Transillumination of an Anterior Fontanelle Dermoid Cyst.

Dermoid cysts are slowly growing benign lesions of ectodermal tissue that often occur in the anterior fontanelle. Clinicians often rely on a negative transillumination test to begin the process of correctly diagnosing a dermoid cyst. However, here the authors present a case of a 7-month-old girl who presents with a transilluminating dermoid cyst.

Pediatric ventriculoperitoneal shunt: a comparative study between anterior fontanel ultrasound-guided versus conventional cranial end insertion.

PURPOSE: Ventriculoperitoneal (V-P) shunt is one of the most common neurosurgical procedures in pediatrics for the treatment of hydrocephalus. Shunt failure is one of the common mechanical complications which lead to major morbidities. This study aims to compare between cranial part insertions of the V-P shunts guided by trans-anterior fontanel ultrasound versus conventional insertion. METHODS: A prospective comparative randomized study was conducted on 60 pediatric patients aged ≤ 2 years who suffered hydrocephalus and allocated into 2 groups. In the first group (n = 30), the cranial parts of the ventriculoperitoneal shunts were inserted guided by trans-anterior fontanel ultrasound, and in the second group (n = 30), the insertions were by the conventional method. The follow-up duration of the patients was 3 months. RESULTS: Proximal part obstruction of the V-P shunt was found in 3 cases of the conventional group during follow-up with statistical insignificance (p = 0.237) while adequate proximal part location recorded statistical significance (p = 0.0005) in favor of ultrasound-guided group. CONCLUSION: The use of the anterior fontanel ultrasound guide during ventriculoperitoneal shunt insertion is a feasible, safe, and effective technique for the placement of ventricular catheters in pediatric patients with a patent anterior fontanel.

Improvement in ventriculomegaly following cervicomedullary decompressive surgery in children with achondroplasia and foramen magnum stenosis.

The role of cervicomedullary decompression (CMD) in the care of hydrocephalic achondroplastic children who present with simultaneous foramen magnum stenosis is not well understood. We sought to determine the percentage of symptomatic achondroplastic children with foramen magnum stenosis who had stabilization or improvement in ventriculomegaly following CMD. The authors retrospectively reviewed the records of pediatric patients at Cedars-Sinai Medical Center with achondroplasia and signs of progressive ventriculomegaly who underwent CMD for symptomatic foramen magnum stenosis between the years 2000 and 2018. Clinical outcomes included changes in fontanelle characteristics, head circumference (HC) percentile, and incidence of ventriculoperitoneal (VP) shunting. Radiographic outcomes measured included changes in Evans ratio. We excluded individuals who were shunted before CMD from our study. Sixteen children presented with symptomatic foramen magnum stenosis and full anterior fontanelle or jump in the HC percentiles. Two children underwent placement of a VP shunt before decompressive surgery and were excluded from further analysis. Of the remaining 14 children who underwent CMD, 13 (93%) showed softening or flattening of their fontanelles post-operatively. Ten of these 14 children had both pre- and post-operative HC percentile records available, with 8 showing increasing HC percentiles before surgery. Seven of those eight children (88%) showed a deceleration or stabilization of HC growth velocity following decompression of the foramen magnum. Among 10 children with available pre- and post-operative brain imaging, ventricular size improved in 5 (50%), stabilized in 2 (20%), and slightly increased in 3 (30%) children after decompression. Two children (14%) required a shunt despite decompression of the foramen magnum. A significant proportion of children with concomitant signs of raised intracranial pressure or findings of progressive ventriculomegaly and foramen magnum stenosis may have improvement or stabilization of these findings following CMD. CMD may decrease the need for VP shunting and its associated complications in the select group of hydrocephalic children with achondroplasia presenting with symptomatic foramen magnum stenosis.

Estimation of elevated intracranial pressure in infants with hydroce-phalus by using transcranial Doppler velocimetry with fontanel compression.

For infants with acute progressive hydrocephalus, invasive drainage of cerebrospinal fluid (CSF) is performed until a ventriculo-peritoneal shunt can be inserted. Surrogate markers of intracranial pressure (ICP) may help optimise the timing of invasive procedures. To assess whether RI with/without fontanel compression helps distinguish between infants with normal (<5 cmH2O), mild (5-11 cmH2O), and moderate (>11 cmH2O) ICP elevation, 74 ICP measures before/after CSF removal and 148 related Doppler measures of the middle cerebral artery were assessed. Higher RI was associated with fontanel compression, elevated ICP, and their interaction (all p < 0.001). Without compression, differences in RI were observed between normal and moderate (p < 0.001) and between mild and moderate ICP elevation (p = 0.033). With compression, differences in RI were observed for all pairwise comparisons among normal, mild, and moderate ICP elevation (all p < 0.001). Without compression, areas under the receiver-operating characteristic curve for prediction of mild and moderate ICP elevation were 0.664 (95% confidence interval (CI), 0.538-0.791; p = 0.020) and 0.727 (95% CI, 0.582-0.872; p = 0.004), respectively, which improved to 0.806 (95% CI, 0.703-0.910; p < 0.001) and 0.814 (95% CI, 0.707-0.921; p < 0.001), respectively, with compression. RI with fontanel compression provides improved discrimination of infants with absent, mild, and moderate ICP elevation.

Accessory maxillary ostium repair using middle turbinate flap: a case series of 116 patients with chronic rhinosinusitis.

BACKGROUND: The ostiomeatal complex (OMC), comprising a small natural maxillary sinus ostium and narrow infundibulum, transmits the air diffusion into the antrum and mucociliary transport from the antrum, and is considered a key area in chronic rhinosinusitis (CRS). Thin membranous anterior and posterior fontanelle areas below the OMC can rupture forming a perforation, accessory maxillary ostium (AMO), that increases antral airflow changing the anatomy and function of the sinus. The purpose of this study was to report the first case series of CRS patients who had undergone repair of fontanelle defects aiming to reconstruct normal structures. METHODS: Between 2011 and 2017, a total of 157 perforations were diagnosed and repaired in 121 of 525 consecutive endoscopic sinus operations performed by the author. Defects were 3 mm to 4 mm in size (range, 1 mm to 7 mm). A flap cut from the undersurface of the middle turbinate was used. In total, 101 patients received concurrent balloon catheter dilation (BCD), while 15 patients had only an AMO repair. The mean endoscopic follow-up time was 16 weeks (range, 1 to 188 weeks). RESULTS: Overall, 101 perforations were closed, 21 open, and 17 partially open. A history of earlier endoscopic sinus surgery (ESS) or BCD surgery, presence of nasal polyposis or whether the repair was made without simultaneous BCD did not influence the closure rate. Postoperative febrile sinusitis occurred in 26 patients. CONCLUSION: Repair of AMO is in theory a beneficial and technically feasible office procedure with only transient side effects. Three out of 4 perforations were closed after repair.

Extent of surgery in endoscopic transsphenoidal skull base approaches and the effects on sinonasal morbidity.

BACKGROUND: Endoscopic transsphenoidal skull base surgery (ETSS) is now considered the criterion standard approach for resection of pituitary adenomas and other midline anterior skull base lesions. Normal sinonasal structures are resected during ETSS, which raises concerns for nasal morbidity and patient-based outcome. OBJECTIVE: To perform a surgical outcome assessment by examining whether the extent of ETSS approaches affected patient-specific sinonasal quality of life as measured by the 22-item Sino-Nasal Outcome Test (SNOT-22). METHODS: A single-center prospective cohort study of patients operated on by the same skull base team between 2012 and 2016. Patients with completed pre- and postoperative SNOT-22 were included. The primary outcome was SNOT-22 scores at preoperative, 0-1 month, 2-4 months, >5 months follow-up. Age, sex, tumor pathology, surgical procedure, and intraoperative cerebral spinal fluid leak repair were also obtained. RESULTS: Of the 249 ETSS performed, 148 patients (59%) had at least one completed SNOT-22; 45 (18%) met the inclusion criteria. Sinonasal quality of life based on SNOT-22 at the 0-1-month follow-up was significantly worse than the presurgical levels (p < 0.05). However, there was a return of SNOT-22 scores to preoperative levels at 2-4 months (p > 0.05), which was sustained at >5 months (p > 0.05). Factors such as the extent of ETSS, a previous nasal surgery, repair of an intraoperative cerebral spinal fluid leak, and the tumor pathology did not affect SNOT-22 scores at any follow-up intervals (p > 0.05). CONCLUSION: Sinonasal quality of life worsened after ETSS at 0-1 month follow-up but returned to preoperative levels at 2-4 months and remained at postoperative levels >5 months. Analysis of these data will allow us to educate our patients that the anticipated nasal morbidity after ETSS is usually only transient and should be expected to recover to preoperative levels.

A comprehensive review of the anterior fontanelle: embryology, anatomy, and clinical considerations.

PURPOSE: Fontanelles are a regular feature of infant development in which two segments of bone remain separated, leaving an area of fibrous membrane or a "soft spot" that acts to accommodate growth of the brain without compression by the skull. Of the six fontanelles in the human skull, the anterior fontanelle, located between the frontal and parietal bones, serves as an important anatomical diagnostic tool in the assessment of impairments of the skull and brain and allows access to the brain and ventricles in the infant. METHODS: Using a standard database search, we conducted a review of the anterior fontanelle, including its embryology, anatomy, pathology, and related surgical implications. CONCLUSIONS: The diagnostic value of the anterior fontanelle, through observation of its shape, size, and palpability, makes the area of significant clinical value. It is important that clinicians are aware of the features and associated pathologies of this area in their everyday practice.

Introduction of percutaneous-tunneled transfontanellar external ventricular drainage in the management of hydrocephalus in extremely low-birth-weight infants.

OBJECTIVE Hydrocephalus treatment in extremely low-birth-weight (ELBW) infants still represents a challenge for the pediatric neurosurgeon, particularly when the patient weighs far less than 1000 g. In such cases, the benefits in terms of neurological outcome following early treatment do not always outweigh the surgical risks, especially considering the great difference in the surgical risk before patient weight increases. To assess the efficacy and reliability of a percutaneous-tunneled, transfontanellar external ventricular drain (PTTEVD) in ELBW infants, the authors started a new protocol for the early surgical treatment of hydrocephalus. METHODS Ten cases of posthemorrhagic hydrocephalus (PHH) in ELBW infants (5 cases < 700 g, range for all cases 550-1000 g) were treated with a PTTEVD that was implanted at bedside as the first measure in a stepwise approach. RESULTS The average duration of the procedure was 7 minutes, and there was no blood loss. The drain remained in place for an average of 24 days (range 8-45 days). In all cases early control of the hydrocephalus was achieved. One patient had a single episode of CSF leakage (due to insufficient CSF removal). In another patient Enterococcus in the CSF sample was detected the day after abdominal surgery with ileostomy (infection resolved with intrathecal vancomycin). One patient died of Streptococcus sepsis, a systemic infection existing prior to drain placement that never resolved. One patient had Pseudomonas aeruginosa sepsis prior to drain insertion; a PTTEVD was implanted, the infection resolved, and the hydrocephalus was treated in the same way as with a traditional EVD, while the advantages of a quick, minimally invasive, bedside procedure were maintained. Once a patient reached 1 kg in weight, when necessary, a ventriculoperitoneal shunt was implanted and the PTTEVD was removed. CONCLUSIONS The introduction of PTTEVD placement in our standard protocol for the management of PHH has proved to be a wise option for small patients.

Cystic Congenital Scalp Inclusion Dermoid: A Case Report.

Dermoid cysts are developmental tumours that develop from germ cells displaced between the 3rd and 5th week of embryogenesis. Although dermoid cysts are known to be the most common scalp swellings; cystic congenital inclusion dermoid of the anterior fontanelle is a very rare scalp swelling. It is a benign, slow-growing, non-tender, soft swelling which is covered with intact skin. Ruling out intracranial extension is necessary. Computed Tomography is the investigation of choice for its diagnosis. We describe such a rare case of cystic congenital inclusion dermoid of the anterior fontanelle where the cyst was completely excised.

Application of hyperdry amniotic membrane patches without fibrin glue over the bony surface of mastoid cavities in canal wall down tympanoplasty.

CONCLUSION: Fibrin glue might be an inessential bioadhesive for attachment of hyperdry amniotic membrane (AM) patches in canal wall down (CWD) tympanoplasty. OBJECTIVE: To clarify the pliability and adherence capability of human hyperdry AM, the feasibility of fixing hyperdry AM without fibrin glue to the bony surface of the mastoid cavity was evaluated in CWD tympanoplasty. METHODS: This was a retrospective chart review. In seven ears of seven patients, the AM was simply attached over the bony surface of the mastoid cavity without fibrin glue (AMG(-) group). In 22 ears of 20 other patients, hyperdry AM (11 ears of 11 patients, AMG(+) group) or temporal fascia (11 ears of 9 patients, TFG(+) group) was attached over the bony surface of the mastoid cavity with fibrin glue. Times for graft epithelization were compared among the three groups. RESULTS: Complete epithelization of the mastoid cavity took place in all patients in all three groups. The mean time to complete epithelization of the graft in the AMG(-) and AMG(+) groups was significantly faster than that in the TFG(+) group (p < 0.05) and was not significantly different between the two AM groups (p > 0.05).

Congenital dermoid cyst at the anterior fontanelle: neuroimaging before and after fontanelle closure.

We report on a 2-month-old boy with a dermoid cyst arising at the anterior fontanelle, with observation during both the open and closed stages of the fontanelle. The etiology of this benign, curable tumor is discussed.

Uncinectomy through the anterior nasal fontanelle in endoscopic sinus surgery.

In functional endoscopic sinus surgery, the resection of the uncinate process is an important step. The traditional method of performing uncinectomy is associated with the risk of penetrating the lamina papyracea and orbital fat exposure. Without timely detection and appropriate treatment, uncinectomy may lead to serious consequences such as blindness. In our study, we used the novel approach of performing uncinectomy through the anterior nasal fontanelle. Orbital complications were observed in 4 of the 112 patients who underwent the traditional method; however, no complication was observed in patients who underwent surgeries with the new approach (P < 0.01). No nasolacrimal duct injury or eye movement disorder was observed in the 2 groups. The percentage of opening of the maxillary sinus ostia by using the new method was significantly greater than that achieved by using the traditional method (97.93% and 70.53%, respectively). The mean score in the visual analog scale, which is used to measure the efficacy of the procedure, for the new method was lower than that for the traditional method, and the difference was statically significant (t = 4.17, P < 0.01). Six and 12 months after the operations, the Lund-Kennedy endoscopy scores obtained when the new method was used were lower than those obtained when the traditional method was used (t = 3.27 and t = 4.40, respectively; P < 0.05). Therefore, uncinectomy through the anterior nasal fontanelle was a convenient and safe procedure and ensured good exposure of the maxillary sinus ostia. This procedure effectively improved the efficacy of endoscopic sinus surgery.

Combined fontanelle puncture and surgical operation in treatment of desmoplastic infantile astrocytoma: case report and a review of the literature.

Desmoplastic infantile astrocytoma is a rare low-grade malignant brain tumor found in infants. Its pathological diagnosis can be made on the basis of its histological characteristics and immunohistochemical staining. A case of desmoplastic infantile astrocytoma, including its clinical manifestations, pathological characteristics, differential diagnosis, treatment, and prognosis, is reported. Presurgical percutaneous decompression and subsequent resection resulted in a satisfactory therapeutic outcome.

The two holes syndrome.

BACKGROUND: Defects in the fontanel region of the lateral nasal wall have been described in the literature as "accessory" or "secondary" ostia. The authors consider them a sign of chronic maxillary sinusitis. Along with mucus recirculation between the natural ostium and the fontanel defect, we call it the Two Holes Syndrome. The aim of this study was to determine the incidence of fontanel defects in patients with chronic rhinosinusitis (CRS) and in healthy subjects. METHODS: Eighty-eight hundred seventy-nine outpatients with CRS were examined by means of nasal fiberendoscopy. The control group consisted of 1442 healthy volunteers with no previous history of CRS. RESULTS: Defect in the posterior fontanel was found in 1713/8879 CRS patients (19.3%). It was bilateral in 1165 cases (68.03%). Defect in the anterior fontanel was found in 54 patients (0.61%). The circulating mucus ring was identified in 162 patients with fontanel defects (9.17%). The defect in the posterior fontanel was found in 7/1442 healthy volunteers (0.48%). It was never bilateral, the circulating mucus was not observed and defect in the anterior fontanel was not found. CONCLUSION: Posterior fontanel defects were found more frequently in CRS patients than in healthy subjects. These defects have been clinically related to chronic infection of the maxillary sinus and should not be called "accessory" or "secondary" ostia. CRS with defects of the fontanel region and mucus recirculation can promote a number of health disturbances (chronic postnasal drip, headache, and cough). We named this entity the Two Holes Syndrome.

Correction of depressed forehead with BoneSource in cleidocranial dysplasia.

Cleidocranial dysplasia is a well-documented rare congenital disorder of the bone characterized by abnormalities of the skull, clavicle, and dentition. Despite numerous observations, there are still comparatively few reports regarding patients with cleidocranial dysplasia that focus on the impact of reconstruction of these defects on facial aesthetics. We report a 19-year-old woman with opened metopic and sagittal sutures and delayed closure of the anterior fontanelle. Through bicoronal incision and pericranial elevation, the defect was fully exposed. BoneSource was used to fill the cranial defect and was contoured to the desired cosmetic outcome. During a 24-month follow-up period, no absorption or recurrence was seen, and the patient was satisfied.