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Autoamputation of a pharyngeal hairy polyp in a neonate with intermittent respiratory distress
International Journal of Pediatric Otorhinolaryngology Extra (2008) 3, 90—93
www.elsevier.com/locate/ijporl
CASE REPORT
Autoamputation of a pharyngeal hairy polyp in a neonate with intermittent respiratory distress Sevim Unal a, Sevda Eker b, Ayse Esin Kibar b,*, Tulin Savas b, Unsal Han c, Hasan Basri d a
Neonatal Intensive Care Unit, Republic of Turkey Ministry of Health Ankara Dıskapı Children’s and Research Hospital, Dıskapı, Ankara, Turkey b Department of Pediatrics, Republic of Turkey Ministry of Health Ankara Dıskapı Children’s and Research Hospital, Dıskapı, Ankara, Turkey c Division of Pathology, Republic of Turkey Ministry of Health Ankara Dıskapı Research Hospital, Dıskapı, Ankara, Turkey d Division of Radiology, Republic of Turkey Ministry of Health Ankara Dıskapı Research Hospital, Dıskapı, Ankara, Turkey Received 25 March 2007; received in revised form 26 November 2007; accepted 26 November 2007 Available online 14 January 2008
KEYWORDS Hairy polyp; Autoamputation; Respiratory distress; Neonate
Summary Hairy polyps are rare congenital benign tumours usually present as a pedunculated mass in the oronasopharynx. We describe a case with a hairy polyp originating from the lateral pharyngeal wall of a neonate, causing intermittent respiratory failure. Symptoms in the neonate disappeared following autoamputation of the mass. Cranial magnetic resonance imaging (MRI) and histopathologic findings were also described. To our knowledge, this is the second case described, with full recovery following autoamputation of a hairy polyp. # 2007 Elsevier Ireland Ltd. All rights reserved.
Introduction Hairy polyps (HP) are rare developmental malformations of the oronasopharynx derived from two germinal layers, ectoderm and mesoderm [1]. They are benign lesions with limited growth potential and most frequently seen as a pedunculated mass in predominantly female neonate [1,2]. Polyhydramnios may * Corresponding author. Tel.: +90 312 5969665; fax: +90 312 3472330. E-mail address: [email protected] (A.E. Kibar).
occur during pregnancy due to obstruction of the foetal swallowing mechanism [3]. There are no reported cases of malignant transformation. They typically present at or shortly after birth, and clinical symptoms depend on the size and location of the polyp, respiratory distress or feeding difficulty [1—4]. Here we describe a female neonate, presenting with intermittent respiratory distress due to pharyngeal HP, and a full recovery following autoamputation of the mass. To our knowledge, this is the second case whose symptoms resolved after autoamputation of the pharyngeal HP.
1871-4048/$ — see front matter # 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.pedex.2007.11.004
Hair polyp in neonates with intermittent respiratory distress
Case report A female neonate with respiratory distress was referred to our hospital on the first postnatal day due to respiratory distress. The patient was born to a 27 years old Turkish mother at 37 weeks of gestation, with spontaneous vaginal delivery. The parents were first degree consanguineous. There were no maternal illnesses, exposure to X-ray irradiation, drug ingestion during pregnancy and no family history of congenital anomalies. The pregnancy was complicated with polyhydramnios in the third trimester. The mother’s obstetric ultrasonography revealed no foetal abnormality. Her birth weight was 2900 g, and Apgar scores at 1st and 5th minutes were 8 and 9, respectively. The patient’s physical examination showed tachypnea, nasal flaring, grunting, occasional stridor, suprasternal retractions, and central cyanosis. Her weight was 3040 g, and head circumference 34 cm. She was intubated and commenced on mechanical ventilation plus ampiric antibiotherapy. There was no abnormal appearance in the oronasopharyngeal cavity during endotracheal intubation. A nasogastric tube was easily inserted into the stomach from each nostril. According to blood gas analysis and clinical findings, she was extubated and put on oxygen with a head-mask on the second day of admission. Respiratory distress was disappeared, but recurred 3 days later. The neonate was then reintubated. A mobile, sausage-shaped, pale, non-tender, skin covered, 2.5 cm 2 cm soft mass was seen over vocal cords. On occasions this mass disappeared, but the neonate continued to have episodes of intermittent respiratory distress and drooling of saliva. Whenever a nasal endotracheal tube was inserted to maintain an open airway, respiratory failure of the neonate disappeared. Due to technical problems nasopharyngoscopy could not be performed. Preoperative documentation was assessed by magnetic resonance imaging (MRI). A pharyngeal ovoid mass, extending into the hypopharynx was identified. It was attached to the left lateral pharyngeal wall, with a pedicle (Fig. 1a—d). We planned surgical excision, and the baby was in good health with no respiratory distress. She no longer required intubation with endotracheal tube, and fed with an orogastric tube plus total parenteral nutrition. The day before resection of the polyp, a gray, round shaped mass was seen in the stool. Histopathologic examination of this mass showed a 2.5 cm 2 cm polypoid mass lined by stratified, keratinized squamous epithelium, with skin appendages: hair follicles, sweat and sebaceous glands. It was presumed that autoamputation of the mass might be due to torsion of the mass resulting in
91
ischaemic necrosis. The torsion of the mass probably increased with the placement of an endotracheal tube. She was discharged with full recovery. She was in good health after 3, 6 and 9 months with normal growth and feeding. There were no complaint and symptom.
Discussion HP are uncommon, benign, developmental malformations of the oronasopharynx [1,2]. Female infants are six times more commonly affected than males [1—4]. There is no evidence to indicate a familial incidence [4]. There are reports of an association with other congenital anomalies such as osteopetrosis, facial hemihypertrophy, soft—hard palate clefts, left carotid artery agenesis, agenesis of the uvula and external auricle and ankyloglossia [5]. HP most frequently arise from the nasopharynx, superior aspect of the soft palate or lateral pharyngeal wall as a pedunculated tumour. The oropharynx is the second site of origin [1—5]. They classically present at, or shortly after birth either as asymptomatic sausage-shaped mass or with respiratory obstruction. Airway obstruction is characteristically intermittent, with stertor developing when the polyp falls back into the larynx, causing a ball-valve effect with resulting cyanosis [3,6]. They may also present with feeding difficulties, as the lesion may prolapse into the upper oesophagus, resulting in attacks of coughing or gagging [4]. Signs and symptoms may include Eustachian tube dysfunction, vomiting, asphyxia, hemoptysis, unilateral nostril drainage, a detectable mass [5,6]. Intermittent respiratory failure requiring mechanical ventilation was the main symptom of our case. In addition, feeding difficulty, drooling of saliva, a sausage-shaped mobile mass over the vocal cords appearing occasionally were the other symptoms in this case. HP might be easily overlooked by endotracheal intubation because they are mobile, soft and pedunculated masses. Budenz reported a case of pharyngeal hairy polyp obscured by physical examination and endotracheal intubation, but diagnosed on brain MRI [2]. We noticed the pharyngeal mass on recurrent intubation in this case. We suggested this might be due to mobile and pedunculated characteristics of the mass. As the mass was pedunculated, it might be located through the lower oesophagus and obscured during intubation. Rhinopharyngoscopy could not be performed in our hospital, and in the maternity hospital where the neonate was born, because of technical difficulty. We managed to visualise the size, origin and extent of the mass by
92
S. Unal et al.
Figure 1
(a—d) Magnetic resonance imaging of the hairy polyp of pharynx.
MRI. Placement of a nasal endotracheal tube maintained an open airway, resolved respiratory distress and prevented putting the case on mechanical ventilation. On the other hand, placement of a nasal endotracheal tube increased drooling of saliva. Jarvis et al. also reported a neonate with nasopharyngeal HP who settled well after inserting a nasal endotracheal tube [4]. The differential diagnosis of a neonatal nasopharyngeal mass includes teratoma, meningoencephalocele, nasal glioma, neuroblastoma, haemangioma, thymic-thyroglossal or a lingual cyst [3,7]. In the case of a mass in the upper respiratory tract, the first diagnostic investigation is rhinopharyngoscopy. This method should help to determine the size, origin and extent of mass. Radiological studies are crucial to help delineate the extent and origin of the lesion. MRI and computed tomography (CT), mostly used today are useful for surgical planning [3,5—7].
The high fat content of hairy polyps is well demonstrated on CT and MRI scans, and is useful to distinguish them from vascular and neurologic lesions [7]. Bony anomalies and dehiscence are best identified by CT scanning; intracranial extension and sagittal images are best demonstrated by MRI. In this case, the lesion was evaluated by MRI (Fig. 1). Pedunculated HP can be cured by surgical excision of the mass at the pedicle base [1,8]. Suture ligation or circumferential excision is carried out for sessile tumours. Surgical excision for our patient was planned. Unfortunately, the lesion had undergone spontaneous autoamputation. We suggested that autoamputation of the mass might be due to torsion of the mass resulting in ischaemic necrosis. This is the second case reporting autoamputation of hairy polyp with full recovery. Large cervical and oropharyngeal teratomas or HP could obstruct the swallowing mechanism of the
Hair polyp in neonates with intermittent respiratory distress foetus resulting in polyhydramnios [3,9—11]. Langer reported a foetus of 23 weeks of gestation presenting with polyhydramnios due to a large cervical teratoma [10]. The larger the tumour the greater the increase in amniotic fluid volume. In this case polyhydramnios was determined by ultrasonography, unfortunately pharyngeal mass could not be seen antenatally. Prenatal diagnosis of masses of the head and neck is important especially for appropriate perinatal management, including establishing an airway after delivery. It can be summarized that the diagnosis of HP should be considered in any pregnancy complicated by polyhydramnios, and in any infant with a history of intermittent airway obstruction. Significant morbidity can be avoided if early diagnosis and treatment are undertaken. We also wanted to underline the need for a multidisciplinary team approach.
[3]
[4] [5]
[6]
[7]
[8]
[9]
References [10] [1] D. De Caluwe, S.M. Kealey, R. Hayes, P. Puri, Autoamputation of a congenital hairy polyp, Pediatr. Surg. Int. 18 (2002) 548—549. [2] C.L. Budenz, M.M. Lesperance, S. Gebarski, Hairy polyp of the pharynx obscured on physical examination by endotra-
[11]
93
cheal tube, but diagnosed on brain imaging, Pediatr. Radiol. 35 (11) (2005) 1107—1109. R.M. Walsh, G. Philip, N.Y. Salama, Hairy polyp of the oropharynx: an unusual cause of intermittent neonatal airway obstruction, Int. J. Pediatr. Otorhinolaryngol. 34 (1996) 129—134. S.J. Jarvis, P.D. Bull, Hairy polyps of the nasopharynx, J. Laryngol. Otol. 116 (2002) 467—469. A. Kelly, I.D. Bough, J.D. Luft, C. Conard, J.S. Reilly, D Tuttle, Hairy polyp of the oropharynx: case report and literature review, J. Pediatr. Surg. 31 (5) (1996) 704—706. J.V. Haesendock, P.H. Van de Heyning, J. Claes, G. Goovaerts, P.V. Reempst, A.D. Schepper, J.F. Marquet, A pharyngeal hairy polyp causing neonatal airway obstruction: a case study, Int. J. Pediatr. Otorhinolaryngol. 19 (1990) 175— 180. S.C. Kochanski, E.M. Burton, F.G. Seidel, L.R. Chanin, S. Hensley, J.D. Acker, Neonatal nasopharyngeal hairy polyp: CT and MR appearance, J. Comput. Assist. Tomogr. 14 (6) (1990) 1000—1001. J.L. Roh, Transoral endoscopic resection of a nasopharyngeal hairy polyp, Int. J. Pediatr. Otorhinolaryngol. 68 (2004) 1087—1090. J.T. Zerella, F.J. Finberg, Obstruction of the neonatal airway from teratomas, Surg. Gynecol. Obstet. 170 (1990) 126—131. J.C. Langer, T. Tabb, P. Thompson, B.A. Paes, C.C. Caco, Management of prenatally diagnosed tracheal obstruction, Fetal. Diagn. Ther. 7 (1) (1992) 12—16. M.J. McMahon, N.C. Chescheir, J.A. Kuller, S.R. Wells, L.N. Wright, D.K. Nakayama, Perinatal management of a lingual teratoma, Obstet. Gynecol. 87 (5 Pt 2) (1996) 848—851.
Available online at www.sciencedirect.com
www.elsevier.com/locate/ijporl
CASE REPORT
Autoamputation of a pharyngeal hairy polyp in a neonate with intermittent respiratory distress Sevim Unal a, Sevda Eker b, Ayse Esin Kibar b,*, Tulin Savas b, Unsal Han c, Hasan Basri d a
Neonatal Intensive Care Unit, Republic of Turkey Ministry of Health Ankara Dıskapı Children’s and Research Hospital, Dıskapı, Ankara, Turkey b Department of Pediatrics, Republic of Turkey Ministry of Health Ankara Dıskapı Children’s and Research Hospital, Dıskapı, Ankara, Turkey c Division of Pathology, Republic of Turkey Ministry of Health Ankara Dıskapı Research Hospital, Dıskapı, Ankara, Turkey d Division of Radiology, Republic of Turkey Ministry of Health Ankara Dıskapı Research Hospital, Dıskapı, Ankara, Turkey Received 25 March 2007; received in revised form 26 November 2007; accepted 26 November 2007 Available online 14 January 2008
KEYWORDS Hairy polyp; Autoamputation; Respiratory distress; Neonate
Summary Hairy polyps are rare congenital benign tumours usually present as a pedunculated mass in the oronasopharynx. We describe a case with a hairy polyp originating from the lateral pharyngeal wall of a neonate, causing intermittent respiratory failure. Symptoms in the neonate disappeared following autoamputation of the mass. Cranial magnetic resonance imaging (MRI) and histopathologic findings were also described. To our knowledge, this is the second case described, with full recovery following autoamputation of a hairy polyp. # 2007 Elsevier Ireland Ltd. All rights reserved.
Introduction Hairy polyps (HP) are rare developmental malformations of the oronasopharynx derived from two germinal layers, ectoderm and mesoderm [1]. They are benign lesions with limited growth potential and most frequently seen as a pedunculated mass in predominantly female neonate [1,2]. Polyhydramnios may * Corresponding author. Tel.: +90 312 5969665; fax: +90 312 3472330. E-mail address: [email protected] (A.E. Kibar).
occur during pregnancy due to obstruction of the foetal swallowing mechanism [3]. There are no reported cases of malignant transformation. They typically present at or shortly after birth, and clinical symptoms depend on the size and location of the polyp, respiratory distress or feeding difficulty [1—4]. Here we describe a female neonate, presenting with intermittent respiratory distress due to pharyngeal HP, and a full recovery following autoamputation of the mass. To our knowledge, this is the second case whose symptoms resolved after autoamputation of the pharyngeal HP.
1871-4048/$ — see front matter # 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.pedex.2007.11.004
Hair polyp in neonates with intermittent respiratory distress
Case report A female neonate with respiratory distress was referred to our hospital on the first postnatal day due to respiratory distress. The patient was born to a 27 years old Turkish mother at 37 weeks of gestation, with spontaneous vaginal delivery. The parents were first degree consanguineous. There were no maternal illnesses, exposure to X-ray irradiation, drug ingestion during pregnancy and no family history of congenital anomalies. The pregnancy was complicated with polyhydramnios in the third trimester. The mother’s obstetric ultrasonography revealed no foetal abnormality. Her birth weight was 2900 g, and Apgar scores at 1st and 5th minutes were 8 and 9, respectively. The patient’s physical examination showed tachypnea, nasal flaring, grunting, occasional stridor, suprasternal retractions, and central cyanosis. Her weight was 3040 g, and head circumference 34 cm. She was intubated and commenced on mechanical ventilation plus ampiric antibiotherapy. There was no abnormal appearance in the oronasopharyngeal cavity during endotracheal intubation. A nasogastric tube was easily inserted into the stomach from each nostril. According to blood gas analysis and clinical findings, she was extubated and put on oxygen with a head-mask on the second day of admission. Respiratory distress was disappeared, but recurred 3 days later. The neonate was then reintubated. A mobile, sausage-shaped, pale, non-tender, skin covered, 2.5 cm 2 cm soft mass was seen over vocal cords. On occasions this mass disappeared, but the neonate continued to have episodes of intermittent respiratory distress and drooling of saliva. Whenever a nasal endotracheal tube was inserted to maintain an open airway, respiratory failure of the neonate disappeared. Due to technical problems nasopharyngoscopy could not be performed. Preoperative documentation was assessed by magnetic resonance imaging (MRI). A pharyngeal ovoid mass, extending into the hypopharynx was identified. It was attached to the left lateral pharyngeal wall, with a pedicle (Fig. 1a—d). We planned surgical excision, and the baby was in good health with no respiratory distress. She no longer required intubation with endotracheal tube, and fed with an orogastric tube plus total parenteral nutrition. The day before resection of the polyp, a gray, round shaped mass was seen in the stool. Histopathologic examination of this mass showed a 2.5 cm 2 cm polypoid mass lined by stratified, keratinized squamous epithelium, with skin appendages: hair follicles, sweat and sebaceous glands. It was presumed that autoamputation of the mass might be due to torsion of the mass resulting in
91
ischaemic necrosis. The torsion of the mass probably increased with the placement of an endotracheal tube. She was discharged with full recovery. She was in good health after 3, 6 and 9 months with normal growth and feeding. There were no complaint and symptom.
Discussion HP are uncommon, benign, developmental malformations of the oronasopharynx [1,2]. Female infants are six times more commonly affected than males [1—4]. There is no evidence to indicate a familial incidence [4]. There are reports of an association with other congenital anomalies such as osteopetrosis, facial hemihypertrophy, soft—hard palate clefts, left carotid artery agenesis, agenesis of the uvula and external auricle and ankyloglossia [5]. HP most frequently arise from the nasopharynx, superior aspect of the soft palate or lateral pharyngeal wall as a pedunculated tumour. The oropharynx is the second site of origin [1—5]. They classically present at, or shortly after birth either as asymptomatic sausage-shaped mass or with respiratory obstruction. Airway obstruction is characteristically intermittent, with stertor developing when the polyp falls back into the larynx, causing a ball-valve effect with resulting cyanosis [3,6]. They may also present with feeding difficulties, as the lesion may prolapse into the upper oesophagus, resulting in attacks of coughing or gagging [4]. Signs and symptoms may include Eustachian tube dysfunction, vomiting, asphyxia, hemoptysis, unilateral nostril drainage, a detectable mass [5,6]. Intermittent respiratory failure requiring mechanical ventilation was the main symptom of our case. In addition, feeding difficulty, drooling of saliva, a sausage-shaped mobile mass over the vocal cords appearing occasionally were the other symptoms in this case. HP might be easily overlooked by endotracheal intubation because they are mobile, soft and pedunculated masses. Budenz reported a case of pharyngeal hairy polyp obscured by physical examination and endotracheal intubation, but diagnosed on brain MRI [2]. We noticed the pharyngeal mass on recurrent intubation in this case. We suggested this might be due to mobile and pedunculated characteristics of the mass. As the mass was pedunculated, it might be located through the lower oesophagus and obscured during intubation. Rhinopharyngoscopy could not be performed in our hospital, and in the maternity hospital where the neonate was born, because of technical difficulty. We managed to visualise the size, origin and extent of the mass by
92
S. Unal et al.
Figure 1
(a—d) Magnetic resonance imaging of the hairy polyp of pharynx.
MRI. Placement of a nasal endotracheal tube maintained an open airway, resolved respiratory distress and prevented putting the case on mechanical ventilation. On the other hand, placement of a nasal endotracheal tube increased drooling of saliva. Jarvis et al. also reported a neonate with nasopharyngeal HP who settled well after inserting a nasal endotracheal tube [4]. The differential diagnosis of a neonatal nasopharyngeal mass includes teratoma, meningoencephalocele, nasal glioma, neuroblastoma, haemangioma, thymic-thyroglossal or a lingual cyst [3,7]. In the case of a mass in the upper respiratory tract, the first diagnostic investigation is rhinopharyngoscopy. This method should help to determine the size, origin and extent of mass. Radiological studies are crucial to help delineate the extent and origin of the lesion. MRI and computed tomography (CT), mostly used today are useful for surgical planning [3,5—7].
The high fat content of hairy polyps is well demonstrated on CT and MRI scans, and is useful to distinguish them from vascular and neurologic lesions [7]. Bony anomalies and dehiscence are best identified by CT scanning; intracranial extension and sagittal images are best demonstrated by MRI. In this case, the lesion was evaluated by MRI (Fig. 1). Pedunculated HP can be cured by surgical excision of the mass at the pedicle base [1,8]. Suture ligation or circumferential excision is carried out for sessile tumours. Surgical excision for our patient was planned. Unfortunately, the lesion had undergone spontaneous autoamputation. We suggested that autoamputation of the mass might be due to torsion of the mass resulting in ischaemic necrosis. This is the second case reporting autoamputation of hairy polyp with full recovery. Large cervical and oropharyngeal teratomas or HP could obstruct the swallowing mechanism of the
Hair polyp in neonates with intermittent respiratory distress foetus resulting in polyhydramnios [3,9—11]. Langer reported a foetus of 23 weeks of gestation presenting with polyhydramnios due to a large cervical teratoma [10]. The larger the tumour the greater the increase in amniotic fluid volume. In this case polyhydramnios was determined by ultrasonography, unfortunately pharyngeal mass could not be seen antenatally. Prenatal diagnosis of masses of the head and neck is important especially for appropriate perinatal management, including establishing an airway after delivery. It can be summarized that the diagnosis of HP should be considered in any pregnancy complicated by polyhydramnios, and in any infant with a history of intermittent airway obstruction. Significant morbidity can be avoided if early diagnosis and treatment are undertaken. We also wanted to underline the need for a multidisciplinary team approach.
[3]
[4] [5]
[6]
[7]
[8]
[9]
References [10] [1] D. De Caluwe, S.M. Kealey, R. Hayes, P. Puri, Autoamputation of a congenital hairy polyp, Pediatr. Surg. Int. 18 (2002) 548—549. [2] C.L. Budenz, M.M. Lesperance, S. Gebarski, Hairy polyp of the pharynx obscured on physical examination by endotra-
[11]
93
cheal tube, but diagnosed on brain imaging, Pediatr. Radiol. 35 (11) (2005) 1107—1109. R.M. Walsh, G. Philip, N.Y. Salama, Hairy polyp of the oropharynx: an unusual cause of intermittent neonatal airway obstruction, Int. J. Pediatr. Otorhinolaryngol. 34 (1996) 129—134. S.J. Jarvis, P.D. Bull, Hairy polyps of the nasopharynx, J. Laryngol. Otol. 116 (2002) 467—469. A. Kelly, I.D. Bough, J.D. Luft, C. Conard, J.S. Reilly, D Tuttle, Hairy polyp of the oropharynx: case report and literature review, J. Pediatr. Surg. 31 (5) (1996) 704—706. J.V. Haesendock, P.H. Van de Heyning, J. Claes, G. Goovaerts, P.V. Reempst, A.D. Schepper, J.F. Marquet, A pharyngeal hairy polyp causing neonatal airway obstruction: a case study, Int. J. Pediatr. Otorhinolaryngol. 19 (1990) 175— 180. S.C. Kochanski, E.M. Burton, F.G. Seidel, L.R. Chanin, S. Hensley, J.D. Acker, Neonatal nasopharyngeal hairy polyp: CT and MR appearance, J. Comput. Assist. Tomogr. 14 (6) (1990) 1000—1001. J.L. Roh, Transoral endoscopic resection of a nasopharyngeal hairy polyp, Int. J. Pediatr. Otorhinolaryngol. 68 (2004) 1087—1090. J.T. Zerella, F.J. Finberg, Obstruction of the neonatal airway from teratomas, Surg. Gynecol. Obstet. 170 (1990) 126—131. J.C. Langer, T. Tabb, P. Thompson, B.A. Paes, C.C. Caco, Management of prenatally diagnosed tracheal obstruction, Fetal. Diagn. Ther. 7 (1) (1992) 12—16. M.J. McMahon, N.C. Chescheir, J.A. Kuller, S.R. Wells, L.N. Wright, D.K. Nakayama, Perinatal management of a lingual teratoma, Obstet. Gynecol. 87 (5 Pt 2) (1996) 848—851.
Available online at www.sciencedirect.com