Cutis Aplasia: Perioperative Management and Case Report

2012 ◽  
Vol 21 (3) ◽  
pp. 212-215
Author(s):  
Steven M. Levine ◽  
Derek D. Reformat ◽  
Charles H. Thorne
Keyword(s):  
Perioperative Management ◽  
Wound Care ◽  
Surgical Techniques ◽  
Full Thickness ◽  
Aplasia Cutis Congenita ◽  
Rare Congenital Disorder ◽  
Scalp Defect ◽  
Life Threatening ◽  
Cutis Aplasia ◽  
Aplasia Cutis

Aplasia cutis congenita, a rare congenital disorder involving defects of some or all of the layers of the cranium, is associated with potential life-threatening complications. Although treatment involves both nonsurgical and surgical techniques, the importance of perioperative management cannot be overstressed. A multidisciplinary team, including personnel from nursing, neonatology, pediatrics, radiology, neurosurgery, and plastic surgery services, diagnosed aplasia cutis congenita and planned local wound care, surgical correction, and prevention of potentially life-threatening complications in a 1-day-old boy with a 6×5-cm full-thickness scalp defect.

Approach in management of Aplasia Cutis Congenita in a newborn with Adam Oliver Syndrome

2020 ◽  
Vol 01 ◽  
Author(s):  
Ihab H. El Kadry ◽  
Tarek Koratum ◽  
Laila Obaid
Keyword(s):  
High Morbidity ◽  
Aplasia Cutis Congenita ◽  
Adjacent Tissue ◽  
Rare Congenital Disorder ◽  
Scalp Defect ◽  
Talipes Equinovarus ◽  
Csf Leakage ◽  
Choice Of Treatment ◽  
Newborn Girl ◽  
Aplasia Cutis

: Aplasia cutis congenita (ACC) is a rare congenital disorder characterized by absence of skin and adjacent tissue that usually affects the scalp. Large scalp defect in ACC with adjacent tissue anomalies is associated with high morbidity and mortality. The management of these lesions can be challenging. The treatment can be either conservative, surgical, or combined. Full-term newborn girl delivered with a huge scalp defect. The dura mater was exposed but intact and there was no cerebrospinal fluid (CSF) leakage. She also had bilateral talipes equinovarus and terminal reduction of multiple toes in the left foot. Genetic testing was positive for Adam Oliver Syndrome. Conservative medical management was initiated at birth and continued until discharge at 4 weeks of age. The patient had regular follow-up with plastic surgery and neurosurgery teams. At 11 months old, the defect became smaller, hairless with good granulation tissue, but still soft and compressible. Large scalp midline ACC is a rare, but potentially devastating disease when involved adjacent tissue. The choice of treatment is not straightforward and it is mainly based upon size, depth, and the risk of pre and post-treatment complications. Conservative management still an option with very complex ACC.

scholarly journals Multiple aplasia cutis congenita type V and fetus papyraceous: a case report and review of the literature

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
V. Thadchanamoorthy ◽  
Kavinda Dayasiri ◽  
M. Thirukumar ◽  
N. Thamilvannan ◽  
S. H. Chandraratne
Keyword(s):  
Rare Condition ◽  
The Body ◽  
Sri Lankan ◽  
Aplasia Cutis Congenita ◽  
Skin Defects ◽  
Underlying Causes ◽  
Cutis Aplasia ◽  
Contracture Formation ◽  
Type V ◽  
Aplasia Cutis

Abstract Background Aplasia cutis congenita is regarded as congenital focal absence of skin in the newborn, and occurrence of more than three similar skin defects is rare. The etiology is thought to be multifactorial, and precise etiopathogenesis is unknown. Case presentation A 13-day-old newborn Sri Lankan Tamil girl was referred to the dermatologic clinic with multiple skin defects at birth. There were six lesions on the body, and two of them had healed during intrauterine period, leaving scars. This was a second twin of her pregnancy. Her first twin fetus had demised before 19 weeks of pregnancy and was confirmed to be fetus papyraceous based on ultrasound-guided fetal assessment. The said child was thoroughly investigated and found to have no other congenital abnormalities. Chromosomal studies yielded normal findings. She was treated with tropical antibacterial ointment, and all lesions resolved spontaneously within 4 weeks, leaving scars. Physiotherapy was commenced to prevent contracture formation, and follow-up was arranged in collaboration with the plastic surgical team. Conclusions Aplasia cutis congenita is a rare condition of uncertain etiology, but consanguinity may play a role. This report described a newborn with type V cutis aplasia congenita in whom the diagnosis was confirmed based on clinical features and revision of antenatal history. The management depends on the pattern, extent, location, severity, underlying causes, and associated anomalies.

Aplasia cutis congenita. A case of scalp defect repair using two opposing bipedicled local flaps

2010 ◽  
Vol 63 (3) ◽  
pp. e242-e244 ◽  
Author(s):  
J.K. O'Neill ◽  
M. Carter ◽  
R.P. Warr
Keyword(s):  
Local Flaps ◽  
Aplasia Cutis Congenita ◽  
Scalp Defect ◽  
Defect Repair ◽  
Aplasia Cutis

Adult thermal burns

2021 ◽  
pp. 155-178
Author(s):  
Jeremy Rawlins ◽  
Isabel Jones
Keyword(s):  
Wound Care ◽  
Surgical Techniques ◽  
Patient Treatment ◽  
Burn Wound ◽  
Severe Injuries ◽  
Burn Care ◽  
The Face ◽  
Life Threatening ◽  
The Many ◽  
Dermal Matrices

Adult burn injuries are heterogeneous in their aetiology and in their severity, and in the many ways in which they affect the burns patient. Treatment should be initiated promptly, ensuring that other life-threatening injuries are dealt with, and that expert burn care is delivered to the patient. First aid followed by emergency management of the patient, fluid resuscitation, and specialist wound care ensures the patient is given the very best opportunity for full recovery and survival. The burn wound may be managed non-surgically, but for deeper and more severe injuries, surgical debridement and wound cover with grafts, cell suspensions, dermal matrices, or flaps is required. Areas of special attention include the face, hands, and perineum—all functionally and aesthetically important, and each with specific dressing needs and surgical techniques that optimize good outcomes. Scar management and physiotherapy are crucial components of adult burn care, with many therapies applied concurrently to ensure good functional and aesthetic recovery. For those patients with ongoing scar and contracture difficulties, scar therapies and reconstructive burns surgery offer the chance of better function and appearance even many years after the original burn. The journey to recovery would not be complete, however, without important psychosocial input for the burns patient, as the psychological scars are often just as painful as the physical ones.

NEONATAL APLASIA CUTIS CONGENITA WITH PYOMENINGITIS – A RARE PRESENTATION

2021 ◽  
pp. 51-52
Author(s):  
Satyendra Satyendra ◽  
Jaiprakash Narayan ◽  
Jeffy Joy
Keyword(s):  
Case Report ◽  
Female Child ◽  
Congenital Disorder ◽  
Congenital Absence ◽  
Unknown Etiology ◽  
Rare Presentation ◽  
Aplasia Cutis Congenita ◽  
New Born ◽  
Rare Congenital Disorder ◽  
Aplasia Cutis

Background - Aplasia cutis congenita is a congenital absence of skin in new born. It can be anywhere over body. Aplasia cutis congenita is a rare congenital disorder of skin. We are presenting a case of aplasia cutis congeni Case report – ta in female child associated with pyomeningitis. Conclusion - Aplasia cutis congenita, its rarity and unknown etiology is the reason of reporting. It is rarely reported a case of Aplasia cutis congenita with association of pyomeningitis.

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.

Aplasia cutis congenital. A case report

2019 ◽  
pp. 79-80
Keyword(s):  
Case Report ◽  
Subcutaneous Tissue ◽  
Congenital Disorder ◽  
Solitary Lesion ◽  
Aplasia Cutis Congenita ◽  
Multiple Lesions ◽  
Rare Congenital Disorder ◽  
Aplasia Cutis

Aplasia cutis congenita (ACC) is a rare congenital disorder that commonly affects the scalp. In this disease, some parts of the skin with or without underlying structures were not formed at birth. The aplastic lesion always presents as a solitary lesion; however, multiple lesions also were reported. In more severe cases, deeper structures, such as the subcutaneous tissue, bone, and dura, can be affected. In this study, we report a case of ACC and its management.

Management of large scalp and skull defects in a severe case of Adams-Oliver syndrome

2009 ◽  
Vol 4 (6) ◽  
pp. 523-527 ◽  
Author(s):  
Mostafa El Khashab ◽  
Samuel T. Rhee ◽  
Sean D. Pierce ◽  
Yasmin El Khashab ◽  
Farideh Nejat ◽  
...  
Keyword(s):  
Skin Grafting ◽  
Severe Case ◽  
Skull Defects ◽  
Rare Congenital Disorder ◽  
Threatening Condition ◽  
Dermal Regeneration Template ◽  
Life Threatening ◽  
Dermal Regeneration ◽  
Cutis Aplasia ◽  
Large Skin

Adams-Oliver syndrome is a rare congenital disorder that includes congenital scalp and skull defects, variable degrees of terminal transverse limb anomalies, and cardiac malformations. Cutis aplasia occurring in 75% of patients is a potentially life-threatening condition. Large skin defects that cannot be closed primarily present a management dilemma, and may require skin grafting or flaps, or a combination of both operative and conservative modalities. The authors' experience in management of huge scalp and bone defects with the Integra Dermal Regeneration Template and regular dressing changes showed good scalp repair and no serious complications attributed to this approach.

Scalp Defect (Giant Aplasia Cutis Congenita of the Scalp in a Newborn)

2015 ◽  
Vol 12 (3) ◽  
Author(s):  
Albahri Z ◽  
Krylova K ◽  
Al Mawiri A ◽  
Bartonova J ◽  
Stefackova S ◽  
...  
Keyword(s):  
Aplasia Cutis Congenita ◽  
Scalp Defect ◽  
Aplasia Cutis

scholarly journals Adams-Oliver syndrome: a case with full expression

2016 ◽  
Vol 8 (2) ◽  
Author(s):  
Amir Dehdashtian ◽  
Masoud Dehdashtian
Keyword(s):  
Weight Gain ◽  
Male Infant ◽  
Multiple Organ ◽  
Limb Defects ◽  
Aplasia Cutis Congenita ◽  
Scalp Defects ◽  
Major Organ ◽  
Cutis Aplasia ◽  
Poor Weight Gain ◽  
Aplasia Cutis

Adams-Oliver syndrome (AOS) is characterized by the combination of congenital scalp defects (aplasia cutis congenita) and terminal transverse limb defects of variable severity. It is believed that Adams-Oliver syndrome without major organ abnormalities does not necessarily alter the normal lifespan. We present a case without detectable major organ abnormality contrary to life but with poor weight gain. A male infant with scalp and skin cutis aplasia, generalized cutis aplasia, dilated veins over scalp and trunk, hypoplastic toes and nails of feet, glaucoma, poor feeding and poor weight gain. This report shows a case of AOS without major multiple organ abnormalities but with poor feeding and abnormal weight gain that may be alter the normal lifespan.

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