The trans-sylvian trans-petrosal "half & half" approach-a how I do it.

BACKGROUND: Petroclival meningiomas are one of the most challenging tumors to be operated in the realm of neurosurgery. Many approaches have been developed over the years. METHOD: The authors describe the Half & Half (H&H) approach whose main indication is petroclival meningiomas with suprasellar extension. The part of the tumor located above CN III and in the retrochiasmatic space is addressed through a trans-sylvian, while the petroclival portion is through an extradural anterior petrosectomy approach. The wide surgical corridor given by this approach allows extensive tumor resection while avoiding the risk associated with the manipulation of intracavernous neurovascular structures. CONCLUSION: The H&H approach is an effective strategy to maximize the safe resection of petroclival meningiomas.

Endoscopic transorbital approach in children: surgical technique and early results.

OBJECTIVE: In this study, the authors aimed to describe the endoscopic transorbital approach (ETOA) in children. METHODS: Six pediatric patients (2 girls and 4 boys) underwent the ETOA for paramedian skull base lesions at a single institution between September 2016 and February 2023. RESULTS: The median age at the time of surgery was 7.5 (range 4-18) years. The median follow-up period was 33 (range 9-60) months. In this series, the ETOA level of difficulty included stage 1 (n = 2, 33.3%), stage 3 (n = 3, 50%), and stage 5 (n = 1, 16.7%). The ETOA was performed for tumor resection in 4 cases; the final pathology consisted of fibrous dysplasia, pilocytic astrocytoma, metastatic neuroblastoma, and choroid plexus papilloma. The procedure was also performed for repair of a petrous apex meningocele and for lateral orbital wall decompression of traumatic lateral rectus muscle entrapment. One patient experienced a transient cranial nerve III palsy after the procedure. There were no operative deaths in this series. CONCLUSIONS: In select cases, the ETOA can be considered a minimally invasive alternative for conventional skull base approaches in the armamentarium of pediatric skull base surgery. Further investigation and the accumulation of experience are warranted in the future to enhance the efficacy and applicability of the ETOA in pediatric patients.

Endoscopic precaruncular medial transorbital and endonasal multiport approaches to the contralateral skull base: a clinicoanatomical study.

OBJECTIVE: Minimally invasive endoscopic endonasal multiport approaches create additional visualization angles to treat skull base pathologies. The sublabial contralateral transmaxillary (CTM) approach and superior eyelid lateral transorbital approach, frequently used nowadays, have been referred to as the "third port" when used alongside the endoscopic endonasal approach (EEA). The endoscopic precaruncular contralateral medial transorbital (cMTO) corridor, on the other hand, is an underrecognized but unique port that has been used to repair CSF rhinorrhea originating from the lateral sphenoid sinus recess. However, no anatomical feasibility studies or clinical experience exists to assess its benefits and demonstrate its potential role in multiport endoscopic access to the other contralateral skull base areas. In this study, the authors explored the application and potential utility of multiport EEA combined with the endoscopic cMTO approach (EEA/cMTO) to three target areas of the contralateral skull base: lateral recess of sphenoid sinus (LRSS), petrous apex (PA) and petroclival region, and retrocarotid clinoidocavernous space (CCS). METHODS: Ten cadaveric specimens (20 sides) were dissected bilaterally under stereotactic navigation guidance to access contralateral LRSS via EEA/cMTO. The PA and petroclival region and retrocarotid CCS were exposed via EEA alone, EEA/cMTO, and EEA combined with the sublabial CTM approach (EEA/CTM). Qualitative and quantitative assessments, including working distance and visualization angle to the PA, were recorded. Clinical application of EEA/cMTO is demonstrated in a lateral sphenoid sinus CSF leak repair. RESULTS: During the qualitative assessment, multiport EEA/cMTO provides superior visualization from a high vantage point and better instrument maneuverability than multiport EEA/CTM for the PA and retrocarotid CCS, while maintaining a similar lateral trajectory. The cMTO approach has significantly shorter working distances to all three target areas compared with the CTM approach and EEA. The mean distances to the LRSS, PA, and retrocarotid CCS were 50.69 ± 4.28 mm (p < 0.05), 67.11 ± 5.05 mm (p < 0.001), and 50.32 ± 3.6 mm (p < 0.001), respectively. The mean visualization angles to the PA obtained by multiport EEA/cMTO and EEA/CTM were 28.4° ± 3.27° and 24.42° ± 5.02° (p < 0.005), respectively. CONCLUSIONS: Multiport EEA/cMTO to the contralateral LRSS offers the advantage of preserving the pterygopalatine fossa contents and the vidian nerve, which are frequently sacrificed during a transpterygoid approach. This approach also offers superior visualization and better instrument maneuverability compared with EEA/CTM for targeting the petroclival region and retrocarotid CCS.

The Displacement Patterns of Petrous Internal Carotid Artery and Its Morphometric Relations with Vidian Canal in Petroclival Chondrosarcomas Relevant to Extended Endoscopic Endonasal Approaches: A Radiological Study.

BACKGROUND: Extended endoscopic endonasal approaches (EEAs) to petroclival chondrosarcomas (PCs) require a thorough understanding of skullbase anatomy, especially the anatomy of petrous internal carotid artery (pICA), as ICA injury is the most dreaded complication of extended EEAs. We conducted this study to determine the displacement patterns of pICA in patients with PCs. METHODS: Contrast enhanced computed tomography scan and angiography images of patients with PCs were analyzed for following parameters-antero-posterior, cranio-caudal, medio-lateral, and direct distances between anterior genu of petrous internal carotid artery (AGpICA) and posterior end of Vidian canal (pVC). pICA encasement/narrowing by tumor was noted on magnetic resonance imaging. RESULTS: We studied 11 patients with histopathologically proven PCs. pICA encasement/narrowing and pVC destruction were observed in one patient each. The mean antero-posterior and cranio-caudal distances on tumor side/normal side were 7.7 ± 1.9/6.4 ± 1.0 mm & 4.5 ± 1.5/3.4 ± 0.9 mm, respectively. The overall displacement was posterior & superior. Medio-lateral displacement was seen in 4 patients (lateral in 3 and medial in 1). In rest, AGpICA was centered on pVC. The mean direct distance was 9.4 ± 2.5 mm. In 3 patients with displacement seen in all three axes, direct distance was measured by the "cuboid method." Overall, posterior-superior-lateral, posterior-superior, and anterior-inferior were the common displacement patterns of AGpICA relative to pVC. CONCLUSIONS: The displacement patterns of AGpICA in PCs are variable. An individualized approach with meticulous analysis of preoperative imaging can help in determining the relation between AGpICA and pVC. This detailed morphometric information can facilitate better orientation to altered anatomy, which can be helpful in preventing pICA injury during extended EEAs.

Assessing the feasibility of the transmastoid infralabyrinthine approach without decompression of the jugular bulb to the extradural part of the petrous apex and petroclival junction prior to surgery.

BACKGROUND AND OBJECTIVE: This study aims to define specific measurements on cranial high-resolution computed tomography (HRCT) images prior to surgery to prove the feasibility of the navigated transmastoid infralabyrinthine approach (TI-A) without rerouting of the facial nerve (FN) and decompression of the jugular bulb (JB) in accessing the extradural-intrapetrous part of petrous bone lesions located at the petrous apex and petroclival junction. MATERIALS AND METHODS: Vertical and horizontal distances of the infralabyrinthine space were measured on cranial HRCT images prior to dissection. Subsequently, the area of access was measured on dissected human cadaveric specimens. Infralabyrinthine access to the extradural part of the petrous apex and petroclival junction was evaluated on dissected specimens by two independent raters. Finally, the vertical and horizontal distances were correlated with the area of access. RESULTS: Fourteen human cadaveric specimens were dissected bilaterally. In 54% of cases, the two independent raters determined appropriate access to the petrous apex and petroclival junction. A highly significant positive correlation (r = 0.99) was observed between the areas of access and the vertical distances. Vertical distances above 5.2 mm were considered to permit suitable infralabyrinthine access to the extradural area of the petrous apex and petroclival junction. CONCLUSIONS: Prior to surgery, vertical infralabyrinthine distances on HRCT images above 5.2 mm provide suitable infralabyrinthine access to lesions located extradurally at the petrous apex and petroclival junction via the TI-A without rerouting of the FN and without decompression of the JB.

Combined microscope-endoscopy resection of petrous bone cholesteatoma with temporary facial nerve transposition versus nontransposition.

PURPOSE: The narrow supralabyrinthine space affects surgical procedures. To study the effect of temporary transposition of geniculate ganglion of facial nerve versus nontransposition on lesion recurrence and facial nerve function in patients with petrous bone cholesteatoma. METHODS: A total of 18 patients with petrous bone cholesteatoma involving the facial nerve were treated in our hospital from November 2016 to March 2023. The main surgical method is the extended supralabyrinthine approach assisted by a microscope and an endoscope. We collected and retrospectively analyzed their medical records. RESULTS: Temporary facial nerve transposition was performed in five patients, and nontransposition was performed in 13 patients. Cholesteatoma recurred in three patients with facial nerve nontransposition, whereas none in patients with facial nerve transposition. In this study, except for one case with a second operation, postoperative facial paralysis in other cases was improved to varying degrees, and there was no significant difference between the two groups. CONCLUSION: Temporary transposition of geniculate ganglion of facial nerve will not affect the postoperative nerve function of patients and can reduce the possibility of cholesteatoma recurrence of the petrous bone.

Dependence of Cochlear Duct Length Measurement on the Resolution of the Imaging Dataset.

HYPOTHESIS: Measurements of the cochlear duct length (CDL) are dependent on the resolution of the imaging dataset. BACKGROUND: Previous research has shown highly precise cochlear measurements using 3D-curved multiplanar reconstruction (MPR) and flat-panel volume computed tomography (fpVCT). Thus far, however, there has been no systematic evaluation of the imaging dataset resolution required for optimal CDL measurement. Therefore, the aim of this study was to evaluate the dependence of CDL measurement on the resolution of the imaging dataset to establish a benchmark for future CDL measurements. METHODS: fpVCT scans of 10 human petrous bone specimens were performed. CDL was measured using 3D-curved MPR with secondary reconstruction of the fpVCT scans (fpVCT SECO ) and increasing resolution from 466 to 99 µm. In addition, intraobserver variability was evaluated. A best-fit function for calculation of the CDL was developed to provide a valid tool when there are no measurements done with high-resolution imaging datasets. RESULTS: Comparison of different imaging resolution settings showed significant differences for CDL measurement in most of the tested groups ( p < 0.05), except for the two groups with the highest resolution. Imaging datasets with a resolution lower than 200 µm showed lower intraobserver variability than the other resolution settings, although there were no clinically unacceptable errors with respect to the Bland-Altman plots. The developed best-fit function showed high accuracy for CDL calculation using resolution imaging datasets of 300 µm or lower. CONCLUSION: 3D-curved MPR in fpVCT with a resolution of the imaging dataset of 200 µm or higher revealed the most precise CDL measurement. There was no benefit of using a resolution higher than 200 µm with regard to the accuracy of the CDL measurement.

Isolated embryonal rhabdomyosarcoma of the anterior petrous bone in a young child: Focusing on the intra-operative diagnosis and differentials.

OBJECTIVE: Rhabdomyosarcoma is a common soft tissue tumor, but isolated involvement of anterior portion of petrous bone is exceedingly rare. Here, we present a case of embryonal rhabdomyosarcoma involving the anterior petrous without involvement of the mastoid and middle ear. PATIENT: A 6-year-old boy presented with a progressive right side lower motor neuron facial paresis for 1-month duration along with headache and recurrent vomiting episodes. Radiology showed a contrast-enhancing lesion involving the right petrous apex. He underwent craniotomy and excision of the lesion. Based on the frozen section, a diagnosis of rhabdomyosarcoma was rendered, and gross total resection could be achieved. Postoperative course was uneventful. CONCLUSION: Isolated petrous bone involvement of embryonal rhabdomyosarcoma is a rare presentation. Intra-operative frozen section plays a key role in decision making regarding the extent of excision. Hence, a prompt and accurate diagnosis is essential in managing these cases.

Anteromedial Petrous (Gardner's) Triangle: Surgical Anatomy and Relevance for Endoscopic Endonasal Approach to the Petrous Apex and Petroclival Region.

BACKGROUND AND OBJECTIVES: Triangular corridors have been used as reliable surgical entry points for open transcranial approaches to the petrous apex (PA) and petroclival region (PCR). The endoscopic endonasal approaches have grown rapidly in the last decade, and the indications have advanced. The knowledge of accurate and reliable anatomic landmarks through endoscopic endonasal route is essential and remain to be established. The purpose of this study was to describe the feasibility and surgical exposure of the anteromedial petrous (Gardner's) triangle as a novel corridor to the PA and PCR. METHODS: Five anatomic specimens were dissected. The PA and PCR were accessed through endoscopic endonasal approaches and contralateral transmaxillary approach. The limits of the anteromedial petrous (Gardner's) triangle were identified and dissected and associated measurements performed. RESULTS: The dissection was divided into 6 steps. The limits of the anteromedial petrous (Gardner's) triangle were identified and defined by the paraclival internal carotid artery anterolaterally, the abducens nerve posteromedially, and the petroclival synchondrosis inferiorly. Three lines were established following the limits of the triangle. The mean distance of the anterolateral limit was 10.03 mm (SD = 0.94), of the posteromedial limit was 20.06 mm (SD = 2.90), and of the inferior limit was 17.99 mm (SD = 2.99). The mean area was 87.56 mm 2 (SD = 20.06). The 3 anatomic landmarks with a critical role to safely define the triangle were the pterygosphenoidal fissure, the petrosal process of the sphenoid bone, and the petroclival synchondrosis. CONCLUSION: The anteromedial (Gardner's) triangle is a well-defined bone corridor which provides access to the entire petrous bone and petroclival junction through endoscopic endonasal route. Regardless of the anatomic variations or tumor location, the landmarks of the abducens nerve, paraclival internal carotid artery, and petroclival synchondrosis are key for understanding lateral access to tumors extending from the clivus.

The M-Point A Novel Landmark in Middle Fossa Surgery: A Cadaveric Morphometric Study.

BACKGROUND: The arcuate eminence (AE) is an anatomically consistent bony protrusion located on the upper surface of the petrous bone that has been previously studied as a reference for lateral skull base approaches. There is a paucity of information in the neurosurgical literature seeking to improve the safety of the extended middle cranial fossa (MCF) approach using detailed morphometric analysis of the AE. OBJECTIVE: To evaluate the use of the AE as an anatomical landmark to help with early identification of the internal acoustic canal (IAC) in MCF approaches by means of a cadaveric study, using a new morphometric reference termed the "M-point." METHODS: A total of 40 dry temporal bones and 2 formalin-preserved, latex-injected cadaveric heads were used. The M-point was established as a new anatomic reference by identifying the intersection of a line perpendicular to the alignment of the petrous ridge (PR), originating from the midpoint of the AE, with the PR itself. Subsequent anatomical measurements were performed to measure the distance between M-point and IAC. Additional distances, including PR length and the anteroposterior and lateral AE surfaces, were also measured. RESULTS: The mean distance between the M-point and the center of the IAC was 14.9 mm (SD ± 2.09), offering a safe drilling area during an MCF approach. CONCLUSIONS: This study provides novel information on identification of a new anatomic reference point known as the M-point that that can be used to improve early surgical identification of the IAC.

Septal Nasopharyngeal "Kite Flap" for Long-Term Patency of Petrous Apex Cholesterol Granuloma.

Medial petrous apex cholesterol granuloma is a benign lesion which treatment is generally based on a trans-nasal marsupialization. When the artificial ostium is created, it is usually kept open with local flaps, like the septal nasopharyngeal "kite flap", a reliable local vascularized flap. Laryngoscope, 134:2111-2114, 2024.

Investigating the Effects of Trigeminal Impression and Internal Acoustic Opening Morphology Differences for Possible Surgical Applications.

PURPOSE: The exhaustive information regarding the types of trigeminal pore (TP) or trigeminal impression (TI), internal acoustic opening (IAO), and related surgical approaches is lacking in the literature. Therefore, this study is performed to further elucidate the types of TP or TI, IAO, and the relationships with critical surgical landmarks in the skull base. METHODS: Trigeminal impression (TI) and internal acoustic opening (IAO) found in 11 dry skulls, 24 right temporal bones, and 25 left temporal bones were examined on both sides to define their relationship to each other and nearby structures. The age and sex of these bones were not identified. Besides these, 77 skulls were examined by radiologic imaging methods. These skulls were identified by gender. RESULTS: According to test results, there was a significant difference between the left and right internal acoustic opening in the case of horizontal dimension (HD). The left HD-IAO is bigger than the right one. In addition, right HD-IAO, vertical dimension (VD) of right internal acoustic opening, left HD-IAO, and left VD-IAO values differed significantly in male and female patients. CONCLUSIONS: Investigating the relationship of TI and IAO with relevant structures suggests that surgical approaches involving the TP and IAO indicated that surgical approaches considering the TI and IAO variations may be used in the development of surgical processes and primary surgical interventions.

Endoscopic endonasal transpterygoid nasopharyngectomy: Anatomical considerations and technical note.

BACKGROUND: The study was designed to identify new landmarks in the parapharyngeal segment of the internal carotid artery (ICA) for nasopharyngectomy and describe a surgical procedure of endoscopic endonasal transpterygoid nasopharyngectomy (EETPN). METHODS: Four cadaveric specimens were injected with colored silicone and subjected to CT scanning before dissection. The nasopharyngeal skull base was exposed using the endoscopic endonasal transpterygoid approach. The clinical data of four patients with nasopharyngeal malignances who underwent EETPN were reviewed. RESULTS: The lateral edge of the longus capitis muscle medially; the foramen lacerum, petrous apex spine and the stump of the levator veli palatini muscle superior laterally; and the upper parapharyngeal ICA laterally constitute the ICA-longus capitis muscle-petrous apex spine triangle which was a novel landmark for the upper parapharyngeal segment of the ICA. CONCLUSION: The ICA-longus capitis muscle-petrous apex spine triangle are important landmarks of the upper parapharyngeal segment of the ICA.

Lateral angle: A landmark-based method for the sex estimation in human cremated remains and application to an Austrian prehistoric sample.

OBJECTIVES: Estimating the sex of cremated human remains is difficult. The petrous bone frequently survives the cremation due to its density. Wahl observed the lateral angle to be sexually dimorphic in the 1980s. Previous studies showed various cut-off points to separate females from males, which are hardly replicable and difficult to apply. We want to test the Wahl method and compare it to a new landmark-based version. MATERIALS AND METHODS: In this study, we measured the lateral angle of 35 cremated petrous bones from late bronze age Austria using micro-CT scans. Technical errors of measurement were calculated for two different methods to intersect the internal acoustic meatus virtually in the midline (manual or landmark-based intersection). Furthermore, sex was estimated based on morphological features and metric measurements. This information was used in logistic regression modeling to define a cut-off point in our sample. RESULTS: The technical errors of measurement suggested that a landmark-based method was more precise in comparison to a manual intersection which was much more intuitive. Inter- and intra-observer errors were low which improved reliability. The logistic regression model produced good results in our sample (p = 0.02, R2 = 0.38, accuracy = 0.8). The mean lateral angle was similar to studies which focused on prehistoric cremated petrous bones. DISCUSSION: The proposed landmark-based method was precise, quick, and could be easily applied, even by unexperienced researchers. The size of the lateral angle seemed to be population-specific but also dependent on the method applied. We recommend to use the proposed landmark-based method which is more precise.

Surgical Approaches to Petrous Apex Cholesterol Granulomas: A Systematic Review and Network Meta-analysis.

OBJECTIVE: To compare the outcomes of different surgical approaches to petrous apex cholesterol granulomas (PACG). DATA SOURCES: PubMed, Embase, Google Scholar, Cochrane, and Web of Science. REVIEW METHODS: Following the Preferred Reporting Items for Systematic Reviews and Meta-analyses-Network Meta-analyses guidelines, databases were searched from inception to November 31, 2022. Studies comparing two or more approaches were included. Reviews and population studies were excluded. The main outcome measures were the resolution of symptoms, serviceable hearing, complication, and revision rates. RESULTS: The search yielded 2132 studies. After applying inclusion and exclusion criteria, 15 studies remained, consisting of 214 patients treated with lateral approaches (n = 182) or anterior endonasal approaches (n = 32). The efficacy of lateral and anterior endonasal approaches in achieving symptom resolution was comparable (73% vs. 68%, p = 0.5). Both exhibited similar rates of complications (33% vs. 37%, p = 0.3), albeit with distinct profiles. Lateral approaches were associated with higher rates of facial palsy and sensorineural hearing loss (44% vs. 18%, p = 0.03). Anterior endonasal approaches demonstrated higher rates of epistaxis and cerebrospinal fluid leak (15% vs. 1%, p = 0.001). Anterior endonasal approaches exhibited lower revision rates (OR: 0.35, 95% CI: 0.14-0.88). The placement of a stent in both approaches was associated with higher symptom resolution (OR: 5.12, 95% CI: 1.05-9.97) and lower revision rates (OR: 0.71, 95% CI: 0.33-0.92). CONCLUSIONS: Anterior endonasal approaches yield lower revision rates compared to lateral approaches for PACG. Both approaches demonstrate similar effectiveness in symptom resolution and comparable rates of complications, with distinct profiles. Facial nerve and hearing status are important factors that should be addressed when selecting the approach. Stenting is beneficial. LEVEL OF EVIDENCE: NA Laryngoscope, 134:1540-1550, 2024.

Clinical correlation research of 3D reconstruction of retrolabyrinthine space based on HRCT of temporal bone.

Background: The retrolabyrinthine approach helps clinicians perform complex surgeries such as vestibular neurectomy, resection of petrous apex cholesteatoma, or use this space to complete endoscopic combined with microscope surgical operations in a relatively safe buffer space. Some of our current studies using 3D reconstruction in the clinic have also helped us perform some complex surgical procedures.Objective: This study aims to reveal the relationship between important structures in retrolabyrinthine space through objective parameters. These measurement data help clinicians locate intraoperatively and provide a reference for clinical surgery. Also, we are intended to help improve surgical techniques and expand the operating space to increase reachable anatomic structure.Material and Methods: The inner structures of the temporal bone from HRCT (High-resolution computed tomography) images which were taken at the Eye & ENT Hospital of Fudan University were reconstructed. Precise measurement of the structures was accomplished by using the software 3D-Slicer (3D Slicer, https://www.slicer.org/; version 4.8.0, Massachusetts, USA).Results: 3D model of temporal bone structures, including the cochlea, semicircular canals (SCCs), the internal auditory canal (IAC), facial nerve (FN), jugular bulb(JB), and carotid artery was reconstructed. The combination of HRCT and 3D models is utilized to analyze the Quantitative data of the retrolabyrinthine space and its adjacent structures.Conclusions and Significance: 3D reconstruction of CT images clearly displayed the detailed structures of the temporal bone. Surgical adaptability of the retrolabyrinthine approach can be assessed preoperatively by image and other methods, and anatomical parameters play an important role in the retrolabyrinthine space. Therefore, this study helps to skeleton the bone as much as possible to expand the surgical space, so that the surgeon can contact the anatomical structure more diversified to expand the surgical indications.

Chondrosarcoma of the petrous apex with nerve abducens involvement in a woman with pre-existing contralateral amblyopia.

We report about a case of a woman in her 50s with a chondrosarcoma of the petrous apex. She has a strabism due to a right-sided amblyopia since childhood and noticed a slowly progressive squint angle in the last months. Her general practitioner (GP) referred her to an ophthalmologist who diagnosed a left abducens paresis and initiated an MRI demonstrating a mass in the left petrous apex. A transnasal endoscopic transsphenoidal biopsy revealed a grade I chondrosarcoma. The patient underwent an endoscopic transsphenoidal anterior petrosectomy approach with complete removal of the tumour. After surgery the squint angle gradually improved to previous levels, so that the patient was able to resume her work. This report demonstrates that even slight changes of a squint angle without diplopia, in this case because of pre-existing amblyopia, could be a hallmark of an intracranial process.

Acute Bacterial Meningitis and Petrous Apicitis in a Child with Aplasia Cutis Congenita: A Case Report.

Petrous apicitis and acute bacterial meningitis are uncommon in the present antibiotic era. The diagnosis of petrous apicitis is seldom considered unless there is cranial nerve palsy. A young child with aplasia cutis congenita presented with acute bacterial meningitis and an incidental opacified left mastoid in brain imaging. During the course, fever persisted, and high-resolution temporal bone imaging showed rapid progression to coalescent mastoiditis, petrous apicitis with erosions of tegmen tympani, and petrous apex. Other findings included bony dehiscences and thinning of left calvaria. Tympanomastoid exploration showed herniated brain and cerebrospinal fluid leak through tegmen tympani, which was closed with temporalis fascia graft. Herein, we report a rare presentation of petrous and tegmen erosion along with aplasia cutis congenita and discuss the challenges in diagnosis and management.