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Trigonocephaly


Presentation

  • A review of 22 patients with Opitz-C syndrome from the literature is presented. Most of the typical facial dysmorphism can be regarded as part of a trigonocephaly "sequence" rather than presenting characteristic features of a syndrome.[ncbi.nlm.nih.gov]
  • Presented at the Consensus Conference on Craniosynostoses, Rome 1995 Google Scholar 28. Posnick JC, Lin KY, Chen P, Armstrong D (1994) Metopic synostosis: quantitative assessment of presenting deformity and surgical results based on CT scans.[doi.org]
Hyperthermia
  • Bulimia and episodic hyperthermia were attributed to hypothalamic dysfunction. Results of unstimulated endocrine studies and gonadotropin releasing hormone (GnRH), and human chorionic gonadotropin (HCG) stimulation tests were normal.[ncbi.nlm.nih.gov]
Bulimia
  • Bulimia and episodic hyperthermia were attributed to hypothalamic dysfunction. Results of unstimulated endocrine studies and gonadotropin releasing hormone (GnRH), and human chorionic gonadotropin (HCG) stimulation tests were normal.[ncbi.nlm.nih.gov]
Angiomatosis
  • The cardinal features of Sturge-Weber syndrome are facial cutaneous capillary malformation (port-wine stain), leptomeningeal angiomatosis, and glaucoma. Premature closure of the metopic suture results in trigonocephaly.[ncbi.nlm.nih.gov]
Bitot's Spots
  • Shawky and Radwa Gamal, C syndrome with skeletal anomalies, mental retardation, eyelid chalazion, Bitot’s spots and agenesis of the corpus callosum in an Egyptian child, Egyptian Journal of Medical Human Genetics, 18, 1, (93), (2017).[doi.org]
Self-Mutilation
  • PATIENTS AND METHODS: Fifty-six children (44 boys, 12 girls) in whom ICP was measured were diagnosed with mild trigonocephaly (nonsyndromic type) with symptoms such as language delay, hyperactivity, autistic tendencies, self-mutilation, motor delay, etc[ncbi.nlm.nih.gov]
Self Mutilation
  • PATIENTS AND METHODS: Fifty-six children (44 boys, 12 girls) in whom ICP was measured were diagnosed with mild trigonocephaly (nonsyndromic type) with symptoms such as language delay, hyperactivity, autistic tendencies, self-mutilation, motor delay, etc[ncbi.nlm.nih.gov]
Facial Edema
  • Woo, Adverse facial edema associated with off-label use of recombinant human bone morphogenetic protein–2 in cranial reconstruction for craniosynostosis, Journal of Neurosurgery: Pediatrics, 1, 3, (255), (2008). D. Renier, É. Lajeunie, M. Catala, É.[doi.org]
Microphallus
  • He has frontal bossing, ridged metopic suture, bilateral ptosis, right squint, depressed nasal bridge, small nose, anteverted nostrils, lobulated tongue, polydactyly of both hands, microphallus, hypoplastic scrotum, microtestes, dysgenesis of corpus callosum[ncbi.nlm.nih.gov]
Perseveration
  • Thus, although some of the striking features of trigonocephaly are eliminated, minor characteristics of the anomaly still persevere at 4 years of age.[ncbi.nlm.nih.gov]

Workup

X-Ray Abnormal
  • Factors contributing to improvement were younger age, relatively higher development quotient, marked digital impressions on skull X-rays, abnormal findings on SPECT, and moderate degree of trigonocephaly.[ncbi.nlm.nih.gov]
Hyponatremia
  • Moritz, James Cray, Lorelei Grunwaldt and Joseph Losee, Postoperative hyponatremia following calvarial vault remodeling in craniosynostosis, Pediatric Anesthesia, 21, 10, (1020-1025), (2011).[doi.org]

Treatment

  • CONCLUSIONS: Early detection and treatment of metopic suture synostosis has a significant, favorable influence on the outcomes.[ncbi.nlm.nih.gov]
  • Anderson FM (1981) Treatment of coronal and metopic synostosis: 107 cases. Neurosurgery 8:143–149 Google Scholar 2. Anderson FM, Gwinn JL, Todt JC (1962) Trigonocephaly: identity and surgical treatment. J Neurosurg 19:723–730 Google Scholar 3.[doi.org]

Prognosis

  • The importance of the minor anomalies in its recognition and its good prognosis are emphasized.[ncbi.nlm.nih.gov]
  • Prognosis In general, a single operation is all that is necessary to treat this condition and the cosmetic and clinical outcomes are usually excellent especially for otherwise healthy, nonsyndromic infants.[skullbaseinstitute.com]
  • Prognosis The clinical outcome for patients having undergone surgery is usually satisfactory with a low requirement for additional interventions, especially in the mild forms. Recurrence of a prominent metopic ridge may occur in some rare cases.[orpha.net]

Etiology

  • Sinn, Classification, Diagnosis, and Etiology of Craniofacial Deformities, Maxillofacial Surgery, 10.1016/B978-0-7020-6056-4.00059-9, (803-834), (2017).[doi.org]
  • However, the concordance rate of isolated trigonocephaly in monozygotic twins is 43%, suggesting that both genetic and environmental factors are involved in the etiology of this disorder.[panafrican-med-journal.com]
  • The etiology of trigonocephaly is mostly unknown although there are three main theories. Trigonocephaly is probably a multifactorial congenital condition, but due to limited proof of these theories this cannot safely be concluded.[en.wikipedia.org]

Epidemiology

  • Bartlett, The Changing Epidemiologic Spectrum of Single-Suture Synostoses, Plastic and Reconstructive Surgery, 10.1097/PRS.0b013e31817d548c, 122, 2, (527-533), (2008).[doi.org]
  • Summary Epidemiology Incidence is estimated at 1/15,000 births. Males are more frequently affected than females (sex ratio of 2:1) and the frequency of trigonocephalic twins is unexpectedly high.[orpha.net]
Sex distribution
Age distribution

Pathophysiology

  • However, because the exact pathophysiology remains unknown, we examined the pathophysiologic mechanisms behind cognitive dysfunction in patients with trigonocephaly, with an aim of providing a model for cognitive dysfunction based on routinely available[ncbi.nlm.nih.gov]
  • Okkerse, Insight into the Pathophysiologic Mechanisms behind Cognitive Dysfunction in Trigonocephaly, Plastic and Reconstructive Surgery, 10.1097/PRS.0000000000003179, 139, 4, (954e-964e), (2017). David M. Yates and Douglas P.[doi.org]

Prevention

  • The possibility of coincidence should not prevent continued surveillance of OTS patients in the future for the occurrence of malignancy.[ncbi.nlm.nih.gov]
  • Conclusion: The early recognition of these anomalies including all craniosynostoses, the deformities of the newborn and infant’s head and the preventive operative reconstruction would prevent abnormal disturbance of the psychomotor development during[degruyter.com]

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