The Laryngoscope C 2014 The American Laryngological, V
Rhinological and Otological Society, Inc.
Case Report
Adenomatous Multinodular Goiter Causing Airway Obstruction in an Infant Auddie M. Sweis, BA; Dharani Guttikonda, BA; David L. Walner, MD; Katherine K. Hamming, MD; Nicholas A. Lygizos, MD; Grant Geissler, MD Nontoxic adenomatous multinodular goiter (AMNG) in infants is a rare condition. We discuss an extremely rare case of a nontoxic AMNG in a 3-month-old female presenting with airway obstruction. Surgical resection of the mass was performed to reduce the burden on the airway and for diagnosis. The literature is reviewed, and the clinical characteristics, pathology, and surgical treatment are discussed. Key Words: Adenomatous multinodular goiter; pediatric; mediastinum; neck mass; airway obstruction. Laryngoscope, 124:2636–2639, 2014
INTRODUCTION Adenomatous multinodular goiter (AMNG) refers to an abnormal growth of the thyroid gland plus at least one thyroid nodule. We report a case of a nontoxic AMNG in a 3-month-old patient. To the best of our knowledge, this is the only reported case of a nontoxic AMNG in an otherwise healthy infant born to a euthyroid mother with prenatal treatment. In addition to discussing the rare diagnosis of a nontoxic AMNG in an infant, this case report also reviews the presentation and etiology of airway obstruction in infants.
CASE REPORT A 3-month-old female presented to the otolaryngology clinic at Advocate Children’s Hospital, Park Ridge, Illinois, because of loud, raspy breathing and suprasternal retractions, both present since birth. The patient was otherwise healthy with a normal birth history and no congenital anomalies, inborn errors of metabolism, or pertinent family history. The infant’s mother has no personal or family history of thyroid disease; and during the pregnancy, she did not consume large amounts of goitrogens, undergo a computed tomography (CT) scan with contrast, have any excess exposure to iodine, or undergo any thyroid function testing. The patient’s
From the Rush Medical College, Rush University (A.M.S., D.G.); the Division of Pediatric Otolaryngology, Rush University Medical Center (D.L.W., K.K.H.), Chicago; the Division of Otolaryngology (N.A.L.), Advocate Lutheran General Hospital; and the Division of Pediatric Surgery (G.G.), Advocate Children’s Hospital (D.L.W., K.K.H.), Park Ridge, Illinois, U.S.A. Editor’s Note: This Manuscript was accepted for publication March 11, 2014. The authors have no funding, financial relationships, or conflicts of interest to disclose. Send correspondence to David L. Walner, MD, 1653 W. Congress Parkway, Chicago, IL, 60612. E-mail: [email protected] DOI: 10.1002/lary.24677
Laryngoscope 124: November 2014
2636
exam was significant for biphasic stridor. No obvious neck mass was detected on inspection or palpation, and the remainder of the exam was unremarkable. In office, fiberoptic laryngoscopy was performed and revealed increased pharyngeal secretions but no appreciable asymmetries or masses. The patient then underwent a more comprehensive evaluation under general anesthesia. Microlaryngoscopy revealed a submucosal bulge along the right posterior pharyngeal wall (Fig. 1). Additionally, compression of the right side of the trachea with flattening and blunting was noted just inferior to the subglottic region, with an estimated narrowing of the airway by 70%. The epiglottis, arytenoid complex, true vocal cords, and subglottic region appeared normal. Normal vocal cord movement was noted at this time. A CT soft tissue neck with contrast was performed and demonstrated a 5.4 cm 3 3.5 cm 3 2.4 cm mass originating in the right lobe of the thyroid gland and extending craniocaudally from the level of C2 to the mediastinum just superior to the aortic arch. The mass induced tracheal deviation to the left and posteriorly and also compressed the trachea at the level of the thoracic inlet (Fig. 2a–Fig. 2b). Postinfusion chest CT showed thoracic inlet compression, resulting in compression of the right innominate vein adjacent to the first rib. The patient’s preoperative thyroid function was assessed. Thyroid stimulating hormone (TSH) was 2.313 microunits/mL and free T4 was 1.5 ng/dL, both of which are within the normal limits of 0.25 to 4.0 microunits/mL for TSH and 0.7 to 1.9 ng/dL for free T4. The patient underwent excision of the right neck mass by the pediatric otolaryngology service, in conjunction with the pediatric surgery service. An incision was made on the right side of the neck. Upon retraction of the sternocleidomastoid muscle (SCM), the mass was Sweis et al.: Adenomatous Multinodular Goiter in an Infant
Fig. 1. Endoscopic photograph of airway compression at the level of the pharynx (before surgery). *The right pharyngeal mass.
discovered just deep to the SCM. The mass was elevated into the neck from the superior mediastinum by blunt dissection. It was then separated from the trachea and the floor of the neck. Finally, a right thyroid lobectomy was performed. The recurrent laryngeal nerve and parathyroid glands were identified and unharmed by the dissection. The pathologist reported the resected specimen to be 5.6 cm 3 3.1 cm 3 2.2 cm. Gross pathological inspection revealed a mass that was described as smooth, tan, and lobulated. Histopathological findings revealed multiple nodules of thyroid follicles separated by thick bands of collagen and appeared to be entirely surrounded by an intact fibrous capsule. The thyroid follicles were of variable size and lined by benign, cuboidal follicular cells filled with abundant col-
loid. Hyperplasia was present, evidenced by papillary infoldings and columnar epithelia. There was no evidence of malignant tissue. The diagnosis was most consistent with a right thyroid lobule with AMNG (Fig. 3). The child was extubated on postoperative day 1 without any complications. The stridor had resolved. On postoperative day 5, microlaryngoscopy revealed a patent airway with minimal residual blunting of the right upper tracheal wall—and normal pharynx, epiglottis, arytenoid structures, true vocal cords, subglottis, and tracheal air columns (Fig. 4). Additionally, a technetium thyroid imaging study showed a normal left thyroid lobe and no visualization of the right thyroid lobe, consistent with a hemithyroidectomy. Three weeks postoperatively, the patient had a normal free T4 of 1.0 ng/ dL with an elevated TSH of 7.648 microunits/mL. The patient was started on 25 mcg levothyroxine (Levothroid) daily. With close follow-up and thyroid hormone replacement, the patient’s TSH levels normalized. These endocrine findings are consistent with primary hypothyroidism secondary to the resection of a goiter with a right thyroid lobectomy. Six months after the surgical intervention and start of the daily levothyroxine, a follow-up visit showed a thriving child with no airway issues and normal TSH and free T4 levels. The child will be maintained on levothyroxine indefinitely, with dosing adjustments made when appropriate.
DISCUSSION The majority of pediatric neck masses are either inflammatory or congenital. Torsiglieri et al. examined 446 neck masses in pediatric patients.1 Congenital masses accounted for 54% of all neck masses and included branchial cleft cysts (18%), thyroglossal duct cysts (16%), dermoid cysts (10%), lymphangiomas (8%), and hemangiomas (2%). Inflammatory lesions accounted for 27% of all neck masses and included reactive lymphadenopathy (16%),
Fig. 2. a. Computed tomography scan of the neck demonstrating the mass and airway compression (coronal view). b. Computed tomography scan of the neck demonstrating the mass and airway compression (axial view).
Laryngoscope 124: November 2014
Sweis et al.: Adenomatous Multinodular Goiter in an Infant
2637
Fig. 3. Nontoxic adenomatous multinodular goiter (low power).
granulomatous disease (7%, including atypical mycobacteria, cat scratch disease, toxoplasmosis, and sarcoidosis), suppurative lymphadenitis (2%), sialadenitis (1%), or other inflammatory lesions (1%). Noninflammatory benign masses accounted for 5% of all neck masses and included inclusion cysts (3%), fibromatoses (2%), and keloids (
Rhinological and Otological Society, Inc.
Case Report
Adenomatous Multinodular Goiter Causing Airway Obstruction in an Infant Auddie M. Sweis, BA; Dharani Guttikonda, BA; David L. Walner, MD; Katherine K. Hamming, MD; Nicholas A. Lygizos, MD; Grant Geissler, MD Nontoxic adenomatous multinodular goiter (AMNG) in infants is a rare condition. We discuss an extremely rare case of a nontoxic AMNG in a 3-month-old female presenting with airway obstruction. Surgical resection of the mass was performed to reduce the burden on the airway and for diagnosis. The literature is reviewed, and the clinical characteristics, pathology, and surgical treatment are discussed. Key Words: Adenomatous multinodular goiter; pediatric; mediastinum; neck mass; airway obstruction. Laryngoscope, 124:2636–2639, 2014
INTRODUCTION Adenomatous multinodular goiter (AMNG) refers to an abnormal growth of the thyroid gland plus at least one thyroid nodule. We report a case of a nontoxic AMNG in a 3-month-old patient. To the best of our knowledge, this is the only reported case of a nontoxic AMNG in an otherwise healthy infant born to a euthyroid mother with prenatal treatment. In addition to discussing the rare diagnosis of a nontoxic AMNG in an infant, this case report also reviews the presentation and etiology of airway obstruction in infants.
CASE REPORT A 3-month-old female presented to the otolaryngology clinic at Advocate Children’s Hospital, Park Ridge, Illinois, because of loud, raspy breathing and suprasternal retractions, both present since birth. The patient was otherwise healthy with a normal birth history and no congenital anomalies, inborn errors of metabolism, or pertinent family history. The infant’s mother has no personal or family history of thyroid disease; and during the pregnancy, she did not consume large amounts of goitrogens, undergo a computed tomography (CT) scan with contrast, have any excess exposure to iodine, or undergo any thyroid function testing. The patient’s
From the Rush Medical College, Rush University (A.M.S., D.G.); the Division of Pediatric Otolaryngology, Rush University Medical Center (D.L.W., K.K.H.), Chicago; the Division of Otolaryngology (N.A.L.), Advocate Lutheran General Hospital; and the Division of Pediatric Surgery (G.G.), Advocate Children’s Hospital (D.L.W., K.K.H.), Park Ridge, Illinois, U.S.A. Editor’s Note: This Manuscript was accepted for publication March 11, 2014. The authors have no funding, financial relationships, or conflicts of interest to disclose. Send correspondence to David L. Walner, MD, 1653 W. Congress Parkway, Chicago, IL, 60612. E-mail: [email protected] DOI: 10.1002/lary.24677
Laryngoscope 124: November 2014
2636
exam was significant for biphasic stridor. No obvious neck mass was detected on inspection or palpation, and the remainder of the exam was unremarkable. In office, fiberoptic laryngoscopy was performed and revealed increased pharyngeal secretions but no appreciable asymmetries or masses. The patient then underwent a more comprehensive evaluation under general anesthesia. Microlaryngoscopy revealed a submucosal bulge along the right posterior pharyngeal wall (Fig. 1). Additionally, compression of the right side of the trachea with flattening and blunting was noted just inferior to the subglottic region, with an estimated narrowing of the airway by 70%. The epiglottis, arytenoid complex, true vocal cords, and subglottic region appeared normal. Normal vocal cord movement was noted at this time. A CT soft tissue neck with contrast was performed and demonstrated a 5.4 cm 3 3.5 cm 3 2.4 cm mass originating in the right lobe of the thyroid gland and extending craniocaudally from the level of C2 to the mediastinum just superior to the aortic arch. The mass induced tracheal deviation to the left and posteriorly and also compressed the trachea at the level of the thoracic inlet (Fig. 2a–Fig. 2b). Postinfusion chest CT showed thoracic inlet compression, resulting in compression of the right innominate vein adjacent to the first rib. The patient’s preoperative thyroid function was assessed. Thyroid stimulating hormone (TSH) was 2.313 microunits/mL and free T4 was 1.5 ng/dL, both of which are within the normal limits of 0.25 to 4.0 microunits/mL for TSH and 0.7 to 1.9 ng/dL for free T4. The patient underwent excision of the right neck mass by the pediatric otolaryngology service, in conjunction with the pediatric surgery service. An incision was made on the right side of the neck. Upon retraction of the sternocleidomastoid muscle (SCM), the mass was Sweis et al.: Adenomatous Multinodular Goiter in an Infant
Fig. 1. Endoscopic photograph of airway compression at the level of the pharynx (before surgery). *The right pharyngeal mass.
discovered just deep to the SCM. The mass was elevated into the neck from the superior mediastinum by blunt dissection. It was then separated from the trachea and the floor of the neck. Finally, a right thyroid lobectomy was performed. The recurrent laryngeal nerve and parathyroid glands were identified and unharmed by the dissection. The pathologist reported the resected specimen to be 5.6 cm 3 3.1 cm 3 2.2 cm. Gross pathological inspection revealed a mass that was described as smooth, tan, and lobulated. Histopathological findings revealed multiple nodules of thyroid follicles separated by thick bands of collagen and appeared to be entirely surrounded by an intact fibrous capsule. The thyroid follicles were of variable size and lined by benign, cuboidal follicular cells filled with abundant col-
loid. Hyperplasia was present, evidenced by papillary infoldings and columnar epithelia. There was no evidence of malignant tissue. The diagnosis was most consistent with a right thyroid lobule with AMNG (Fig. 3). The child was extubated on postoperative day 1 without any complications. The stridor had resolved. On postoperative day 5, microlaryngoscopy revealed a patent airway with minimal residual blunting of the right upper tracheal wall—and normal pharynx, epiglottis, arytenoid structures, true vocal cords, subglottis, and tracheal air columns (Fig. 4). Additionally, a technetium thyroid imaging study showed a normal left thyroid lobe and no visualization of the right thyroid lobe, consistent with a hemithyroidectomy. Three weeks postoperatively, the patient had a normal free T4 of 1.0 ng/ dL with an elevated TSH of 7.648 microunits/mL. The patient was started on 25 mcg levothyroxine (Levothroid) daily. With close follow-up and thyroid hormone replacement, the patient’s TSH levels normalized. These endocrine findings are consistent with primary hypothyroidism secondary to the resection of a goiter with a right thyroid lobectomy. Six months after the surgical intervention and start of the daily levothyroxine, a follow-up visit showed a thriving child with no airway issues and normal TSH and free T4 levels. The child will be maintained on levothyroxine indefinitely, with dosing adjustments made when appropriate.
DISCUSSION The majority of pediatric neck masses are either inflammatory or congenital. Torsiglieri et al. examined 446 neck masses in pediatric patients.1 Congenital masses accounted for 54% of all neck masses and included branchial cleft cysts (18%), thyroglossal duct cysts (16%), dermoid cysts (10%), lymphangiomas (8%), and hemangiomas (2%). Inflammatory lesions accounted for 27% of all neck masses and included reactive lymphadenopathy (16%),
Fig. 2. a. Computed tomography scan of the neck demonstrating the mass and airway compression (coronal view). b. Computed tomography scan of the neck demonstrating the mass and airway compression (axial view).
Laryngoscope 124: November 2014
Sweis et al.: Adenomatous Multinodular Goiter in an Infant
2637
Fig. 3. Nontoxic adenomatous multinodular goiter (low power).
granulomatous disease (7%, including atypical mycobacteria, cat scratch disease, toxoplasmosis, and sarcoidosis), suppurative lymphadenitis (2%), sialadenitis (1%), or other inflammatory lesions (1%). Noninflammatory benign masses accounted for 5% of all neck masses and included inclusion cysts (3%), fibromatoses (2%), and keloids (
