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CASE REPORT
Year : 2020  |  Volume : 13  |  Issue : 5  |  Page : 560-561  

Unusual association of anorectal malformation and macrocystic lymphatic malformation


Department of Pediatric Surgery, SMS Medical College Jaipur, Rajasthan, India

Date of Submission22-Jun-2019
Date of Decision03-Sep-2019
Date of Acceptance15-Oct-2019
Date of Web Publication7-Sep-2020

Correspondence Address:
Aditya Pratap Singh
Near the Mali Hostel, Main Bali Road, Falna, Pali - 306 116, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mjdrdypu.mjdrdypu_178_19

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  Abstract 


Anorectal malformations (ARMs) are common congenital anomalies encountered in pediatric surgery. ARMs are often associated with other anomalies. The estimated incidences of associated anomalies are 40%–70%. We report a case of low ARM with lymphangioma neck.

Keywords: Anorectal malformation, lymphangioma, neonate


How to cite this article:
Garg D, Singh AP. Unusual association of anorectal malformation and macrocystic lymphatic malformation. Med J DY Patil Vidyapeeth 2020;13:560-1

How to cite this URL:
Garg D, Singh AP. Unusual association of anorectal malformation and macrocystic lymphatic malformation. Med J DY Patil Vidyapeeth [serial online] 2020 [cited 2020 Sep 20];13:560-1. Available from: http://www.mjdrdypv.org/text.asp?2020/13/5/560/294343




  Introduction Top


Anorectal malformations (ARMs) are among the more common congenital anomalies, with an incidence ranging from 1 in 2000 to 1 in 5000 live births.[1] They may present as isolated anomaly or occur as a part of combined anomaly. Surgeons must be aware of the high incidence of associated abnormalities in cases of ARMs.[2]

About 20%–80% of patients born with ARMs also have associated defects including including gastrointestinal, genitourinary, cardiovascular and of the central nervous system.[3] These anomalies have an important impact on the survival of such patients. Here, we present a case of low ARM associated with lymphangioma neck. In literature, we did not find any case of ARM associated with lymphangioma.


  Case Report Top


A 2-day-old male newborn brought to pediatric surgery outdoor by his father with a complaint of swelling neck. On examination, the swelling was cystic and irregular in shape [Figure 1]. His father was also gave a history of abnormal anal opening. On perineal examination, we find low ARM with bucket-handle deformity [Figure 1].
Figure 1: Lymphangioma neck with bucket-handle deformity

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The patient was admitted in neonatal intensive care unit. All routine investigations were done. Ultrasound was done for neck swelling. Ultrasound neck revealed unilocular swelling as lymphangioma. Ultrasound abdomen was normal. All other investigations were in normal limit. The operation was planned for ARM first. Cutback anoplasty was done for ARM, and bleomycin sclerotherapy was given for lymphangioma. Bleomycin was given in dose of 0.5 mg/kg. The fluid of the lymphangioma was aspirated as much as possible with a 10-ml disposable syringe. After that, bleomycin was injected intralesionally in a ratio of 5:1 (aspirated volume: bleomycin volume). A total of three doses were given with a 3-week interval. The patient was discharged on the third postoperative day. On follow-up, lymphangioma was disappeared after the third dose of bleomycin.


  Discussion Top


The associated anomalies in ARMs are common. The genitourinary anomalies are the most common. The most common associated anomalies reported across the world were related to the urogenital system, approximately 50% of patients with ARM being also found to have urinary defects.[4]

The genitourinary anomalies (40%–50%) are the most common, followed by cardiovascular (30%–35%), spinal cord tethering (25%–30%), gastrointestinal (5%–10%), and Vertebral anorectal Cardiac tracheo-oesophageal fistula Renal Limb defect (VACTERL) (4%–9%) anomalies.[5]

ARMs have more chance of associated anomalies if the rectal pouch ends high. In literature, we did not find any case of ARM associated with macrocystic lymphatic malformation.

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. Neelam Dogra, MD in Anaesthesia, Senior Professor, SMS Medical College, Jaipur, Rajasthan, India.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Levitt MA, Pena A. Imperforate anus and cloacal malformations. In: Holcomb GW 3rd, Murphy JP, editors. Ashcraft's Pediatric Surgery. 5th ed. Philadelphia, PA: Saunders Elsevier; 2010. p. 468-90.  Back to cited text no. 1
    
2.
Levitt MA, Peña A. Anorectal malformations. In: Mattei P, editor. Fundamentals of Pediatric Surgery. New York: Springer Science; 2011. p. 499-511.  Back to cited text no. 2
    
3.
Mirza B, Ijaz L, Saleem M, Sharif M, Sheikh A. Anorectal malformations in neonates. Afr J Paediatr Surg 2011;8:151-4.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Goossens WJ, de Blaauw I, Wijnen MH, de Gier RP, Kortmann B, Feitz WF, et al. Urological anomalies in anorectal malformations in the Netherlands: Effects of screening all patients on long-term outcome. Pediatr Surg Int 2011;27:1091-7.  Back to cited text no. 4
    
5.
Watanabe Y, Ando H, Seo T, Kaneko K, Katsuno S, Shinohara T, et al. Three-dimensional image reconstruction of an anorectal malformation with multidetector-row helical computed tomography technology. Pediatr Surg Int 2003;19:167-71.  Back to cited text no. 5
    


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