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Journal of Indian Association of Pediatric Surgeons
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LETTERS TO THE EDITOR
Year : 2018  |  Volume : 23  |  Issue : 3  |  Page : 175-177
 

Aplasia cutis congenita in a newborn: A rare case


1 Department of Pediatric Surgery, SMS Medical College, Jaipur, Rajasthan, India
2 Department of Obstetrics and Gynecology, SMS Medical College, Jaipur, Rajasthan, India

Date of Web Publication4-Jul-2018

Correspondence Address:
Dr. Aditya Pratap Singh
Near The Mali Hostel, Main Bali Road, Falna, Pali, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.JIAPS_220_17

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How to cite this article:
Singh AP, Gupta AK, Pardeshi R, Shukla AK. Aplasia cutis congenita in a newborn: A rare case. J Indian Assoc Pediatr Surg 2018;23:175-7

How to cite this URL:
Singh AP, Gupta AK, Pardeshi R, Shukla AK. Aplasia cutis congenita in a newborn: A rare case. J Indian Assoc Pediatr Surg [serial online] 2018 [cited 2019 Sep 21];23:175-7. Available from: http://www.jiaps.com/text.asp?2018/23/3/175/235902




Sir,

A parent of a male newborn was presented to us with the complaint of skin defect over the trunk. The baby was term with birth weight of 2.6 kg and product of nonconsanguineous marriage. The baby was the second child for the parents without any demise. Antenatal history was unremarkable with single live fetus and without fetus papyraceous. There was no history of any in vitro fertilization, multiple pregnancy, and abortion. On examination, there was skin defect involving the lower chest and upper abdomen bilateral symmetrically without any discharge [Figure 1]. There were thin transparent membranes on both sides, and ribs were visible under the defect. These were full-thickness defect with the involvement of muscles bilaterally. The size of defect was 10 cm × 6 cm on both sides. Both defects were connected by a band-like defect. There were no other organ abnormalities. Routine blood investigations were within normal limits including complete blood count, renal function test, serum electrolytes, and liver function test. Chest X-ray and abdomen were normal. Ultrasonography abdomen and echocardiography were normal. Affected area was treated by mupirocin and Vitamin A + D ointment. The patient was lost to follow-up, so could not comment on the results.
Figure 1: Photograph showing newborn with aplasia cutis congenita

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Aplasia cutis congenita (ACC) is an uncommon disorder presented at birth, which commonly involves the skin of the scalp and other areas but may involve the subcutaneous tissue, bone, and dura mater. The most common presentation is the solitary lesion on the scalp, but in our case, the lesion was on the trunk. The cause is not clear, but numerous factors have been considered as possible causes of ACC, including placental infarcts, genetics, teratogenic substances, intrauterine infections and trauma, vascular compromise, amniogenesis, adhesions of the amniotic membrane to the fetal skin, amniotic rupture sequence, ectodermal dysplasia, imperfect neural tube closure, and maternal intrapartum drug use.[1] The recent increase in the use of in vitro fertilization techniques has caused a marked rise in the incidence of multiple gestation pregnancies.[2]

The cause of the symmetrical type of aplasia cutis is a vascular disruption inducing abnormal dermoepidermal development or cutaneous defect through ischemic and thrombotic events. The intrauterine death of one of the fetuses should cause the release of the thrombosis-promoting material from the dead fetus. These substances can cause placental infarction, disseminated intravascular coagulation, and cutaneous lesions.

Other abnormalities such as hepatic hematoma, duodenal atresia, and biliary atresia may be observed. These findings prove hypothesis of the vascular origin of the disorder. Our patient did not have any other organ abnormalities.

It is seen after fetus papyraceous in multiple pregnancies. Whereas, in our case, there is no such finding. Affected patients show linear areas of absence of skin that have bilateral pattern of distribution along the flanks and the lateral aspect of the limbs. Therefore, prenatal ultrasound has been a great help in allowing a better understanding of this disorder. However, in our case, prenatal ultrasound was normal. In familial cases, genetic counseling is needed. Prenatal diagnosis through amniocentesis and chorionic villous sampling can be helpful to rule out junctional and dystrophic epidermolysis bullosa.

Besides isolated presentation, it may manifest with other developmental malformations of cardiovascular, gastrointestinal, genitourinary, and central nervous systems (CNSs), and syndromes such as Adams–Oliver syndrome, SCALP syndrome (nevus sebaceus, CNS malformations, ACC, limbal dermoid, and pigmented nevus), Opitz syndrome, and chromosomal disorders are associated with this lesion.[3]

Histological details are available in very few reports. Histological features vary depending on the depth and duration of aplasia. Ulcers are seen at birth. After healing, the epidermis appears flattened with proliferation of fibroblasts within a connective tissue stroma. Total absence of the epidermal appendages remains a characteristic feature.

Conservative treatment is the most popular regimen. Although there have been reports of the surgical treatment of ACC, the use of the scalp as a donor site has rarely been reported.[4]

An ACC scalp defect can be an indicator of internal organ involvement and increase the risk of complications.[4] The occurrence of complications can increase the likelihood of a poor outcome. Attention should be paid to the prevention and treatment of complications such as infection, bleeding, electrolyte imbalance from increased epidermal water loss, nutritional deficiency from chronic blood loss, and pain at wound dressing.[4]

The selection of treatment depends on the condition of the infant and the complications that occur. Multiple treatment regimens have been reported for cases of ACC.[4]

Cerebral damage, bleeding, and infections should be carefully monitored when the patient is being conservatively treated with dressing changes. A multidisciplinary approach can be useful. Treatment for ACC is currently controversial and may be conservative, including dressings, surgery, or a combination of both. There are a range of conservative dressing options, such as moist dressing, burn cream, dressing with silver sulfadiazine cream, and dressings containing epidermal growth factor. Skin grafting and local flaps are the predominant surgical treatment options for ACC. Skin graft is recommended for large facial defects that are at risk of sagittal sinus thrombosis and cerebral hemorrhage. Surgical management should be selected for large and deep lesions that are not healed through conservative treatment.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgment

We would like to thank Dr. Maryem Ansari, Assistant Professor, MD in Pathology, SMS Medical College Jaipur, Rajasthan, India.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Blouin MM, Bernard J, Caron F, Auger I. Aplasia cutis congenita of the trunk and scalp associated with fetus papyraceus. Int J Dermatol 2011;50:733-5.  Back to cited text no. 1
    
2.
Ustüner P, Dilek N, Saral Y, Ustüner I. Coexistence of aplasia cutis congenita, faun tail nevus and fetus papyraceus. J Dermatol Case Rep 2013;7:93-6.  Back to cited text no. 2
    
3.
Lam J, Dohil MA, Eichenfield LF, Cunningham BB. SCALP syndrome: Sebaceous nevus syndrome, CNS malformations, aplasia cutis congenita, limbal dermoid, and pigmented nevus (giant congenital melanocytic nevus) with neurocutaneous melanosis: A distinct syndromic entity. J Am Acad Dermatol 2008;58:884-8.  Back to cited text no. 3
    
4.
Tempark T, Shwayder TA. Aplasia cutis congenita with fetus papyraceus: Report and review of the literature. Int J Dermatol 2012;51:1419-26.  Back to cited text no. 4
    


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