Rare Presentation of Rapidly Involuting Congenital Hemangioma of the Skull: A Case Report.

BACKGROUND Rapidly involuting congenital hemangioma (RICH) of the fetal skull is an extremely rare vascular disease which undergoes proliferation only in utero and progresses with maximal size at birth. RICH can be detected by prenatal imaging but is easily misdiagnosed. CASE REPORT A 28-year-old nulliparous woman was referred at 38 weeks of gestation for routine screening with obstetric ultrasonography. The ultrasonography revealed a female fetus with a previously undetected head tumor (32×22 mm). Certain unusual sonographic features were observed: the lesion was fusiform, with a wide base adjacent to the frontal bone. Tumor growth appeared to be toward the brain parenchyma rather than outwards (ie, toward the skull), which suggested that the mass may have been derived from the skull. The mass may have remained undiagnosed due to its small size or due to the superimposition of the skull in poor quality ultrasound images. On the basis of ultrasound findings, the lesion was diagnosed as an intracranial tumor, but fetal MRI findings led to the suspicion of RICH of the fetal skull. Finally, the patient was followed up until 1 year after birth, by which time the lesion had completely disappeared. CONCLUSIONS Careful evaluation of prenatal ultrasound is necessary to ensure detection of any mass adjacent to the skull, and the ultrasonography technician should carefully examine the features of any suspected mass to diagnose it correctly to avoid affecting the treatment strategy.

Neonatal Teratoma: Craniofacial Treatment.

Teratomas are rare congenital neoplasms. Head and neck locations of the tumor are uncommon with combined intracranial and extracranial extensions being even more rare. The authors present a case of teratoma involving the temporal, buccal, maxillary, orbital and extending to the intracranial regions, which was successfully managed by surgical resection.

Congenital Calvarial Hemangioma.

OBJECTIVES: The authors describe a case of congenital calvarial hemangioma successfully managed using propranolol therapy. Presenting symptoms, radiological and pathological features, differential diagnosis, and management of this rare congenital mass are described. CASE PRESENTATION: A 2-year-old boy presented with a 1-year history of a growing right parietal skull mass. No obvious etiology was apparent. No focal neurological deficits or associated craniofacial anomalies were identified. Plain film imaging demonstrated focal thickening of the right parietal bone with internal trabeculations in a sunburst appearance. Computed tomography (CT) scan showed bone thickening with coarsening of the bony trabeculae, minor irregularity of the outer table, unaffected inner table, and no evidence of aggressive features. A diagnostic biopsy of the lesion was performed in the operating room. Microscopic examination was consistent with hemangioma. Based on histological and radiological features of the lesion, it was identified as a cavernous hemangioma. Medical treatment utilizing propranolol was initiated for over 3 years with interval reduction in the lesion size. MRI head following treatment with propranolol demonstrated reduction of the mass compared to preoperative imaging. CONCLUSIONS: Although a rare entity, it is important to consider congenital calvarial hemangioma in the differential diagnosis of slow growing skull lesions due to the possibility of complications as a result of the hemangioma's intracranial extension, and the potential for treatment. En bloc resection has classically been described as a treatment for such lesions, although our case demonstrates that medical treatment with propranolol therapy may be appropriate in certain situations.

Congenital intraosseous cavernous hemangioma of the skull: an unusual case.

Intraosseous hemangiomas are benign vascular malformations mostly seen in the spine. They rarely occur in the skull. The usual age-group involved is the 2nd to 4th decades, and females outnumber males. We hereby report a rare case of congenital intraosseous cavernous hemangioma of the skull bone in a male infant. The patient underwent total excision of the lesion.

Congenital skull defect and neurofibroma: without scalp and other abnormalities.

Congenital skull defect is a rare malformation that is usually associated with congenital anomalies of the scalp and comparable lesions in the brain, spinal cord, limbs, and skeletal muscle. Most previously reported cases have described skull defects with aplasia cutis congenita and other congenital abnormalities. Very few patients with skull defects present with an intact scalp or neurofibroma. The authors report an adult patient with a rare congenital skull defect and local neurofibroma.

Huge mastoid congenital cholesteatoma in a 52-year-old patient.

Congenital cholesteatoma may arise in various locations within the temporal bone. The rarest site of origin is the mastoid process. We report an unusual case of a 52-year-old man with mastoid congenital cholesteatoma that manifested as a persistent ear discharge. The preoperative suspicion was based on the imaging findings and the patient's history. A simple mastoidectomy was conducted and the cholesteatoma was completely removed while using facial nerve monitoring. Although rare, mastoid congenital cholesteatoma can be considered as an alternative in the differential diagnosis of persistent otorrhea.

Midline dermoid cyst resulting in frontal bone erosion.

A dermoid cyst is a thin-walled benign tumor formed by the entrapment of ectodermal tissue during embryologic development, resulting in the inclusion of epithelium and adnexal elements within the tumor. Dermoids are not unique to a single anatomic location but are often isolated to the skin and subcutaneous tissue. They may occur intracranially or intra-abdominally, oftentimes associated with the ovary. If presenting as a midline mass of the skull, preoperative imaging with computed tomography and/or magnetic resonance imaging is necessary to evaluate for possible intracranial extension, given the altered embryologic development behind the formation of these cysts. Differential diagnosis of a midline frontonasal mass includes epidermoid or dermoid cyst, encephalocele, glioma, and sinus pericranii. The management of suspected dermoid cysts includes complete surgical excision, which may require a combined intracranial and extracranial approach. We present a 2-year-old boy who presented to our institution with a congenital midline scalp mass separate from the anterior fontanelle with complete underlying bony erosion to the sagittal sinus demonstrated on preoperative imaging, who required early surgical excision and reconstruction of the bony defect.

Congenital cavernous hemangioma of the skull.

Calvarial cavernous hemangiomas (CHs) comprise 0.2% of benign neoplasms of the skull and frequently occur in the second and fourth decades. Their occurrence in infancy is extremely rare and they can initially be misdiagnosed as cephal hematoma or caput succadeneum that can occur due to the birth trauma, but trauma is not a predisposing factor. CT scan is more helpful than other neuro-imaging studies and untreated CHs of the skull may show progression. Only two cases of congenital primary CH of the skull without intracranial invasion have been reported in the literature. Herewith we reported a 4-month-old infant operated on due to a CH of the parietal bone and discussed the diagnostic and therapeutic modalities.

Giant craniofacial immature teratoma with primary intracranial lesions.

Teratomas can occur in almost any region of the body and are the most common extragonadal germ cell childhood tumors. Immature teratomas in the head and neck region cause severe deformity and threat life because of several reasons. Imaging investigation (ultrasonography, computed tomography, magnetic resonance imaging) is of outmost importance for the diagnosis and preoperative planning, considering that the total surgical resection of the teratoma constitutes the treatment of choice. Untreated teratomas have mortality rates of 80% to 100%. In this study, a giant craniofacial immature teratoma having both intracranial and extracranial primary lesions is reported. As a conclusion, intracranial primary lesions should be kept in mind in extracranial head and neck immature teratoma cases.

Congenital cavernous hemangioma of the calvaria. Case report.

The authors present the case of a 6-month-old infant with a cavernous hemangioma of the parietal bone and discuss the radiological, operative, and pathological features and differential diagnosis of these extremely rare lesions in infants. Only 1 case of an infant with a calvarial cavernous hemangioma without intracranial invasion has previously been reported, and that case involved a 4 month old. Although a cavernous hemangioma of the calvaria is extremely rare in the newborn, this condition should be included in the differential diagnosis of calvarial lesions. During surgical treatment of calvarial cavernous hemangiomas, utmost attention should be paid to avoid blood loss, which could be fatal in infants.

Prenatal imaging findings in rapidly involuting congenital hemangioma of the skull.

We report two cases of rapidly involuting congenital hemangioma (RICH) of the skull diagnosed in the third trimester of gestation, and also present a brief review of the literature. In both of our cases ultrasound examination showed a soft tissue vascular mass of the skull with a specific sonographic finding: a thin hyperechogenic line over the lesion and continuous with the calvaria, suggesting a subperiosteal origin and possibly accounting for a mass effect on the underlying skull. This was slight in one case and marked in the other (and associated with involvement of the calvaria). On prenatal T2-weighted magnetic resonance imaging, the signal of each of the lesions was less marked than the hypersignal encountered in the postnatal period. Postnatal clinical and radiological follow-up over the first few months after delivery confirmed the diagnosis of RICH in each case by demonstrating a significant decrease in the size of the tumor and regression of the vascular component, with complete involution of the lesion within a year.

Gardner's syndrome: genetic testing and colonoscopy are indicated in adolescents and young adults with cranial osteomas: a case report.

We present a case of a 25-year-old female with diagnosed familial adenomatous polyposis and elevated carcinoembryonic antigen with negative family history. The suspicion of Gardner's syndrome was raised because extirpation of an osteoma of the left temporo-occipital region was made 10 years ago. Restorative procto-colectomy and ileal pouch anal anastomosis was made but histology delineated adenocarcinoma of the rectum (Dukes C stage). We conclude that cranial osteomas often precede gastrointestinal manifestations of familial adenomatous polyposis or Gardner's syndrome and such patients should be evaluated with genetic testing followed by colonoscopy if results are positive to prevent the development of colorectal carcinoma. If the diagnosis is positive all family members should be evaluated for familial adenomatous polyposis.

Dermoid cyst of the anterior fontanelle in adults: case report.

Head and neck dermoid cysts are relatively rare, and usually occur during childhood as solitary lesions. They are often identified and surgically removed at birth, being uncommon in adults. A 23-year-old male presented with a congenital tumor of the anterior fontanelle, which histopathological examination revealed a dermoid cyst. Surgical intervention is the treatment of choice to remove this lesion. The objective of this study is to report the case, once this type of lesion is rare in adults.

Dermoid cyst of the anterior fontanelle in adults: case report

Head and neck dermoid cysts are lesions relatively rare, which usually occur during childhood as solitary lesions. They are often identified and surgically removed at birth, being uncommon in adults. A 23-year-old male presented with a congenital tumor of the anterior fontanelle, which histopathological examination revealed a dermoid cyst. Surgical intervention is the treatment of choice to remove this lesion. The objective of this study is to report the case, once this type of lesion is rare in adults.

Cistos dermóides de cabeça e pescoço são relativamente raros e, usualmente, ocorrem na infância como lesões solitárias. Eles são diagnosticados e operados ao nascer, na maioria dos casos; portanto, essa é uma lesão incomum no adulto. Um homem de 23 anos apresentava tumoração congênita na fontanela anterior, cujo exame histopatológico revelou ser cisto dermóide. Foi submetido a tratamento cirúrgico. O objetivo desse estudo é relatar o caso, uma vez tratar-se de condição rara no adulto.

Bilateral parieto-occipital dermoid sinuses in a Rottweiler.

Bilateral dermoid sinuses were identified on the parieto-occipital region of a Rottweiler. Diagnosis was confirmed by histological examination after successful complete surgical resection. The dermoid sinuses were independent with separate tracts. This unusual parasagittal location can be explained by craniofacial development: dermoid sinuses on the head could occur along the lines of embryological fusion and not only in the sagittal plane. A hypothesis of an origin at the level of the suture between the parietal and interparietal bones is possible in this case.