Vallecular cyst

Suganya Periyanayagi1*, Dhariniya S2, Ruby Ravichandran3

1Nursing Supervisor, NICU, Maa Kauvery, Trichy, Tamil Nadu

2Nursing Educator, Maa Kauvery, Trichy, Tamil Nadu

3Senior Deputy Nursing Superintendent, Maa Kauvery, Trichy, Tamil Nadu

*Correspondence

Abstract

Vallecular cyst is a rare congenital laryngeal anomaly that may present in the neonatal period with respiratory distress, feeding difficulty, stridor, and airway obstruction. Early diagnosis is crucial because delayed recognition can result in life-threatening respiratory compromise. This case presentation describes a late preterm female neonate born at 35 weeks and 2 days gestation who developed severe respiratory distress soon after birth. During intubation, an upper airway cystic swelling was identified. Through further ENT evaluation, histopathological examination and imaging studies the neonate was diagnosed as having a well-defined vallecular cyst. Surgical excision of the cyst and supportive management like treatment for respiratory distress syndrome, neonatal hyperbilirubinemia, and hypocalcemia was provided. This case emphasizes the importance of considering congenital upper airway anomalies in neonates with persistent respiratory distress and highlights the role of multidisciplinary management in achieving favorable outcomes.

Key words: Vallecular cyst; Stridor; Hyperbilirubinemia

Introduction

Vallecular cysts are uncommon benign cystic lesions arising in the vallecular region between the base of the tongue and the epiglottis. These cysts are generally caused by obstruction of mucous gland ducts leading to mucus retention and cyst formation. Although rare, vallecular cysts are clinically significant in neonates and infants because even small lesions can compromise the narrow neonatal airway. Clinical manifestations range from mild feeding difficulties and stridor to severe respiratory distress and acute airway obstruction. Neonates often present with inspiratory stridor, cyanotic episodes, poor feeding, apnea, and failure to thrive.

Congenital vallecular cysts account for a small percentage of laryngeal cysts and are frequently associated with delayed diagnosis because symptoms may mimic more common neonatal respiratory disorders such as respiratory distress syndrome, laryngomalacia, sepsis, or transient tachypnea of the newborn. Flexible laryngoscopy and imaging studies such as computed tomography or magnetic resonance imaging are important diagnostic tools for identifying the lesion and determining its extent. Definitive treatment involves surgical excision or marsupialization of the cyst to relieve airway obstruction and prevent recurrence.

Premature neonates are particularly susceptible to respiratory compromise because of immature lungs and reduced respiratory reserve. The coexistence of respiratory distress syndrome and vallecular cyst can further complicate clinical presentation and management. This case presentation discusses the diagnosis and successful management of late preterm neonate with respiratory distress syndrome associated with a right vallecular cyst and reviews the relevant literature regarding presentation, diagnosis, and treatment of congenital vallecular cysts.

Case Presentation

A 13-day-old female late preterm neonate was admitted with respiratory distress since birth. The baby was born at 35 weeks and 2 days gestation by lower segment cesarean section on 03/01/2026 at 1:39 PM. Birth weight was 2.4 kg, and the admission weight was 2.35 kg. No immediate postnatal resuscitation was required. However, within a few hours of life, the baby developed respiratory distress characterized by tachypnea and increased work of breathing, prompting referral to a tertiary neonatal center at 9 hours of life for advanced management.

The mother was a 29-year-old Gravida 3 with a significant antenatal history of gestational diabetes mellitus, pregnancy-induced hypertension, and hypothyroidism. Antenatal scans were reportedly normal, and there was no history of consanguinity. Maternal blood group was B positive. The mother was managed with dietary modifications for gestational diabetes mellitus and oral labetalol for hypertension during pregnancy. There was no history of maternal infection or significant antenatal complications apart from the existing medical disorders.

The neonate was admitted to the neonatal intensive care unit because of persistent respiratory distress and suspected respiratory distress syndrome. During hospitalization, additional findings suggested the presence of an upper airway lesion, leading to further investigations and eventual diagnosis of a vallecular cyst.

Clinical Findings

On admission, the neonate appeared tachypneic with signs of respiratory distress. Respiratory rate was 80 breaths per minute, heart rate was 162 beats per minute, and oxygen saturation was 97% with 40% Fio2 support. The Downes score was 7/10, indicating significant respiratory compromise. Acrocyanosis was present, although peripheral perfusion remained normal. Activity was normal, and the baby had a good cry.

Systemic examination revealed normal first and second heart sounds without murmuring. Respiratory system examination showed bilateral equal air entry. Abdominal examination revealed a soft abdomen without organomegaly. Central nervous system examination showed an open anterior fontanelle with fair neonatal reflexes. Examination of bones, joints, and soft tissues was normal. While securing the airway through intubation, clinicians observed a cystic swelling in the upper airway suggesting a congenital laryngeal abnormality. This finding raised suspicion of a vallecular cyst and led to ENT consultation and further imaging studies. The persistence of respiratory distress despite standard management for respiratory distress syndrome also supported the need for evaluation of an anatomical airway obstruction.

Investigations and Results

Initial laboratory investigations demonstrated hemoglobin of 18.1 g/dL, total leukocyte count of 20,150/mm³, platelet count of 2.87 lakh/mm³, and elevated C-reactive protein levels suggestive of inflammatory response. Serial blood glucose monitoring remained within normal limits. Blood culture showed no bacterial growth. TSH level was 1.30 mIU/L.

Chest radiography demonstrated findings suggestive of respiratory distress syndrome, and the neonate received surfactant therapy accordingly. Screening echocardiogram revealed a Patent Ductus Arteriosus (PDA), which was managed conservatively. Screening cranial ultrasonography showed no evidence of intraventricular hemorrhage or hydrocephalus. ENT evaluation suggested a right vallecular cyst. Computed tomography of the neck demonstrated a well-defined rounded cystic lesion in the vallecula located near the midline. The lesion consisted of a vallecular cyst causing upper airway obstruction. Histopathological examination of the excised specimen later confirmed the diagnosis of vallecular cyst. The neonate also developed neonatal hyperbilirubinemia requiring phototherapy and hypocalcemia requiring calcium supplementation. These associated neonatal complications were managed successfully during hospitalization.

Fig (1): Pre-operative

Fig (2): Intra operative

Fig (3): Post-operative

Diagnosis

Based on clinical presentation, airway findings, imaging studies, and histopathological confirmation, the final diagnosis was established as a late preterm neonate with respiratory distress syndrome complicated by a right vallecular cyst causing upper airway obstruction. Associated diagnoses included low birth weight, neonatal hyperbilirubinemia, hypocalcemia, and patent ductus arteriosus.

Management

Definitive management of vallecular cysts is surgical. Needle aspiration alone is associated with recurrence and therefore is not considered adequate treatment. Endoscopic marsupialization or complete excision under general anesthesia are the preferred treatment methods and are associated with good outcomes and low recurrence rates.

The neonate was admitted to the neonatal intensive care unit and managed aggressively because of severe respiratory distress. Initial management included mechanical ventilation, CPAP support, blended oxygen therapy, intravenous fluids, and empirical intravenous antibiotics including Piperacillin-Tazobactam, Amikacin, and Meropenem. Surfactant therapy was administered for respiratory distress syndrome. Nebulization with budesonide, adrenaline, and hypertonic saline was also provided along with dexamethasone therapy. Minimal enteral feeding through an orogastric tube was initiated and gradually advanced as the neonate stabilized. During airway management, the identification of an upper airway cyst prompted ENT consultation and further evaluation. CT imaging confirmed the diagnosis of a vallecular cyst, and surgical management was planned. Under general anesthesia, the child was placed in the supine position with neck extension. A laryngoscope was introduced, and the vallecular cyst was visualized clearly. The cyst was aspirated, thick fluid was evacuated, and the cyst wall was completely excised using forceps. Adequate hemostasis was achieved during the procedure. Examination of the epiglottis, epiglottic folds, and vocal cords showed normal anatomy. The excised specimen was sent for histopathological examination.

Postoperatively, the neonate remained stable and was shifted with endotracheal tube support. Respiratory status gradually improved, and the neonate tolerated spontaneous breathing trials. The infant was successfully extubated and transitioned to oxygen support through nasal prongs before eventual weaning to room air by the ninth day of life. Phototherapy was administered for neonatal hyperbilirubinemia. Hypocalcemia was managed with oral calcium supplementation. Kangaroo mother care, breastfeeding support, lactation counseling, and early neurodevelopmental stimulation exercises were provided to the caregivers throughout hospitalization.

Outcome

The postoperative course was uneventful, and the neonate demonstrated gradual clinical improvement. Respiratory distress resolved progressively following excision of the vallecular cyst and supportive neonatal care. The baby tolerated oral feeding adequately, and direct breastfeeding was established successfully before discharge. Oxygen saturation remained stable in room air without episodes of respiratory distress or cyanosis. The neonate maintained stable hemodynamic parameters and showed satisfactory neurological activity. Weight gain and feeding tolerance improved gradually. Histopathological examination confirmed features consistent with vallecular cyst, validating the diagnosis and management approach. Overall, the infant responded well to multidisciplinary neonatal and surgical care.

Discharge

At discharge, the neonate was active and maintained stable vital signs. Heart rate was 142 beats per minute, respiratory rate was 44 breaths per minute, and oxygen saturation ranged between 97% and 99% in room air. The discharge weight was 2.1 kg. Physical examination showed good activity and normal movement of all four limbs. Red reflex was present bilaterally. Head circumference measured 32 cm, chest circumference 29 cm, abdominal girth 26 cm, and length 45 cm. The infant was discharged on oral multivitamin drops, Vitamin D3 supplementation, Calcium-Phosphorus-vitamin D syrup, and Domperidone drops. Parents were advised to continue exclusive breastfeeding, maintain warmth, and monitor for warning signs such as fever, seizures, excessive crying, poor feeding, jaundice, reduced activity, or respiratory difficulty. Follow-up with a pediatrician and neurodevelopmental surveillance were advised along with routine immunization.

Conclusion

This case illustrates the successful management of a rare congenital vallecular cyst in a late preterm neonate presenting with severe respiratory distress. The coexistence of respiratory distress syndrome and upper airway obstruction complicated the clinical picture and required a multidisciplinary approach involving neonatologists, ENT surgeons, anesthesiologists, and nursing staff. Early recognition of the airway lesion during intubation, prompt imaging, and definitive surgical excision were critical in preventing further respiratory compromise and ensuring favorable recovery. Comprehensive neonatal intensive care, including respiratory support, nutritional management, phototherapy, and developmental care, contributed to the positive outcome. This case underscores the importance of considering congenital upper airway anomalies such as vallecular cyst in neonates with persistent respiratory distress and emphasizes the role of timely surgical intervention in reducing morbidity and mortality.

Reference

  1. Lee, W. S., Tsai, C. S., Lin, C. H., & Hsu, H. T. (2012). Vallecular cyst in infants: Report of seven cases and literature review. European Archives of Oto-Rhino-Laryngology, 269(4), 1173–1177.

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