Correlapaedia - a Correlative Encyclopedia of Pediatric Imaging, Surgery, and Pathology

Case 45

Clinical History:
This was a term female who had a prenatal diagnosis of a large right sided cystic neck and facial mass as seen on a prenatal ultrasound at 34 weeks gestation. The mother had an elective cesarean section in order to ensure proper management of the airway during delivery. During the cesarean section, the baby's head was delivered onto the mother's abdomen with the umbilical cord still attached. A laryngoscopy was performed. The airway was suctioned and an endotracheal tube was placed. The cord was then clamped. The baby was taken to an adjoining operating room and a direct laryngoscopy was performed which showed a sufficient airway. The baby was then extubated and did well on room air for 3 hours, but then required CPAP for adequate oxygenation.

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Clinical Physical Exam:
Right sided facial swelling, protuberant tongue, swelling of the right eyelid and right side of the neck.

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Clinical Labs:
Non-contributory

Clinical Differential Diagnosis:
Lymphatic Malformation (formerly known as Cystic Hygroma), hemangioma

Imaging Findings:
An ultrasound from the second day of life showed the neck mass to consist of cystic components with few blood vessels within it.

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Imaging Differential Diagnosis:
Lymphatic malformation (cystic hygroma).

Operative Findings:
Due to tenuous ventilation, the patient underwent tracheostomy. At the time of tracheostomy on the eleventh day of life, laryngoscopy revealed extensive infiltration of the lymphatic malformation into the glottis and subglottis.

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Pathological Findings:
A tissue biopsy was not performed on this patient.

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Final Diagnosis:
Lymphatic Malformation (formerly known as Cystic Hygroma)

Follow-up and Prognosis:
The patient developed a gradual requirement for oxygen, due to increasing upper airway obstruction. She had a tracheostomy on the eleventh day of life. The mass was slowly enlarging in size. A MRI of the neck performed at 3 months of age showed the mass to extensively involve the neck. A laryngoscopy performed at 3 and a half months of age showed the epiglottis and vallecula replaced by the lymphatic malformation with the hypopharynx on the left side effaced by the lymphatic malformation. The subglottic area also showed evidence of lymphangioma. The next day the patient underwent experimental sclerosis of six cystic lesions in the lymphatic malformation using direct injection of Ethanol and Sotradecol.

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Similar Cases:
Case 16

References:

• Paediapedia
• WebPath
• WebPath

URL: http://www.virtualpediatrichospital.org/