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ONLINE ONLY - CASE REPORT
Year : 2022  |  Volume : 6  |  Issue : 1  |  Page : 57

Fetal varicella syndrome: A rare case report and literature review


Department of Dermatology, SKNMC, Pune, Maharashtra, India

Date of Submission28-Aug-2020
Date of Decision17-Mar-2021
Date of Acceptance29-Mar-2021
Date of Web Publication25-Feb-2022

Correspondence Address:
Abhishek S Patokar
Department of Dermatology, SKNMC, Pune, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/CDR.CDR_118_20

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  Abstract 


Fetal varicella syndrome (FVS) is an extremely rare condition of the newborn, characterized by cutaneous scars, limb defects, ocular, and central nervous system abnormalities. It follows maternal varicella infection during early pregnancy. We are reporting a 4-day-old female child who presented with a linear, depressed, erythematous, localized scarring over the right knee with hypoplasia of the right lower limb and malformed right great toe. Clinical presentation of her right foot was resembling congenital talipes equinovarus. Mother had a history of varicella during the first trimester of pregnancy. Ocular examination revealed subretinal hypopigmented exudation with scanty vitreous hemorrhage and tunica vasculosa lentis seen on indirect ophthalmoscopy in the right eye. Ultrasonic A/B scan of the right eye was suggestive of microphthalmia. We confirmed the diagnosis of FVS based on characteristic history, clinical features, and varicella-zoster IgG antibodies were positive.

Keywords: Congenital, congenital talipes equinovarus, hypoplasia, microphthalmia, scarring, varicella


How to cite this article:
Patokar AS, Holani AR, Khatu S, Chaudhari N, Palaskar N. Fetal varicella syndrome: A rare case report and literature review. Clin Dermatol Rev 2022;6:57

How to cite this URL:
Patokar AS, Holani AR, Khatu S, Chaudhari N, Palaskar N. Fetal varicella syndrome: A rare case report and literature review. Clin Dermatol Rev [serial online] 2022 [cited 2022 May 22];6:57. Available from: https://www.cdriadvlkn.org/text.asp?2022/6/1/57/338580




  Introduction Top


Fetal varicella syndrome (FVS) is an extremely rare complication of maternal varicella infection occurring in <2% of the babies born to women infected with varicella between the 7th and 28th weeks of pregnancy. The incidence of FVS is 0.91% when women contract varicella in the first 20 weeks of gestation. The newborn may present with low-birth-weight, cutaneous scars, localized absence of skin on a limb, papular lesions, hypoplasia of one or more limbs, and malformed digits along with various ocular and central nervous system (CNS) abnormalities. The segmental distribution of scars probably reflects damage to the fetal nervous system by the neurotrophic virus. We are reporting a 4-day-old female child because of the paucity of reports from all over the world. There are only a few cases on FVS reported from India [Table 1].
Table 1: Cases of fetal varicella syndrome reported from India

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  Case Report Top


A 4-day-old female child was referred to the dermatology outpatient department in the view of a scar over the right lower limb since birth. She was born of a second-degree consanguious marriage to an unregistered 22-year-old female at full term by normal vaginal delivery. The baby weighed 2235 g at birth. Her APGAR score was 7 and 9 at 1 min and 5 min after birth, respectively. The baby had no history of vesiculobullous lesions or trauma before this scar. After a detailed history, mother revealed that she had an episode of varicella during the 12th week of pregnancy, for which she did not seek any medical advice. Mother did not get any of her antenatal check-ups done and directly came to our hospital in the second stage of labor. Hence, data about fetal anomaly scan or TORCH battery were lacking. The previous delivery was a full-term normal vaginal delivery, and the baby was healthy.

Cutaneous examination of the baby revealed a linear, irregular, depressed, erythematous scar with peripheral hypopigmentation measuring 7 cm horizontally and 5 cm vertically over the right knee. The scar was extending toward the shin of the right leg [Figure 1]. It was associated with right lower limb hypoplasia and malformed right great toe [Figure 2]. The left lower limb was completely normal [Figure 3]. The hair and nail examination was normal. On anthropometric examination on day 4, weight is 2115 g, length is 45 cm, and head circumference is 31 cm, which suggests low-birth weight baby.
Figure 1: Irregular, depressed, erythematous scar over right knee

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Figure 2: Right lower limb hypoplasia with malformed right toe. It also shows medial and posterior foot crease, curved lateral border, and rigid equinus seen in congenital talipes equinovarus

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Figure 3: Comparison of affected right lower limb with normal left lower limb

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The physical examination of the right foot revealed medial and posterior foot skin creases, empty heel, talar head coverage, curved lateral border, and rigid equinus which was suggestive of congenital talipes equinovarus (CTEV) [Figure 2]. There were no skeletal deformities detected on the X-ray of right foot.

Ocular examination of the right eye by indirect ophthalmoscopy revealed an elevated bossellated yellowish-white lesion in the superior quadrant of the retina with surrounding scarring suggestive of subretinal hypopigmented exudation with scanty vitreous hemorrhage and tunica vasculosa lentis, while the left eye was completely normal. The ultrasonic A/B scan of the right eye was suggestive of microphthalmia [Figure 4]. There were no CNS abnormalities detected on ultrasonography of anterior fontanelle.
Figure 4: The ultrasonic A/B scan of the right eye was suggestive of “microphthalmia”

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The patient's blood and urine parameters were within normal limits. Her blood group was B positive. The VDRL test and ELISA for HIV were nonreactive. No abnormality was detected on the chest X-ray. No abnormality was detected in the ultrasonography of abdomen and pelvis. The varicella-zoster virus (VZV) DNA was not detected by real-time polymerase chain reaction. The varicella-zoster IgG antibodies were positive (>4000 mIU/ml), whereas IgM antibodies were negative (0.121).

Differential diagnosis of neonatal scars was considered such as congenital varicella syndrome, aplasia cutis congenita, and focal dermal hypoplasia syndrome. The diagnosis of congenital varicella syndrome was made based on the characteristic history, clinical features, and the presence of varicella-zoster IgG antibodies.

The parents of the baby were counseled about the condition and reassured. No active intervention was advised at this stage. Mother was taught physiotherapy exercises to be performed daily on the child's right foot. She was referred to a vitreoretinal surgeon, orthopedic surgeon, and plastic surgeon for further management.


  Discussion Top


FVS was first described in 1947 by La Foret and Lynch.[1] Srabstein et al. Rediscovered the syndrome in 1974 and named it “congenital varicella syndrome.”[1] Subsequently, the condition has been named as congenital varicella-zoster syndrome, fetal varicella-zoster syndrome, and varicella embryopathy.

Varicella may develop during pregnancy in 1–10 individuals per 10,000 pregnancies. Maternal varicella occurring between the 7th and 28th week of pregnancy may lead to either spontaneous abortions or “FVS.” Fetal consequences of varicella complicating pregnancy depend on the period of gestation at which the infection is contracted [Table 2].[5] The risk of transmission to the fetus is lower (0.55%) when the mother has infection between 0 and 12th week and is higher (1.4%) between the 13th and 20th weeks of gestation.[2]
Table 2: Gestational period-wise outcome of maternal varicella

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Pathogenesis of birth defects in FVS can be attributed to a disturbance of the developing nervous system, involving disseminated VZV infection and/or in utero herpes zoster. This supports the hypothesis that the lesions are due to herpes zoster in utero, based on the observation that skin lesions follow the dermatomal pattern distribution associated with herpes zoster, and that musculoskeletal and nervous system lesions are also segmental. VZV is neurotropic and can therefore arrest development of the central, peripheral, or autonomic nervous systems. Infection of the spinal cord and ganglia can lead to denervation of the limb bud, resulting in hypoplasia. Infection of the optic tract can result in optic atrophy and chorioretinitis, and infection of the fetal brain may lead to microcephaly. It is thought that brain lesions are due to intrauterine encephalitis during VZV reactivation through destructive and inflammatory lesions.[6]

The typical clinical features of FVS are described in [Table 3].[3],[7] This neurotrophic virus usually affects the rapid development phase of the tissues during the early stages of development, thus producing the characteristic manifestation of “the cicatrix” with multiple organ involvement, whereas less severe forms have only cutaneous and ocular involvement. Often, manifestations do not progress in the postnatal phase and are supposed to be noninfective, hence do not require antiviral or immunoglobulin treatment.[3]
Table 3: Principle clinical features of fetal varicella syndrome

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In anticipated cases of FVS, amniocentesis, fetal blood, and chorionic villi sampling may be performed to isolate the virus or to detect specific IgM.[6] Management of varicella complicating pregnancy depends on the period of gestation at which the infection is contracted[8] [Table 4]. Pregnant women who are suspected not to be immune and who experience exposure to varicella-zoster should be given varicella-zoster immunoglobulin (VZVIG) within 3 days of contact, although its efficacy is doubtful.[4] VZVIG reduces maternal disease and complications by 75%. There is also evidence that VZVIG also reduces the risk of fetal infection. For efficacy, it should be administered within 72–96 h after the exposure. The dose is 125 or 250 units/10 kg of body weight.[6] Termination of pregnancy is not indicated as the risk of fetal damage is less but attempts of early identification of FVS should be made and mothers can be offered termination once FVS is suspected. In our case, the FVS outcome could have been prevented if the mother had sought adequate medical checkups and advice regarding immune status against varicella during the antenatal period.
Table 4: Gestational period-wise management of maternal varicella

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The risk of mortality is 30% within the 1st year of life.[9] If the child is born with full-blown FVS, the mortality is around 25% in the first 3 months of life. A follow-up report in literature demonstrates that, in spite of initially poor prognosis, a good long-term outcome can occur in patients with CVS.[10] If patient of congenital varicella syndrome is provided with adequate counseling and treatment good long term outcome can be achieved.

The case described above had a history of maternal varicella during the first trimester of pregnancy and presented with low birth weight, cutaneous scars, limb hypoplasia, a malformed toe, and unilateral CTEV with few ocular complications which are typical for FVS. Our case is being reported to increase awareness about this extremely rare but fascinating condition and its associations, which requires a careful history and clinical examination for diagnosis.


  Conclusion Top


FVS is an embryopathy that requires a multidisciplinary and holistic approach for its diagnosis, possible management, and prevention of complications. In the absence of effective treatment, prevention remains the best means to avoid FVS.

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 initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Ramachandra S, Metta AK, Haneef NS, Kodali S. Fetal varicella syndrome. Indian J Dermatol Venereol Leprol 2010;76:724.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Auriti C, Piersigilli F, De Gasperis MR, Seganti G. Congenital Varicella syndrome: Still a problem? Fetal Diagn Ther 2009;25:224-9.  Back to cited text no. 2
    
3.
Gupta V, Pandita A, Panghal A, Hassan N. Unusual cause of brachial palsy with diaphragmatic palsy. BMJ Case Rep 2018;2018:bcr2017223624.  Back to cited text no. 3
    
4.
Chaudhary A, Padhiar B, Karia U, Parmar K, Shah B. Congenital Varicella syndrome. Indian J Paediatr Dermatol 2016;17:151-2.  Back to cited text no. 4
  [Full text]  
5.
Sasidharan CK, Anoop P. Congenital varicella syndrome. Indian J Pediatr 2003;70:101-3.  Back to cited text no. 5
    
6.
Mandelbrot L. Fetal varicella – Diagnosis, management, and outcome. Prenat Diagn 2012;32:511-8.  Back to cited text no. 6
    
7.
Paige DG. Dermatoses of the neonate. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's Textbook of Dermatology. 9th ed. Oxford: Blackwell Science Ltd; 2016. p. 116-22.  Back to cited text no. 7
    
8.
Blumental S, Lepage P. Management of varicella in neonates and infants. BMJ Paediatr Open 2019;3:e000433.  Back to cited text no. 8
    
9.
Sauerbrei A, Wutzler P. The congenital varicella syndrome. J Perinatol 2000;20:548-54.  Back to cited text no. 9
    
10.
Sauerbrei A, Wutzler P. Herpes simplex and varicella-zoster virus infections during pregnancy: Current concepts of prevention, diagnosis and therapy. Part 1: Herpes simplex virus infections. Med Microbiol Immunol 2007;196:89-94.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1], [Table 2], [Table 3], [Table 4]



 

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