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Stridor in a neonate evokes a series of prompt evaluations to determine the etiology. Common etiologies include inherent structural defects (such as.
Perinatal/Neonatal Case . . . Presentation . . . . . . . .

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Laryngeal Lymphatic Malformation in a Newborn Alexander Kenton, MD Newton Duncan, MD Kushal Bhakta, MD Caraciolo J. Fernandes, MD

Stridor in a neonate evokes a series of prompt evaluations to determine the etiology. Common etiologies include inherent structural defects (such as laryngomalacia), vocal fold paralysis, acquired infectious etiologies, or extrinsic compressions. We report a neonate with a lymphatic malformation (lymphangioma) initially confined to the larynx that presented with stridor and progressive respiratory failure. Only seven other cases have been reported to present with lymphatic malformation confined solely to the larynx. There are several different modalities available to treat this condition, but recurrences are common. The presentation and management are discussed below. Physicians should be aware that stridor in a neonate can result from this unusual intrinsic obstruction of the larynx. Journal of Perinatology (2003) 23, 567–571. doi:10.1038/sj.jp.7210942

INTRODUCTION Stridor in a neonate is a presenting sign of upper airway obstruction.1 Usual evaluation seeks to delineate whether the cause involves an anatomic anomaly, vocal fold paralysis, laryngeal edema, or extrinsic compression. Rarely do benign growths, such as papilloma, hemangioma, or lymphatic malformations, present as upper airway obstruction in the neonate.2 Although lymphatic malformations are known to cause upper airway obstruction, only a few cases have documented a lymphatic malformation confined solely to the larynx and glottis. Those lymphatic malformations that cause laryngeal obstruction are known to be difficult to manage, with a high degree of recurrence or persistent disease.2 We report a neonate who presented with stridor in the first few hours of life. The evaluation and management are discussed below.

Department of Pediatrics (A.K., K.B., C.J.F.), Baylor College of Medicine, Houston, TX, USA; and Departments of Otolaryngology & Pediatrics (N.D.), Baylor College of Medicine, Houston, TX, USA. Address correspondence and reprint requests to Alexander B. Kenton, MD, Department of Pediatrics, Baylor College of Medicine, MC 1-3460, Section of Neonatology, One Baylor Plaza, Houston, TX 77030-3498, USA.

CASE REPORT A term baby boy presented to our institution with stridor and cyanosis. He was born at 40 weeks gestation at the referring hospital to a 23-year-old primigravida woman by vacuum-assisted vaginal delivery. Maternal laboratory details were as follows: blood type, B positive; RPR, nonreactive; hepatitis B surface antigen screen, negative; group B streptococcal screen, negative. Pregnancy and delivery were reported to be without complications. Rupture of membranes was 6 hours prior to delivery. Initial physical examination was normal. Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. At 2 hours of age, the infant was noted to be agitated, tachypneic, and cyanotic. He displayed inspiratory stridor and retractions. The infant improved following administration of supplemental oxygen. At 7 hours of life, a similar incident occurred, which resolved with supplemental oxygen and the placement of a ‘‘shoulder roll’’. He was subsequently transferred to our institution for further evaluation and management. Following transfer, initial physical examination revealed a sleeping full-term infant without signs of respiratory distress. Physical examination was normal, with the exception of mild supraclavicular retractions and stridor on auscultation of the neck. Complete blood count, serum electrolytes, whole-blood glucose, and serum lactate were normal. Initial arterial blood gas, without supplemental oxygen, revealed a pH of 7.34, and PaCO2 of 36 mmHg and a PaO2 of 82 mmHg with a HCO3 concentration of 19 mmol/l. Direct laryngoscopy showed no gross abnormalities, although radiographs of the neck suggested soft tissue swelling in the epiglottic area. Exam via flexible naso-pharyngoscopy revealed mild laryngomalacia, but no other abnormalities. Over the next 48 hours, the infant developed worsening stridor accompanied by tachypnea, supraclavicular and subcostal retractions, and cyanosis. Owing to progressive stridor, retractions and decrease of oxygen saturation on pulse oximetry, the patient underwent laryngoscopy and bronchoscopy followed by intubation on day-of-life (DOL) 3. Exam at that time revealed erythema and edema of the epiglottis and surrounding laryngeal tissues (Figure 1). A minor Type I posterior laryngeal cleft was noted, and mild laryngomalacia persisted. In view of the clinical deterioration, blood cultures were drawn, and antibiotics initiated empirically. Ranitidine and metoclopramide were initiated for possible gastro-esophageal reflux disease. Dexamethasone was initiated to decrease the laryngeal edema.

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Figure 1. Erythema and edema of larynx with laryngomalacia is demonstrated.

Lymphatic Malformation in a Newborn Larynx

Figure 3. Biopsy of the larynx revealed numerous submucosal vascular channels lined by a single layer of flat endothelium and separated by bundles of collagen. The arrows show two examples, with the lower one having a more complex irregular shape (hematoxylin– eosin stain, original magnification  100).

continued airway compromise, on DOL 15, a tracheostomy was performed for airway maintenance and safety. The patient was subsequently weaned from the ventilator to a tracheostomy collar without difficulty, and was discharged home. He has had no further airway difficulties. At 1 month of age, he developed a left submandibular soft fullness compatible with lymphatic malformations in this region. Recent physical exam revealed soft, cystic submandibular masses, and magnetic resonance imaging (MRI) confirmed lymphatic malformation not only of the larynx, but now also involving the surrounding areas as well (Figure 4). After 6 months of age, he will be a candidate for OK-432 sclerosing therapy, laser therapy in the laryngeal area, or possible further surgical therapy in efforts for early tracheostomy tube decannulation. DISCUSSION Figure 2. Progression of erythema and edema of larynx is demonstrated 5 days later with cystic structures now seen (most notably on the left side).

Tracheal secretions were sampled, and found to be negative for viruses and lipid-laden macrophages. The blood was sterile. On DOL 8, a repeat laryngoscopy and bronchoscopy revealed progression of the edema in the epiglottic and glottic tissues accompanied by developing cystic areas (Figure 2). Histopathological examination of biopsy specimens of laryngeal and glottic tissues revealed few tortuous irregular thin-walled vessels in a fibrous connective tissue mass consistent with lymphangioma or lymphatic malformation (Figure 3). Owing to 568

Stridor is a respiratory sound because of turbulent airflow in the upper airways, and may be caused by extrinsic or intrinsic airway obstruction. Its presence in the neonate should trigger a prompt and thorough history and physical exam to determine the seriousness and cause of the obstruction. Infants with respiratory distress are usually referred to tertiary care centers where anesthesia and otolaryngology services are available. Indirect visualization through naso-pharyngo-laryngoscopy combined with direct laryngoscopy or bronchoscopy along with supportive imaging modalities complement the evaluation.1 The possible causes of stridor in a neonate are many (Figure 5). The differential diagnosis can be classified by whether the obstruction is congenital or acquired, and whether it is intrinsic or extrinsic to the tissues of the airway. In a clinical setting, the Journal of Perinatology 2003; 23:567–571

Lymphatic Malformation in a Newborn Larynx

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diagnosis is often reached by a careful elimination of possible causes. Lymphangiomas are thought to arise from lymphatic vessel malformations. There is still considerable controversy surrounding the origin of these lesions. It is known that the lymphatic system originates around the 6th week of embryogenesis with the formation of paired jugular sacs arising as clefts in the cervical

Figure 4. MRI several weeks later, after the appearance of cystic neck masses, showing lymphatic malformation on T2 weighted images.

mesenchyme.3 One theory, presented by McClure and Silvester,4 proposes that the primordial lymphatic sacs fail to connect to the venous system with which they normally communicate, thus leading to the development of a lymphangioma. Another theory proposes that lymphangiomas are the result of lymph tissues that are deposited in an incorrect area during embryogenesis, thus failing to connect with the normal lymph system.5 A third theory proposes that lymphangiomas are derived from a process similar to hemangiomas; isolated lymphatic vessels take on a neoplastic-like behavior and proliferate, thus forming a new network of lymph channels which never communicate with a major lymph channel.6 None of these theories have been proven absolutely to be the correct one, thus even the classification of these benign growths is in doubt. In 1947, Eggston and Wolfe7 proposed that lymphangiomas be classified as telangiectatic or simple capillary, cavernous, or cystic lymphangioma (also known as cystic hygroma). Many authors feel that there is a continuum of the disease.8 At times, all histological types can be found in a single lesion, even if one type dominates the clinical and pathological picture. Currently, the best term describing this spectrum is lymphatic malformation.9 Lymphangiomas or lymphatic malformations make up between 5 and 6% of all benign soft tissue tumors in the pediatric population. Most (80 to 90%) are detected by 2 years of age.9,10 Confirming previous studies, a recent retrospective review indicated that the majority of these lesions occur in the head and neck region.11 Less often, they can involve the trunk and extremities. In 10% of the patients, the visceral organs are involved. These lesions appear to affect males and females equally. Although lymphatic malformations present equally in all races, lymphatic malformations involving or confined to the larynx seem to occur more often in Caucasians than African Americans.2,12,13 While a

Stridor in the Newborn Acquired

Congenital

Infectious Tracheitis Epiglottitis Laryngeal papilloma

Structural Laryngeal or subglottic hemagioma Laryngeal or subglottic lymphangioma

Intrinsic

Extrinsic

Laryngomalacia Tracheomalacia Bronchomalacia Laryngeal cysts Laryngotracheoesophageal clefts Laryngotracheal stenosis Subglottic stenosis Tracheal rings Neuromuscular disorders

Vascular rings Anomalous innominate arteries Pulmonary artery slings Cystic hygroma

Trauma/ Inflammatory Vocal cord paralysis Subglottic stenosis following intubation Post-extubation glottic inflammation Laryngo-tracheal inflammation secondary to gastroesophageal reflux disease

Figure 5. Differential diagnosis of a newborn presenting with stridor. Journal of Perinatology 2003; 23:567–571

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Table 1 Patients Presenting with Laryngeal Lymphangioma Year reported

Sex

Age at presentation

Presenting symptom(s)

Maximal extent of disease

Therapy instituted

Outcome

195016 197418

F M

3 months 20 months

Left subglottis Epiglottis/valleculae

Tracheostomy Resection

198519

F

16 years

Stridor Mass from base of tongue Dyspnea

Epiglottis

199320

F

9 days

Stridor

199614

M

6 months

Stridor

199817

M

1 day

200015

F

13 years

Cyanosis/Difficult intubation Airway obstruction & laryngeal mass

Valleculae/epiglottis/left cervical and submandibular area Glottis/epiglottis/aryepiglottic folds Epiglottis and right-sided glottic structures Supraglottis/epiglottis/ aryepiglottic folds

Tracheostomy/supraglottic partial laryngectomy Tracheostomy/resection/ modified neck dissection/laser ablation Tracheostomy/laser ablation

Not described Recurrence at one year. Disease free since second resection Decannulated/disease free at 6 month follow-up As of report, continued to have recurrence and need repeat laser ablation therapies Disease free at 14 months of age. Decannulated. Partial mobility of right true vocal cord; no recurrence of lesion Decannulated at day 20; Disease free at 2 year follow-up

lymphatic malformation may appear to be confined to the larynx initially, in reality most are usually the result of a continuity of other foci of the disease. These lesions are rarely found confined solely to the larynx, seven cases having been reported in the literature (Table 1).14–20 As occurred in our patient, these lesions often progress to involve other structures of the neck, chest, and mediastinum. They are usually diagnosed in the neonatal period, and are rarely diagnosed after 2 years of age. Surgical excision or vaporization had been the mainstay of treatment of head and neck lymphatic malformations through the 1990s. Owing to the possible functional sequelae due to excision, and the frequency of recurrence, alternatives such as sclerosing therapy and laser surgery have been tried with varying degrees of success. In the series reviewed by Alqahtani et al.,11 recurrence occurred 100% of the time in those who underwent aspiration as the sole method of treatment. There was a 40% recurrence rate in the group undergoing partial resection with laser surgery; recurrence occurred 17% of the time in those who underwent complete excision. Unfortunately, among those who underwent complete excision, the recurrence rate in those with head and neck lesions was 33%, as compared to 0% in those with truncal or visceral lesions.11 According to Hartl et al.,21 extensive infiltration of the upper airway and esophageal structures could be an important predictive factor in poor prognosis. In this series of patients treated for lymphatic malformation as a cause of upper airway obstruction, the postoperative mortality was 6%, similar to other reports.22 Early surgery did not seem to eliminate the rate of recurrence or the need for additional surgeries. Laser surgery for lymphatic malformations is reported sporadically. In a paper by White and Adkins,23 three cases are described, with a range of success from initial complete vaporization to the need for repeat procedures. Laser surgery would theoretically offer several advantages to conventional surgery by minimizing trauma to adjacent structures, decreasing the incidence of bleeding, and 570

Tracheostomy/resection and laser ablation Tracheostomy/laser ablation/resection

possibly decreasing the incidence of edema, all important for critical structures like the larynx.23 To date, no randomized controlled trials have demonstrated the superiority of one modality of treatment over another. Individualization of treatment is needed in most cases depending on cyst type, extent, and location. Treatment modality also depends on age of presentation and concomitant medical problems. Currently, sclerosing injection therapy, laser vaporization, and surgery may be used alone or in combination for the best long-term result. In summary, we report a rare cause of upper airway obstruction in the newborn infant. Management of lymphatic malformation involving the larynx is controversial, and fraught with the difficulties of progression and recurrence. Neonatologists should be aware that lymphatic malformations of the upper airway can present as life-threatening emergencies in the early neonatal period, and may require tracheostomy for airway maintenance. References 1. Mancuso RF. Stridor in neonates. Pediatric Clin N Am 1996;43:1339–56. 2. Schloss MD, Sweet RC, Blais C, et al. Lymphangioma in children. J Otolaryngol 1984;13:95–8. 3. Sabin FR. On the origin of the lymphatic system from the veins and the development of lymph, heart and thoracic duct in the pig. Am J Anat 1901;1:367–89. 4. McClure CFW, Silvester CF. A comparative study of the lymphatic-venous communications in adult mammals. Anat Rec 1909;3:534–53. 5. Phillips HE, McGahan JP. Intrauterine fetal cystic hygroma: sonographic detection. Am J Roentgenol 1981;136:799–802. 6. Lee K-J, Klein TR. Surgery of cysts and tumors of the neck. In: Paparella MM, Shumrick DA, editors. Otolaryngology. 2nd ed. Philadelphia, PA: WB Saunders; 1980. pp. 2987–9. 7. Eggston AA, Wolf D. Histopathology of the Ear, Nose and Throat. Baltimore, MD: Williams and Wilkins Co; 1947. pp. 770–4. 8. Handler SD, Raney Jr. BB. Management of neoplasms of the head and neck in children. Head Neck Surg 1981;3:395–405. Journal of Perinatology 2003; 23:567–571

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9. Zadvinskis DP, Benson MT, Kerr HH, et al. Congenital malformations of thecervicothoracic lymphatic system: embryology and pathogenesis. Radiographics 1992;12:1175–89. 10. Coffin CM, Dehener LP. Vascular Tumors in Children and Adolescents: aclinicopathologic study of 228 tumors in 222 patients. Pathology Annual 1993;28:97–120. 11. Alqahtani A, Nguyen LT, Flageole H, Shaw K, Laberge JM. 25 years experience with lymphangiomas in children. J Pediatr Surg 1999;34:1164–8. 12. Orvidas L, Kasperbauer J. Pediatric lymphangiomas of the head and neck. Ann Otol, Rhinol Laryngol 2000;109:411–21. 13. Cohen SR, Thompson JW. Lymphangiomas of the larynx in infants and children. A survey of pediatric lymphangioma. Ann Otol, Rhinol, and Laryngol F Supplement 1986;127:1–20. 14. Papsin BC, Evans G. Isolated laryngeal lymphangioma: a rare cause of airway obstruction in infants. J Laryngol Otol 1996;110:969–72. 15. Sobol SE, Manoukian JJ. Acute airway obstruction from a laryngeal lymphangioma in a child. Int J Otolaryngol 2001;58:255–7.

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16. Holinger PH, Johnston KC. Benign tumors of the larynx. Ann Otol, Rhinol, and Laryngol 1951;60:496–509. 17. Selz PA, Arjmand EM. Laryngeal lymphangioma: a case report of an uncommon entity. Otolaryngol F Head Neck Surg 1998;118:382. 18. Ruben RJ, Kucinski SA, Greenstein N. Cystic lymphangioma of the vallecula. Can J Otolaryngol 1974;4:180–4. 19. Carlos P, Viscasillas S, Carlos A, Carlos Jr. A. Lymphangioma of the larynx as a cause of progressive dyspnea. Int J Pediatr Otolaryngol 1985;9:263–8. 20. Williams WT, Cole RR. Lymphangioma presenting as congenital stridor. Int J Pediatr Otolaryngol 1993;26:185–91. 21. Hartl D, Roger G, Denoyelle F, et al. Extensive lymphangioma presenting with upper airway obstruction. Arch Otolaryngol Head Neck Surg 2000;126: 1378–82. 22. Raveh E, De Jong AL, Taylor G, et al. Prognostic factors in the treatment of lymphatic malformations. Arch Otolaryngol, Head Neck Surg 1997;123:1061–5. 23. White B, Adkins WY. Use of the carbon dioxide laser in head and neck lymphangioma. Lasers Surg Med 1986;6:293–5.

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