0.079) for syndromic craniosynostosis patients were statistically narrower compared with those of normal patients when a significance level of 0.1 was considered. Scatter plots for the ages of patients versus the above parameters gave three separated clusters that suggested the arresting of optic canal development with age. The findings from this study demonstrated a narrowing of the optic canal in syndromic craniosynostosis patients, and indicate that optic canal anatomical characteristics may have an association with visual impairment among pediatric syndromic craniosynostosis patients.
Paediatric craniomaxillofacial (CMF) surgery requires a multidisciplinary team approach to ensure the optimal and holistic management of children with craniofacial deformities. The aim of this retrospective study was to analyse the complications following functional interventions among 34 CMF deformity patients in a single multidisciplinary craniofacial centre. Electronic data including patient demographic characteristics and clinical entry were analysed. Inclusion criteria were all paediatric patients with CMF deformities who underwent various functional interventions. A total of 64 interventions (48 intermediate and 16 definitive) were conducted. Based on the Sharma classification of complications, 20.3% were type I, 4.7% were type II, 1.6% were type III, and 4.7% were type IV . Most complications were type I, which included local infection (3.1%) and premature opening of tarsorrhaphy (3.1%). More serious complications (types III and IV) included temporary visual loss (1.6%) and intraoperative haemorrhage (1.6%). Although a low complication rate was observed in intermediate interventions, a higher complication rate was observed in more complex definitive interventions such as monobloc distraction osteogenesis. Although most complications were manageable, effective prevention remains mandatory, as serious complications may lead to permanent damage and mortality. This analysis highlights the importance of a multidisciplinary team approach to optimize the outcomes in CMF patient management.