Conjoined twinning has always been a phenomenon of great interest to the medical community because of its rarity and complexity. Each additional documented case bolsters our knowledge of embryologic development, anatomic anomalies, and strategic approaches to surgical challenges. Twins conjoined at the head, that is, craniopagi, are the rarest subtype of conjoined twins, and rates of survival to birth and through surgical separation are low. Those that do survive usually require multiple intensive procedures and often face lifelong developmental challenges due to shared brain tissue that must be parsed at time of separation. Here we present a case study of partial angular frontal craniopagus twin females with a soft-tissue-only conjuncture currently unrepresented in medical literature. Documentation of this case, in which a one-stage surgical separation occurred at day of life 1 without complication or any long-term disability, helps inform those that provide care for conjoined twins alongside their expectant parents of the wide possible range of clinical presentations and prognosis for craniopagi. A 28-year follow-up investigates how the anatomic configuration of the craniopagi and mechanical forces in utero impacted craniofacial development and their phenotypic characteristics as adults. Using clinical examination, CT imaging with 3D reconstruction, and Vectra® H1 stereophotogrammetry, overall symmetry and congenital deformities of both women’s external features and underlying craniofacial skeleton were characterized. Both twins exhibit near-identical skull deformities including right frontal bone flattening, ipsilateral parietal and occipital bossing, flattened malar eminence, and curvature of sagittal plane through the skull. Reported right anterolateral displacement of anterior fontanelles directly under the twins’ junctional area during infancy is reflected by asymmetry of the coronal suture withanterior displacement on the right side in both cases. In addition, Twin B has a persistent metopic suture that also shows lateral displacement toward site of junction. Soft tissue deformities include minor cosmetic asymmetries such as a sharp hairline irregularity below previous site of conjuncture, misaligned ears, sparse right eyebrow growth and weakened movement of this brow, and deviation of nasal tip in one twin. Both twins also sport a 5cm by 1.5-2cm surgical scar. Overall symmetry is good and most of these irregularities go unnoticed by laypeople; the women manage most of them with cosmetics and side-parting of the hair. Though some minor plastic surgery procedures could be offered to optimize symmetry, none are recommended or desired by the patients at this time. Through careful evaluation of their adult state, we conclude that minimally conjoined craniopagus twins sharing only soft tissue of the scalp at time of birth can still exhibit a number of lifelong cranial deformities and asymmetry of facial landmarks.
| Identifer | oai:union.ndltd.org:ETSU/oai:dc.etsu.edu:asrf-1886 |
| Date | 07 April 2022 |
| Creators | Howard, Rebecca A, Billington, Alicia R, MD, PhD, Powers, Jeremy M, MD, FACS |
| Publisher | Digital Commons @ East Tennessee State University |
| Source Sets | East Tennessee State University |
| Detected Language | English |
| Type | text |
| Source | Appalachian Student Research Forum & Jay S. Boland Undergraduate Research Symposium |
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