|Year : 2020 | Volume
| Issue : 5 | Page : 560-561
Unusual association of anorectal malformation and macrocystic lymphatic malformation
Dileep Garg, Aditya Pratap Singh
Department of Pediatric Surgery, SMS Medical College Jaipur, Rajasthan, India
|Date of Submission||22-Jun-2019|
|Date of Decision||03-Sep-2019|
|Date of Acceptance||15-Oct-2019|
|Date of Web Publication||7-Sep-2020|
Aditya Pratap Singh
Near the Mali Hostel, Main Bali Road, Falna, Pali - 306 116, Rajasthan
Source of Support: None, Conflict of Interest: None
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|| |
Anorectal malformations (ARMs) are among the more common congenital anomalies, with an incidence ranging from 1 in 2000 to 1 in 5000 live births. 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.
About 20%–80% of patients born with ARMs also have associated defects including including gastrointestinal, genitourinary, cardiovascular and of the central nervous system. 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|| |
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].
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|| |
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.
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.
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.
We would like to thank Dr. Neelam Dogra, MD in Anaesthesia, Senior Professor, SMS Medical College, Jaipur, Rajasthan, India.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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