  Case of the Week - Patient Summary 12571Peer Reviewed and Certified - | |
| Demographics: an infant y.o. boy | |
| History & Chief complaint: | |
| Presentation: 3 month old male presents for follow up after myelomeningocele repair and ventricular shunt placement
Birth history: Born at 36 weeks gestation via spontaneous vaginal delivery to a 23 y/o mother. Birth weight was 2790 grams and initial APGARS or 7 and 9. Social/Family History: Noncontributary Past medical history: Myelomeningocele repaired on day 2 of life and VP shunt placed at 2 weeks. | |
| Physical exam and Laboratory: | |
| Physical exam: Healing incision scar on lumbar spine and mild supination deformity and minimal spontaneous motion of right foot. Otherwise within normal limits.
| |
| Summary of Findings: | |
| Plain Film Findings: Skull: Well-defined lucent areas of nonossified fibrous bone in the calvaria with "copper beaten" appearance | |
| Differential Diagnosis: | |
| Cleido-cranial dysostosis
hyperparathyroidism histiocytosis X | |
| Diagnosis: | |
 Lacunar Skull | |
| Patient Specific Discussion: (Also Read the Disease Discussion) | |
| Lacunar skull, (a.k.a. Luckenschaedel and crainolacunia), is a finding seen in association with myelomeningocele, myelocele, and encephalocele. It is the result of dysplasia of the membranous bones of the calvarium and can be seen as early as 8 months gestation and usually persists until the age of 1-3 months but typically has resolved by age 6 months. The normal development of the membranous skull requires distension of the developing brain and ventricular system. The skull develops from ossification centers in each cranial plate. As the brain expands, collagen bundles in each center are drawn out in an orderly radial manner. Ossification of the collagen bundles also takes place radially. In patients with lacunar skull, the collagen bundles are disorganized and form whorls with varying thickness of fibrous tissue between them. Ossification of the collagen bundles occurs in a disorderly manner producing lacunar skull. This is independent of the presence of hydrocephalus. It should not be confused with normal convolutional markings seen in the vault during the period of rapid brain growth (3 to 7 years).
| |
| Lacunar skull, (a.k.a. Luckenschaedel and crainolacunia), is a finding seen in association with myelomeningocele, myelocele, and encephalocele. It is the result of dysplasia of the membranous bones of the calvarium and can be seen as early as 8 months gestation and usually persists until the age of 1-3 months but typically has resolved by age 6 months. The normal development of the membranous skull requires distension of the developing brain and ventricular system. The skull develops from ossification centers in each cranial plate. As the brain expands, collagen bundles in each center are drawn out in an orderly radial manner. Ossification of the collagen bundles also takes place radially. In patients with lacunar skull, the collagen bundles are disorganized and form whorls with varying thickness of fibrous tissue between them. Ossification of the collagen bundles occurs in a disorderly manner producing lacunar skull. This is independent of the presence of hydrocephalus. It should not be confused with normal convolutional markings seen in the vault during the period of rapid brain growth (3 to 7 years).
| |
|