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Effective Role of Oral Sildenafil in the Treatment of Cystic Hygroma in an Infant: A Case Report |
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Sumit Jeena, Atul Londhe, Amol Joshi, LS Deshmukh 1. DM Neonatology 3rd Year Senior Resident, Department of Neonatology, GMC, Aurangabad, Maharashtra, India. 2. Associate Professor, Department of Neonatology, GMC, Aurangabad, Maharashtra, India. 3. Associate Professor, Department of Neonatology, GMC, Aurangabad, Maharashtra, India. 4. Professor, Department of Neonatology, GMC, Aurangabad, Maharashtra, India. |
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Correspondence Address : Sumit Jeena, Room No. 2, A-Block, Mard Hostel, Ghati Hospital, Aurangabad-431001, Maharashtra, India. E-mail: sumit.jeena@gmail.com |
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| ABSTRACT |  | ||||||||||||||||||||||||||||||||
: Cystic hygroma is a benign congenital malformation of the lymphatic system. Most cystic hygromas are found in the neck; rarer locations include the axilla, mediastinum, and limbs. Symptoms range from incidental findings to significant morbidity due to compression of adjacent organs, infection, haemorrhage, etc. Treatment is primarily aimed at complete surgical resection. Other treatment modalities include sclerotherapy, radiotherapy, laser ablation, and medical therapy with sirolimus, but recurrence rates are high. Surgery is not always possible since Lymphatic Malformations (LMs) can be intertwined within muscles or organs, and incomplete resection of LMs can result in recurrence; hence, alternative therapies have been explored. Recently, authors reported a case of a full-term female infant with a left-sided cystic hygroma. There was marked regression of the cystic hygroma with oral sildenafil, a selective inhibitor of phosphodiesterase-5. | |||||||||||||||||||||||||||||||||
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| Keywords : Lymphangiomas, Lymphatic malformations, Sclerotherapy | |||||||||||||||||||||||||||||||||
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DOI and Others :
DOI: 10.7860/IJNMR/2025/79602.2451
Date of Submission: Mar 27, 2025 Date of Peer Review: Apr 27, 2025 Date of Acceptance: Aug 13, 2025 Date of Publishing: Sep 30, 2025 AUTHOR DECLARATION: • Financial or Other Competing Interests: None • Was informed consent obtained from the subjects involved in the study? Yes • For any images presented appropriate consent has been obtained from the subjects. Yes PLAGIARISM CHECKING METHODS: • Plagiarism X-checker: Mar 27, 2025 • Manual Googling: Jun 04, 2025 • iThenticate Software: Aug 11, 2025 (12%) ETYMOLOGY: Author Origin EMENDATIONS: 8 |
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| Case Report |
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A full-term female infant weighing 2500 g was delivered vaginally to a 28-year-old mother at Department of Neonatology, Government Medical College (GMC), Aurangabad, Maharashtra, India. Delayed cord clamping after one minute was performed. Appearance, Pulse, Grimace, Activity, Respiration (APGAR) scores were 8 and 9 at one and five minutes, respectively. A swelling (approximately 10×4 cm) was noted on the left-side of the neck and back. The mass was antenatally detected on prenatal ultrasound in the third trimester at 33 weeks, which showed cervical meningocoele (52×39 mm cystic lesion with septations). At birth, the baby weighed 2500 g and had a head circumference of 33 cm. The vitals are shown in (Table/Fig 1). The baby was initially fed Expressed Breast Milk (EBM) via a spoon every three hours, followed by breastfeeding. No signs of respiratory distress were noted on admission. Local examination of the neck swelling: On inspection, a swelling was present on the left-side of the posterior triangle of the neck, with a horizontal dimension of 10 cm and a vertical dimension of 4 cm, having a rounded shape, sharp margins, and a smooth surface. The overlying skin showed slight bluish discoloration with normal surrounding tissue. On palpation, there were no signs of inflammation or tenderness. The mass was soft, cystic, and compressible with fluctuation. Translucency was absent; it was not reducible and non-pulsatile. Mobility was not restricted (Table/Fig 2). Diagnosis: Ultrasound of the lesion showed a multicystic lesion with internal septations and no blood flow detected on colour Doppler ultrasound, suggestive of cystic hygroma. Paediatric surgeon consultation was obtained for further evaluation and treatment. Magnetic Resonance Imaging (MRI) of the neck could not be performed due to the patient’s family’s financial constraints. Treatment: Since the patient’s parents did not consent to surgical management, the alternative option of medical management with sildenafil was started after informed consent was obtained. At baseline (day 6), treatment was initiated with sildenafil (Vigorex 25 mg) at 1 mg/kg per dose, administered three times daily orally mixed with milk (1). Baseline physical examinations were performed, and vital signs, including blood pressure, heart rate, respiratory rate, oxygen saturation, and basal body temperature, were obtained before initiating sildenafil. After dispensing the initial dose, vital signs were monitored every 30 minutes during a 2-hour observation period. The neonate remained well and on breastfeeding during her stay in the neonatal unit. The neonate was given sildenafil thrice daily for 20 weeks. The swelling did not show any regression one month after treatment (Table/Fig 3). The infant was evaluated in the clinic at weeks 4, 8, 12, and 20 and was contacted by phone in between. At week 20 (end of treatment), sildenafil was discontinued. The infant was observed for 12 weeks after the last dose and followed-up till one year of age. The swelling began regressing at eight weeks (2 months) of treatment (Table/Fig 4), (Table/Fig 5), (Table/Fig 6), (Table/Fig 7). Outcomes included both the physician’s and parents’ assessments of swelling improvement compared with baseline. At each visit, the size of the swelling, changes in texture, and overall change compared with baseline were recorded (Table/Fig 8). No changes in texture were noted. Photographs were taken for comparison. Parents were asked to review and report any adverse events. No specific side effects were observed during the treatment course. Parents remain in regular outpatient follow-up; no untoward effects have been noted so far. | |||||||||||||||||||||||||||||||||
| Discussion |
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The patient in the present report was treated with sildenafil (Vigorex 25 mg) orally as 1 mg/kg per dose thrice daily for 20 weeks, in the same dosages as those in the study by Ullah MS et al., (1). Cystic LMs are localised areas of abnormal development of the lymphatic system (2). Several mechanisms have been proposed to explain the pathophysiology of cystic hygroma. They can be categorised based on cyst size as microcystic, macrocystic, and mixed lymphangiomas (3). Cystic hygroma, a macrocystic lymphangioma, occurs more frequently than other types of lymphangiomas and may be composed of single or multiple macrocystic lesions with limited communication with normal lymphatic channels (4). The term is usually used for congenital LMs detected in utero or observed at birth. Diagnosis is commonly made clinically based on large size, location, and translucence. Although it tends to enlarge progressively over months, a relatively rapid increase in size has also been described (5). Patients can present with visible deformity, pain related to compression of adjacent structures, or sudden enlargement of LM due to haemorrhage or infection (5),(6). The management of cystic hygroma is preferably surgical. Indications for surgery in paediatric cases include significant cosmetic deformity, obstructive symptoms, bleeding, and recurrent infections (4),(7). Other treatment modalities include aspiration, radiation, and injection of sclerosants such as bleomycin and OK-432, derived from a strain of Streptococcus pyogenes (8). Lymphangiomas have also been successfully treated with rapamycin (sirolimus) (9),(10). Sclerosants such as ethanol, doxycycline, bleomycin, and OK-432 are less effective for treating microcystic and mixed lesions. Although macrocystic lesions may respond well initially, patients often require repeated sclerotherapy treatments throughout their lifetime (5),(11). The efficacy of oral medications for the treatment of LMs, including sirolimus and propranolol, requires further investigation (10),(12). Sildenafil selectively inhibits phosphodiesterase-5, preventing the breakdown of cyclic Guanosine Monophosphate (cGMP). Inhibition of phosphodiesterase-5 decreases the contractility of vascular smooth muscle, producing vasodilation (13). A potential explanation for the therapeutic effect seen in these cases is that relaxation of the lymphatic vasculature may allow lymphatic spaces to open, thereby decreasing LM volume. Similar cases and studies reported in the literature has been depicted in (Table/Fig 9) (1),(14),(15). | |||||||||||||||||||||||||||||||||
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Case report
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