Case Of Aplasia Cutis Congenita Research Articles

Large aplasia cutis congenita of the vertex conservative management.

Aplasia cutis congenita (ACC) of the vertex with bone defect is a rare and begnin anomaly that can involve the epidermis, dermis, and subcutaneous tissues of the scalp with significant bone defect Bajpai and Pal (J Pediatr Surg 38(2):e4, 2003). When associated with skull defect, this rare malformation carries the risk of severe complications such as rupture of the superior sagittal sinus or infections. We report a case of aplasia cutis congenita of the scalp with skull defect measuring 9 × 10cm and an exposed sagittal sinus in a newborn. Both conservative and surgical methods have been proposed to treat this condition. In our case, conservative treatment was planned led to complete epithelization and the patient was healing well at 5years of follow-up. ACC of the vertex with a large scalp defects present a management dilemma Rocha et al. (Clin Case Rep 3(10):841-4, 2015). Based on a review of the literature, we report this case to demonstrate that even for the largest skin and bone defects, an initial conservative approach may allow for complete wound closure without the need for early surgical intervention.

A Case Of Congenital Aplasia Cutis Occupying A Large Area On The Scalp

Aplasia cutis congenita (ACC) is a rare heterogeneous group of diseases characterized by the absence of skin at birth. It affects males and females equally. It is usually located on the scalp and can be in different sizes from 0.5 up to 15 cm. Typically, it is sporadic, although hereditary cases have also been reported. In cases with bone tissue absence under the lesion, the risk of developing complications increases, and surgical intervention may be required. In this article, we presented a rare case of ACC without underlying bone tissue that covers a very large area on the scalp. In this case, the lesion epithelialized with local treatment without need for any reconstructive surgery.

APLASIA CUTIS CONGENITA – A CASE SERIES OF VARIED PRESENTATIONS

Aplasia cutis congenita (ACC) belongs to a heterogeneous group of disorder, characterised by localised absence of skin and in some cases subcutaneous tissues, involving multiple possible body locations. The most common site is the scalp (70%), however any skin site can be affected including the trunk and the extremities . Herein we report a series of 4 cases of aplasia cutis congenita with varied cutaneous manifestations ranging from absence of epidermis to deeper defects extending upto the dermis and subcutaneous tissue. Our rst case presented to us with congenital absence of skin with ulceration and eschar formation of extremities, buttocks and bullae formation over hands and lower back. Clinicopathological diagnosis was made to be aplasia cutis congenita associated with epidermolysis bullosa (Bart’s syndrome). The second case presented with a single, well dened, rhomboid shaped deep ulceration with overlying eschar formation over the vertex extending upto the right parietal region of scalp, diagnosed to be a case of Type 2 Aplasia cutis congenita - Adams-Oliver syndrome. Our third case was a supercial variant of aplasia cutis congenita with linear , erythematous, blanchable atrophied parchment like scar on the back, without any underlying bony deformity. The last was a sporadic case of bitemporal aplasia cutis congenita with thinning of skin over bilateral temporal areas with overlying telangiectasia.

Adams-oliver syndrome with congenital absence of lower lip

Adams-Oliver syndrome is a rare disease characterized by aplasia cutis congenita (ACC) and transverse terminal limb defects (TTLD). Both familial and non-familial cases have been described with predominantly autosomal dominant inheritance and autosomal recessive inheritance in a few. Various systemic involvements lik e central nervous system, cardiovascular system etc. have also been described. We describe here a case of ACC with TTLD characterized by syndactyly of hands and feet without any systemic involvement in a 13 months old female child, without any family history. She also had congenital absence of lower lip, not being described earlier.

A Case of Aplasia Cutis Congenita with Scalp Reconstruction by Tissue Expander in Infancy

A Case of Aplasia Cutis Congenita with Scalp Reconstruction by Tissue Expander in Infancy

NEONATAL APLASIA CUTIS CONGENITA WITH PYOMENINGITIS – A RARE PRESENTATION

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.

A complex scalp resurfacing utilizing Integra® as temporary dressing in aplasia cutis congenita

BACKGROUND: Aplasia cutis congenita is a rare newborn malformation characterized by focal absence of skin. It possesses difficulty in reconstruction surgery for neurosurgeons and plastic surgeons. We report a challenging case of aplasia cutis congenita who received treatment in our center.
 CLINICAL CASE: A 2-year-old boy, presented to Plastic and Reconstructive Surgery, Hospital USM, with bilateral vertex defect with encephalocele who received a series of surgical interventions since 1 month old. Unfortunately, he returned after 2 years with a chronic nonhealing scalp wound associated with dura defect and cerebral spinal fluid leakage. The wound was debrided and the swab culture result showed no organism growth. Part of the wound bed with dura defect was repaired using a small piece of transplanted fascia lata and Integra was applied.
 DISCUSSION: There is scarcity in the medical literature on the reconstructive technique of aplasia cutis congenita. In the case we described here, we successfully managed the wound with multiple application of dermal substitute (Integra) dressing with negative pressure wound therapy and split-thickness skin graft.
 CONCLUSIONS: Management of aplasia cutis congenita with skull defect remains a controversy. Its management varies depends on its pattern and underlying condition. We successfully develop a new simple method in treating scalp accutilizing Integra.

Naujagimio įgimta odos aplazija. Klinikinis atvejis

Santrauka. Įgimta odos aplazija yra retas įgimtas odos ir poodžio defektas, dažniausiai išsidėstęs naujagimio skalpo srityje. Žymiai rečiau šį odos defektą lydi kiti apsigimimai. Tikroji šio apsigimimo priežastis nežinoma. Šiame straipsnyje aprašomas įgimtos odos aplazijos, lydimos kitų apsigimimų – neužakusio šlapimo latako ir Mekelio divertikulo, – klinikinis atvejis ir pateikiama literatūros apžvalga.

Aplasia Cutis Congenita (ACC): An Extremely Rare Case in Childhood

Aplasia cutis congenita (ACC) is a rare disease characterized by a local absence of skin and, in some cases, subcutaneous tissue. Most cases occur in the scalp, but the lesion can occur anywhere in the trunk and extremities. ACC is usually an isolated defect, but can also be associated with other abnormalities. Most reported cases are sporadic, with a few reports of family events. Neither pathogenesis nor etiology are clarified. In most cases, healing is spontaneous, and apart from keeping the lesion clean, no specific treatment is required. This report presents a case of ACC at the head and provides a brief review of the literature.

Oliver Syndrome with Extensive Aplasia Cutis Congenita: A Case Report and Literature Review

Aplasia cutis congenita is a very rare and heterogeneous disease. It is characterized by a localized or extensive skin defect, isolated or associated with damage to the underlying structures, including the bone. The diagnosis is based on the clinical examination. Other abnormalities of various etiologies and severities may be associated with this pathology. Adams-Oliver syndrome is a genetic polymalformative syndrome. The typical form is defined by the presence of Aplasia cutis congenita and limb anomalies. Managing Aplasia cutis congenita, especially in severe cases, is a real challenge. We report a case of a newborn, treated surgically, for extensive occipito-parietal Aplasia cutis congenita. The clinical presentation was suggestive of AdamsOliver syndrome. The evolution was favorable. Describing our experience of managing a case of Aplasia cutis congenita is interesting because: the proposed treatment is very controversial, the rarity and the high morbidity and mortality of Aplasia cutis congenita and Adams-Oliver syndrome. As well as clinical and genetic diversity.

Aplasia cutis congenital. A case report

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.

A Familial Case of Aplasia Cutis Congenita in Two Korean Siblings: A Review of Genetic Aspects.

A Familial Case of Aplasia Cutis Congenita in Two Korean Siblings: A Review of Genetic Aspects.

Our experience with a severe case of aplasia cutis congenita with a large skull defect

Our experience with a severe case of aplasia cutis congenita with a large skull defect

Coexistence of aplasia cutis congenita, faun tail nevus and fetus papyraceus

Aplasia cutis congenita is a disorder of the skin embryonic development characterized by a defect of localized or widespread areas of skin at birth. The lesions are mostly oval, 1-3 cm in diameter, with localization on the parietal part of scalp (60%) and rarely on the face and extremities. Herein, we reported a case of aplasia cutis congenita termly born at 39 weeks of gestation to a 30-year-old mother with bronchial asthma attacks. She was referred for 3 punched-out punctate depressed defective lesions in 0.4 cm's diameter on the vertex covered with necrotic and hemorrhagic crusts. There was a hypertrichotic area consisting of tufts of terminal hair on the lumbosacral area over a sinus tract. Maternal perinatal drugs included aerosol salbutamol sulfate, ipratropium bromide and oral montelukast sodium for bronchial asthma. The pregnancy was firstly started as a di-chorionic, di-amniotic twin gestation, but deteriorated after the fetal resorption of the co-twin in the 20th gestational week resulting in fetus papyraceus. In multi-gestational pregnancies, the presence of the fetus papyraceus or the death of the co-twins should make the neonatologists and dermatologists be aware of the possible cutaneous defects like aplasia cutis congenita. We emphasize that the possibility of this rare entity should be kept in mind in the presence of fetus papyraceus, perinatal drug use, maternal cigarette smoke, or maternal diseases like bronchial asthma in multiple gestations.

Aplasia cutis congenita of eyelid: case report

Aplasia cutis congenita (ACC) is a rare, congenital defect of the skin, which mostly affects the scalp and the trunk. The main complications include bleeding and infection. We present a rare case of ACC that involved solely the eyelid and confirms the effectiveness of conservative therapy.

Fetus Papyraceus: Congenital Pulmonary Anomalies Associated with Congenital Aplasia Cutis on the Surviving Twin

Aplasia cutis congenita (ACC) can be associated with fetus papyraceus. We report here the first case of ACC linked to fetus papyraceus with pulmonary anomalies. At birth, the patient presented with skin lesions of the trunk consisting of well-defined, symmetrically distributed, bilateral atrophic ulcerations. Physical examination was otherwise normal. Persistent bronchospasm occurred at the age of 7months; computed tomography images showed small bilateral pulmonary bullae. At the age of 5years, skin and pulmonary lesions had not extended. Although the mechanisms of ACC linked to fetus papyraceus are unclear, vascular ischemia is strongly suggested, and could explain the bilateral and symmetric congenital skin and lung aplasia.

Multiple aplasia cutis congenita lesions located along Blaschko's lines in a patient with tetralogy of Fallot-A

Aplasia cutis congenita (ACC) is a congenital absence of skin, and a single alopecic lesion on the scalp is the most common form. We present a case of ACC with tetralogy of Fallot-A. Differetial diagnosis included Adams-Oliver syndrome and nevus psiloliparus. Interestingly, our patient showed multiple ACC lesions, which were located along Blaschko's lines. As far as we know, our case is the third case of ACC with tetralogy of Fallot-A. Also, this is the first case of ACC associated with Blaschko's lines.

A case of aplasia cutis congenita in mother infected with human immunodeficiency virus

선천성 피부형성부전증은 출생 시에 피부의 한 부위 또는 여러 부위에 경계가 명확한 궤양성 피부결손을 보이는 드문 질환이다. 산모는 인간면역결핍바이러스 감염이 확진되어 항레트로바이러스제를 복용하였고, 산전 검사상 이상소견은 없었다. 출생 후 환아의 정수리 부위 두피에 피부결손 부위가 관찰되었으며 선천성 피부형성부전증으로 진단되었다. 이에 간단한 문헌 고찰과 함께 보고하는 바이다.

Aplasia cutis congenita du vertex avec atteinte osseuse : synthèse sur la prise en charge et revue de la littérature

Aplasia cutis congenita (ACC) of the vertex is a rare and potentially severe malformation. We report the case of a newborn boy presenting ACC of the vertex with a skull defect. Surgical closure of the skin was performed 5 days following birth, which allowed skin healing. The skull spontaneously remodelled perfectly 6 months later. When associated with skull defect, this rare malformation characterized by the absence of skin carries the risk of severe complications such as rupture of the superior sagittal sinus or infections. Based on a review of the literature, we purpose an overview of the medical and surgical management of vertex ACC, which depends on the size of the skin defect and the presence of a skull defect.

Systemic aplasia cutis congenita: A case report and review of the literature

Aplasia cutis congenita (ACC) belongs to a heterogeneous group of conditions characterized by a congenital absence of skin, usually on the vertex of the scalp. It can occur as an isolated defect or can be associated with a number of other congenital anomalies. Two cases of systemic ACC of a more severe and extensive type were recently reported. Here, we describe the third case of systemic ACC and review the available literature. A female infant was born with an extensive defect of the skin, a skull defect, imperforate hymen, and some other anomalies. She died soon after birth probably due to asphyxia and dehydration. We also compared the pathologic findings of the current case with those of the other two previously reported cases. This case suggests that systemic ACC might be recognized as a new syndrome. A limitation is that there is only 1 case, and it is difficult to gain a deeper understanding of its etiology and diagnostic criteria.