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Craniofacial & Cleft Care Essentials

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Surgical Journey Stories1 item
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Anterior encephaloceles present complex reconstructive challenges requiring multidisciplinary craniofacial expertise. Prenatal diagnosis via fetal MRI enables early surgical planning, though definitive tissue characterization remains impossible until delivery.[e12199-c1, e12199-c3] In cases with large bony defects and significant tissue herniation—extending from orbital level to the mouth—initial management focuses on airway support and nutritional optimization via feeding tube while awaiting adequate growth for definitive repair.[e12199-c2, e12199-c8, e12199-c9] The rarity of these lesions (surgeons may encounter only one comparable case in their career) necessitates collaborative planning between neurosurgery and craniofacial surgery.[e12199-c5, e12199-c6] Definitive reconstruction typically occurs at 4–5 months of age and involves systematic exposure of the defect, amputation of herniated tissue with brain preservation, and layered reconstruction of the skull base and facial skeleton.[e12199-c10, e12199-c11] Successful repair confirms benign pathology and establishes anatomic barriers critical for normal development.
  1. Fetal MRI detects anterior encephaloceles prenatally, but malignancy cannot be excluded until postnatal pathology.[e12199-c1, e12199-c3]
  2. Large defects with tissue extending from orbit to mouth require feeding tube and oxygen support pending surgical candidacy.[e12199-c2, e12199-c8, e12199-c9]
  3. Definitive repair is typically delayed 4–5 months to allow sufficient growth for safe reconstruction.[e12199-c10]
  4. Surgical technique involves defect exposure, tissue amputation with brain preservation, and layered skull base reconstruction.[e12199-c11]
  5. The extreme rarity of these cases (one per surgical career) mandates collaborative neurosurgical-craniofacial planning.[e12199-c5, e12199-c6]
For patients & families
When Velika Riegel was midway through her pregnancy, doctors found an encephalocele on a fetal MRI — a condition where tissue escapes through an opening in the skull. Her son Zachary was born with a large growth covering the area from his eyes down to his mouth, leaving only a small opening visible. The medical team didn't know until after delivery whether the tissue was cancerous, and they knew complex reconstruction of his face and nasal bridge would be needed. Dr. Charles Stevenson and Dr. Hector Pan worked together on Zachary's case — they had seen this type of condition only once before. Zachary needed a feeding tube and oxygen support, and spent four and a half months in the newborn intensive care unit growing strong enough for surgery. The operation involved carefully removing the tissue, protecting the healthy brain, and rebuilding the layers that hadn't formed properly. The surgery was successful, and the tissue was not cancerous. Velika and Zachary moved closer to Cincinnati Children's to be near his care team.
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A new face for Zakary | Cincinnati Children's
A fetal MRI detected an encephalocele midway through Velika Riegel's pregnancy.
Host summaryThe host summarizes what Dr. Velika Riegel said — not the host's own clinical position0:15 ↗
Zachary had a large opening in the bone between his eyes through which tissue escaped.
Host summaryThe host summarizing the discussion — not the host's own clinical position0:28 ↗
Until Zachary was born, it was unknown whether the encephalocele was cancerous.
Host summaryThe host summarizing the discussion — not the host's own clinical position0:34 ↗
Dr. Charles Stevenson knew following delivery that complex reconstruction of the face and nasal bridge would be required.
clinicalCharles Stevenson0:47 ↗
Dr. Stevenson consulted Dr. Hector Pan because complex facial reconstruction would be needed.
clinicalCharles Stevenson0:47 ↗
The surgeons had seen a case like this only once before.
Host summaryThe host summarizing the discussion — not the host's own clinical position1:12 ↗
Zachary was delivered December 29th, 2016.
Host summaryThe host summarizing the discussion — not the host's own clinical position1:18 ↗
At birth, the growth covered from Zachary's eyes down to his mouth; only a little mouth was visible.
clinicalVelika Riegel1:25 ↗
Zachary needed a feeding tube and supplemental oxygen.
Host summaryThe host summarizing the discussion — not the host's own clinical position1:34 ↗
Zachary spent 4.5 months in newborn intensive care at Cincinnati Children's before he grew enough to have the operation.
Host summaryThe host summarizing the discussion — not the host's own clinical position1:38 ↗
The surgical steps involved exposing the forehead, exposing the defect, amputating the tissue, preserving the normal brain, and reconstructing the normal layers or barriers which had not formed.
clinicalCharles Stevenson1:49 ↗
The operation was successful and the tissue was found not to be a cancerous tumor.
Host summaryThe host summarizing the discussion — not the host's own clinical position2:08 ↗
Zachary and Velika moved to the area to be closer to Cincinnati Children's.
Host summaryThe host summarizing the discussion — not the host's own clinical position2:31 ↗
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