Congenital scalp defects: aplasia cutis congenita

1975 ◽  
Vol 42 (1) ◽  
pp. 32-36 ◽  
Author(s):  
Edward I. Kosnik ◽  
Martin P. Sayers
Keyword(s):  
Primary Closure ◽  
Trisomy 13 ◽  
Normal Children ◽  
Aplasia Cutis Congenita ◽  
Content Type ◽  
Scalp Defects ◽  
Scalp Defect ◽  
Rare Lesion ◽  
Aplasia Cutis ◽  
Fine Print

✓ The authors discuss the rare lesion of congenital scalp defect, both partial and full thickness. The majority occur in children who are otherwise normal, but a few are seen in children who have many concomitant anomalies. A high percentage of the multiple anomaly cases were found to have trisomy 13–15. Etiology of these lesions is not clear, but there seems to be an inherited component. Treatment of otherwise normal children is conservative for the smaller lesions, with excision and primary closure when possible for the larger ones.

Nonsurgical approach to congenital scalp and skull defects

1982 ◽  
Vol 56 (5) ◽  
pp. 711-715 ◽  
Author(s):  
Kamel F. Muakkassa ◽  
Robert B. King ◽  
David B. Stark
Keyword(s):  
Natural Course ◽  
Aplasia Cutis Congenita ◽  
Content Type ◽  
Skull Defects ◽  
Aplasia Cutis ◽  
Fine Print

✓ The management of congenital scalp and skull defects, as generally advocated, is surgical. The authors report such a case that was treated conservatively. At her 3-year follow-up review, the patient's scalp and skull defects and other associated cutaneous defects were fully reconstituted. Such a nonoperative approach, while rarely reported, emphasizes the natural course that some of these lesions may follow. The literature on aplasia cutis congenita is briefly reviewed.

Aplasia cutis congenita

1996 ◽  
Vol 85 (1) ◽  
pp. 170-173 ◽  
Author(s):  
Dennis D. Nichols ◽  
Anthony G. Bottini
Keyword(s):  
Surgical Approach ◽  
Surgical Intervention ◽  
Tissue Expansion ◽  
Congenital Defect ◽  
Aplasia Cutis Congenita ◽  
Content Type ◽  
Aplasia Cutis ◽  
Fine Print

✓ The authors report the use of intraoperative tissue expansion in the treatment of a neonate with aplasia cutis congenita, a congenital defect of the scalp and skull. The case for immediate surgical intervention is presented, and intraoperative tissue expansion and cranioplasty are recommended as components of an effective surgical approach.

Ventricular volume change in childhood

2002 ◽  
Vol 97 (3) ◽  
pp. 584-590 ◽  
Author(s):  
Chris Xenos ◽  
Spyros Sgouros ◽  
Kalyan Natarajan
Keyword(s):  
Statistical Difference ◽  
Linear Regression Analysis ◽  
Ventricular Volume ◽  
Intracranial Volume ◽  
Normal Children ◽  
Content Type ◽  
Ventricular Volumes ◽  
Significant Difference ◽  
The Mean ◽  
Fine Print

Object. The aim of this study was to construct a model of age-related changes in ventricular volume in a group of normal children ages 1 month to 15 years, which could be used for comparative studies of cerebrospinal fluid circulation disorders and cerebral atrophy developmental syndromes. Methods. A magnetic resonance imaging—based segmentation technique was used to measure ventricular volumes in normal children; each volume was then plotted against the child's age. In addition, intracranial volumes were measured and the ratio of ventricular to intracranial volume was calculated and plotted against age. The study group included 71 normal children, 39 boys and 32 girls, whose ages ranged from 1 month to 15.3 years (mean 84.9 months, median 79 months). The mean ventricular volume was 21.3 cm3 for the whole group, 22.7 cm3 in boys and 19.6 cm3 in girls (p = 0.062, according to t-tests). The mean ventricular volume at 12 months for the whole group was 17 cm3 (20 cm3 in boys and 15 cm3 in girls), representing 65% of the volume achieved by 15 years of age (87% in boys and 53% in girls). The volume increased by a factor of 1.53, to 26 cm3 (23 cm3 in males and 28 cm3 in females, increase factors of 1.15 and 1.86, respectively) at 15 years of age. The change in ventricular volume with age is not linear, but follows a segmental pattern. These age periods were defined as: 0 to 3, 4 to 6, 7 to 10, and 11 to 16 years. A statistical difference based on sex was only demonstrated in the first 6 years of life. The mean ventricular volume for the first 6-year period was 22.4 cm3 in boys and 15.7 cm3 in girls, and the difference was significant for the two sexes (linear regression analysis for age and sex, significant according to analysis of variance regression at 0.007, p = 0.108 for age, p = 0.012 for sex). Thereafter, there was no significant difference in ventricular volume between boys and girls with further growth. The ratio of ventricular volume to intracranial volume was 0.0175 for the whole group, 0.017 in boys and 0.018 in girls (p = 0.272, according to t-tests). At 12 months of age the ratio was 0.019; it stabilized to 0.015 at 8 years of age, and increased to 0.018 at 15 years of age. No statistical difference based on sex was demonstrated with growth. Conclusions. The ventricular volume in normal children increases with age by a factor of 1.5; the increase is in a nonlinear segmental pattern. Boys have significantly higher ventricular volumes only in the first 6 years of life. The ventricular/intracranial volume ratio remains stable throughout childhood.

Aplasia Cutis Congenita Complicated by Sagittal Sinus Hemorrhage

PEDIATRICS ◽  
1980 ◽  
Vol 66 (6) ◽  
pp. 948-950 ◽  
Author(s):  
Brian M. Schneider ◽  
Robert A. Berg ◽  
Allen M. Kaplan
Keyword(s):  
Surgical Repair ◽  
Aplasia Cutis Congenita ◽  
Sagittal Sinus ◽  
Scalp Defects ◽  
Aplasia Cutis

An infant with aplasia cutis congenita complicated by sagittal sinus hemorrhage is described. In cases of large defects, plastic surgical repair has been recommended for prevention of hemorrhage and infection. The complication of sagittal. sinus hemorrhage should be considered in patients with large midline scalp defects, especially when associated with absence of the underlying cranium.

Epileptic Encephalopathy in Adams–Oliver Syndrome Associated to a New DOCK6 Mutation: A Peculiar Behavioral Phenotype

2018 ◽  
Vol 49 (03) ◽  
pp. 217-221 ◽  
Author(s):  
Livia Pisciotta ◽  
Valeria Capra ◽  
Andrea Accogli ◽  
Thea Giacomini ◽  
Giulia Prato ◽  
...  
Keyword(s):  
Epileptiform Activity ◽  
Epileptic Encephalopathy ◽  
Compound Heterozygosity ◽  
Limb Defects ◽  
Aplasia Cutis Congenita ◽  
Scalp Defects ◽  
Brain Malformations ◽  
Limb Malformations ◽  
Electroencephalogram Eeg ◽  
Aplasia Cutis

AbstractAdams–Oliver syndrome (AOS) is characterized by a combination of congenital scalp defects (aplasia cutis congenita) and terminal transverse limb malformations of variable severity. When neurological findings are present, patients are reported as AOS variants. We describe a child with compound heterozygosity of the DOCK6 gene, aplasia cutis, terminal transverse limb defects, cardiovascular impairment, intellectual disability, and brain malformations with intracranial calcifications. He suffers from a severe refractory epileptic encephalopathy characterized by polymorphic seizures with prolonged periods of electroencephalogram (EEG), continuous epileptiform activity related to clinical inactivity, and closure of eyes with an “ON-OFF” behavior.

Multiple Aplasia Cutis Congenita Lesions of the Scalp: A Case Study

2020 ◽  
Vol 39 (2) ◽  
pp. 83-91
Author(s):  
Mary Whalen
Keyword(s):  
Chromosomal Abnormalities ◽  
Rare Condition ◽  
Trisomy 13 ◽  
Aplasia Cutis Congenita ◽  
Live Births ◽  
Patau Syndrome ◽  
Males And Females ◽  
Aplasia Cutis

Aplasia cutis congenita (ACC) is a rare condition that presents at birth as an absence of skin that does not usually involve underlying structures. Occurring in 3/10,000 live births, ACC is evenly distributed between males and females; the risk of ACC increases to 7 percent in consanguineous marriages. Up to 86 percent of lesions are found on the scalp in the midline vertex position. Lesions can also be found on the trunk and limbs, as with Adams-Oliver syndrome or accompanying epidermolysis bullosa. ACC is associated with chromosomal abnormalities and 35–50 percent of the time with trisomy 13 (Patau syndrome). This case study presents an infant with multiple ACC lesions of the scalp. The pathophysiology, treatment, potential long-term complications, and nursing considerations are discussed.

Postirradiation sarcomatous transformation of a pituitary adenoma: a combined pituitary tumor

1982 ◽  
Vol 56 (2) ◽  
pp. 283-286 ◽  
Author(s):  
Steve Pieterse ◽  
Trevor A. R. Dinning ◽  
Peter C. Blumbergs
Keyword(s):  
Pituitary Adenoma ◽  
Pituitary Tumor ◽  
The Other ◽  
Content Type ◽  
Large Size ◽  
Irradiation Growth ◽  
Chromophobe Adenoma ◽  
Rare Lesion ◽  
Latent Interval ◽  
Fine Print

✓ A case is reported in which a fibrosarcoma developed 20 years after irradiation of a pituitary chromophobe adenoma. This rare lesion, like most of the other documented postirradiation pituitary sarcomas, was a combination of fibrosarcoma and pituitary adenoma. These lesions tend to behave in a characteristic fashion, with onset after a long latent interval following irradiation, growth to a large size, and failure to metastasize.

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