Prenatal consultation with the pediatric otolaryngologist

International Journal of Pediatric Otorhinolaryngology 78 (2014) 679–683 Contents lists available at ScienceDirect International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl Prenatal consultation with the pediatric otolaryngologist§ Andrew R. Scott a,*, Huy Nguyen b, Jeannie C. Kelly c, James D. Sidman d,e a Department of Otolaryngology – Head & Neck Surgery and Facial Plastic Surgery, Floating Hospital for Children – Tufts Medical Center, Boston, MA, United States University of Minnesota Medical School, Minneapolis, MN, United States c Department of Obstetrics and Gynecology, Division of Maternal Fetal Medicine, Tufts Medical Center, Boston, MA, United States d Department of Otolaryngology – Head & Neck Surgery, University of Minnesota, Minneapolis, MN, United States e Children’s ENT and Facial Plastic Surgery, Children’s Hospitals and Clinics of Minnesota, Minneapolis, MN, United States b A R T I C L E I N F O A B S T R A C T Article history: Received 15 November 2013 Received in revised form 14 January 2014 Accepted 28 January 2014 Available online 7 February 2014 Objectives: To examine the spectrum of fetal head and neck anomalies that may prompt prenatal referral and to determine the frequency of these consultations. Study design: Case series with chart review. Methods: The billing databases of two urban pediatric otolaryngology practices were queried for ICD-9 codes corresponding to fetal anomalies between January 2010 and December 2012. The pediatric otolaryngology practices in this study evaluate all fetal head and neck anomalies referred to their respective institutions, including craniofacial disorders. Results: Over a three-year period, 53 women presented for fetal otolaryngology consultation, with each practice seeing approximately one consultation every 6 weeks (every 5 weeks (JDS) and every 7 weeks (ARS)). The average maternal and gestational age at presentation were 28.7 years and 27.2 weeks, respectively. 83% of the cases (n = 44) involved some form of cleft lip with or without cleft palate. Other head and neck anomalies included fetal goiter/other congenital neck mass (9% (n = 5)) and micrognathia (6% (n = 3)). Macroglossia (n = 1) and facial cleft (n = 1) each accounted for 2% of cases. Cleft mothers presented earlier in pregnancy (average 26.8 weeks) than those with a neck mass (average 32.3 weeks) (p < 0.05). Only 3 cases (6%) merited ex utero intrapartum treatment. Conclusions: Depending on the referral practices at a given medical center, craniofacial surgeons rather than pediatric otolaryngologists may be evaluating the majority of fetal head and neck anomalies, as orofacial clefts account for most prenatal consultations. The wide spectrum of congenital neck masses may or may not demand monitoring of the airway during the peripartum period. ß 2014 Elsevier Ireland Ltd. All rights reserved. Keywords: Prenatal consultation Cleft lip and palate Micrognathia Fetal diagnosis Ex utero intrapartum treatment (EXIT) 1. Introduction Since its introduction in the late 1950s, obstetric sonographic examination of the fetus has evolved into a routine part of the prenatal anatomic survey. As imaging modalities have further improved, identification of craniofacial and airway abnormalties during the fetal period has become possible. In a 1996 study by Crombleholme et al., 221 fetuses were referred for consultation based on abnormal prenatal ultrasound findings; 2.5% of these cases involved a head and neck anomaly [1]. In the 21st century, § Presented as a poster at the 2013 American Society of Pediatric Otolaryngology Annual Meeting, Arlington, VA, April 2013. * Corresponding author at: Divisions of Pediatric Otolaryngology and Facial Plastic and Reconstructive Surgery, Floating Hospital for Children at Tufts Medical Center, 800 Washington Street, Box #850, Boston, MA 02111, United States. Tel.: +1 617 636 2820; fax: +1 617 636 1479. E-mail address: [email protected] (A.R. Scott). 0165-5876/$ – see front matter ß 2014 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijporl.2014.01.039 many of these same conditions are now being diagnosed in the second trimester, allowing maternal fetal medicine (MFM) physicians and expecting parents to obtain an expert opinion, long before the birth of the affected child. In tertiary medical centers practicing high-risk obstetrics it is not uncommon for a pediatric otolaryngologist to be consulted for the management of a fetal patient with a head and neck anomaly. Although our input is considered integral to the formulation of a safe and reliable delivery plan in many of these cases, most otolaryngologists have limited (if any) training in maternal-fetal medicine. The first specific aim of this study was to determine the spectrum of fetal head and neck anomalies that may prompt a prenatal consultation. Data was collected from two tertiary pediatric otolaryngology practices located in different parts of the country, each of which evaluates all fetal head and neck anomalies referred to their institution, including craniofacial disorders. The pediatric otolaryngologists in these practices perform cleft lip and palate surgery and serve as the medical 680 A.R. Scott et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 679–683 directors of their respective institution’s multidisciplinary cleft lip and palate team. The second specific aim of this study was to examine possible differences in the volume of prenatal head and neck referrals that may present for consultation in a given year. One of the institutions (Children’s Hospitals and Clinics of Minnesota – Minneapolis) is affiliated with the leading regional referral center for high-risk obstetrics, and the pediatric otolaryngologist at this institution (JDS) has established a referral center for craniofacial disorders over several decades. The other center (Tufts Medical Center) is one of four high-risk obstetrics referral centers in Boston (ARS), Massachusetts. The pediatric otolaryngologist at this institution is in his first decade of practice and was in the process of establishling a cleft and craniofacial practice at the time of this study. 2. Materials and methods After obtaining institutional review board approval, the otolaryngology billing databases from two urban pediatric tertiary referral centers were queried for ICD-9 codes corresponding to fetal anomalies between January 2010 and December 2012. This allowed for identification of all documented prenatal consultations for cervicofacial anomalies referred to these practices. Confirmation of the anatomic defect as well as relevant maternal information regarding the pregnancy and delivery was obtained from the electronic medical record at each respective institution. Data collected retrospectively included maternal and fetal demographics (maternal age, fetal sex and gestational age at the time of consultation), indications for referral (results of prenatal imaging studies), and any action taken after referrals. Details of the delivery as well as any peripartum complications were tabulated as well. The resulting information was entered into a spreadsheet for analysis. 3. Results A total of 53 mothers presented to two pediatric otolaryngology clinics for consultation over a 3-year period. At each of the two practices, there was an average of one prenatal referral every 6 weeks (one referral every 5 weeks in Minneapolis (JDS) and one referral every 7 weeks in Boston (ARS)). Overall, 83% of the cases (44 of 53) involved cleft lip with or without cleft palate. Additional fetal anomalies were present in 9% of these cleft cases (4 of 44), and these abnormalities included holoprosencephaly, Turner’s syndrome, micrognathia and situs inversus. Fetal goiter or an unspecified neck mass accounted for 9% of cases (5 of 53), and approximately 5% of consultations (3 of 53) were for micrognathia. Macroglossia and facial cleft each accounted for 2% of total cases (1 of 53). Representative prenatal images are shown in Fig. 1; the distribution of cases is presented in Table 1. In Minneapolis, approximately 120 newborns with cleft lip with or without cleft palate presented over this 3-year period, 28 of whom (23%) were referred through a prenatal source. In Boston, a total of 21 newborns with cleft lip with or without cleft palate presented over the same time frame, 16 of these patients (76%) were referred through a prenatal source (Fig. 2). Fig. 1. Representative prenatal imaging studies. (A) 3D ultrasound showing left unilateral complete cleft lip and alveolus. (B) Fetal magnetic resonance imaging (MRI) showing coronal T2 image of a fetus with ascites and a large, left cervical lymphatic malformation (arrow: lymphatic malformation, arrow-head: brain). (C) 3D ultrasound of a child with micrognathia, upon delivery the child had isolated Robin sequence with cleft palate. (D) Fetal MRI showing coronal T2 image of a fetus with an oral cavity teratoma (small arrow). A.R. Scott et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 679–683 Table 1 Diagnoses, maternal age and fetal gestational age at time of consultation. Number of cases (n) Average maternal Average gestational age (years) age (weeks) Cleft lip/palate 44 Neck masses 4 3 Micrognathia Macroglossia 1 Facial cleft 1 1 Fetal goiter 28.6 27.5 27 21 29 35 26.1 32.25 26.3 33 28 34 Difference between maternal age at presentation for cleft lip/palate and fetal neck masses are not statistically significant (t-test, p value = 0.62). Differences between gestational age at presentation for cleft lip/palate and fetal neck masses are statistically significant (t-test, p value = 0.04). Maternal age at the time of consultation ranged from 16 to 43 years (average, 28.7 years). Mothers of fetuses diagnosed as having an orofacial cleft were slightly older (average age, 29.6 years) than those of fetuses diagnosed as having unspecified cervical neck mass (average, 25 years) or micrognathia (average, 25 years), but this was not statistically significant (t-test, p value = 0.70 and 0.64, respectively). Gestational age at initial consult ranged from 19 to 37 weeks (average, 27.2 weeks). The average gestational age of a fetus diagnosed with an orofacial cleft was 26.7 weeks, slightly younger than the average gestational age of presentation for a fetal patient with an uncharacterized cervical neck mass (32.2 weeks) (t-test, p value < 0.05). Micrognathia referrals presented on average at 26.3 weeks gestational age. The age of presentation for micrognathia was not significantly different from that of clefting (t-test, p value = 0.90) or neck mass (t-test, p value = 0.23). These data are summarized in Table 1. Approximately 89% (n = 47) of prenatal consultation cases were managed with counseling alone, with no alteration in the delivery 681 plan. Three cases (6%), which involved large neck masses, were felt to merit ex utero intrapartum treatment (EXIT) for stabilization of the fetal airway. In the cases of micrognathia, patients were encouraged to deliver at a tertiary medical center with anesthesia, otolaryngology, and neonatology support. All 3 of these newborns were delivered without complication and had minimal or no airway symptoms in the first 24 hours of life. 4. Discussion Ultrasound is now used almost universally in pregnancy to aid in prenatal diagnosis. A standard (‘‘basic’’ or ‘‘Level I’’) ultrasound can be performed after 18 weeks of gestation to evaluate fetal anatomy, as fetal development by this time allows for adequate visualization of structures in order to detect malformation [2]. The American College of Obstetricians and Gynecologists (ACOG) guidelines include the fetal head, face, upper lip, and neck as essential elements of this exam [2]. A specialized (‘‘targeted’’ or ‘‘Level II’’) ultrasound is typically performed by technicians and physicians with expertise in prenatal diagnosis, and may include additional components determined necessary by the specialist [2]. Typically, a specialized ultrasound is recommended in high-risk patients or when fetal anomalies are detected by a basic ultrasound; however, specialized ultrasounds to evaluate fetal anatomy are sometimes universally offered if they are readily available in the prenatal practice [2]. The ability of ultrasound to diagnose specific malformations can vary widely. For example, the rate of diagnosing myelomeningocele by ultrasound is 95%, whereas the rate of diagnosing transposition of the great arteries can be as low as 6% [3,4]. Additionally, detection rates vary greatly by institution due to the availability of specialists and targeted ultrasound exams; as Fig. 2. Prenatal consults as a referral source for cleft lip with or without cleft palate, established vs. nascent craniofacial practice. Note how prenatal consults were the dominant referral source in a nascent craniofacial practice (76%, n = 16/21) compared to an established craniofacial practice (23%, n = 28/120). 682 A.R. Scott et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 679–683 demonstrated by the RADIUS Trial, non-tertiary care centers were unable to significantly detect any craniofacial malformation, compared to a 73% detection rate of cleft lip at a tertiary care center [5,6]. Furthermore, as the technology behind ultrasound improves, evaluation of fetal anatomy has been attempted as early as the first trimester. A recent systematic review analyzed the ability of ultrasound to detect fetal malformations at 11–14 weeks of gestation, and demonstrated an overall detection rate of 51%, achieving a 92% detection rate of neck anomalies and 34% detection rate of face anomalies. The authors concluded that early ultrasound evaluation of anatomy could not be fully performed due to the longer time required for development of certain fetal structures, including the face [7]. Recently, three-dimensional (3D) ultrasound has also been used to construct topographic renderings of anatomic planes unattainable by conventional ultrasound as another possible tool for detection and diagnosis of fetal anomalies. The use of 3D ultrasound has not demonstrated a difference in obstetrical management or outcome in the few studies that currently exist, and is not recommended by ACOG guidelines at this time [2]. However, rapid advancements in this technology have shown promise especially in confirming the diagnosis and assessing the severity of facial anomalies in conjunction with routine 2D ultrasound [2,8]. We report that the majority of cases (85%) referred for prenatal otolaryngology consultation at our respective institutions involved an orofacial cleft abnormality, with 91% (48/53) of consults involving some sort of craniofacial condition. Cleft lip with or without cleft palate is one of the more common head and neck anomalies. The incidence of cleft lip in the Caucasian population is approximately 1 in 1000 live births. The incidence is doubled in Asian and Latino populations and halved in among patients of African ancestry. Cleft lip with or without associated cleft palate is more common among males. However, isolated cleft palate is more prevalent in females. Combined cleft lip and palate is the most common presentation (50%), followed by isolated cleft palate (30%) and isolated cleft lip (20%) [9]. Improvement of imaging techniques has enabled us to detect orofacial clefts at an increasing rate over the past decade. The use of MRI as an adjunct technique to detect facial clefts has become more popular. It offers more accurate and detailed evaluation of the primary palate when compared to ultrasound [10]. However, fetal MRI is an expensive modality, and has not been proven to be cost effective nor adapted as a routine part of the fetal cleft lip and palate work-up at most institutions. The birth of an infant with undiagnosed cleft lip may result in considerable emotional and psychological stress for unsuspecting parents [11,12]. The initial shock of discovering that a child has a facial disfigurement may be followed by fear, guilt anger and sadness. A recent study showed prenatal diagnosis and consultation of orofacial clefts may better prepare parents for adjusting to the reality of having a newborn with a craniofacial condition [13]. Upwards of 85% of women with a prenatal diagnosis of an orofacial cleft felt that fetal consultation prepared them psychologically for the birth of their cleft child. However other studies have suggested that prenatal consultation may only provide a marginal benefit, if any, over identification of an orofacial cleft at birth; interestingly the outcome most positively affected by early detection and consultation for cleft lip and palate appears to be in preparing parents for feeding problems after birth [14,15]. Parents who visit our clinic for prenatal consultation may be referred to feeding specialists and social workers regarding perinatal care. In these particular cohorts, cleft pregnancies were closely followed until delivery among those families who chose to deliver at our affiliates, and pediatric otolaryngologists were available for immediate consultation regarding airway asssessment at birth. The majority of cleft cases in this study were diagnosed during routine 18 week ultrasound and referred shortly thereafter to either our teams or a MFM provider. In those cases in which MFM was consulted, a confirmatory level 2 or level 3 ultrasound was then performed at approximately 22–24 weeks, prior to referral to otolaryngology. Prenatal consultations in our clinics rarely had an influence on whether or not to pursue an elective pregnancy termination. In our series, the average gestational age of presentation for cleft lip and palate was 27 weeks, well after the cut off for elective pregnancy termination in those states where abortion is legal. In a prior study examining prenatal consultation for orofacial clefting, 93% of these women said that they had not contemplated pregnancy termination [13]. Fetal neck masses accounted for 9% of consultations. These included fetal goiter, lymphatic malformation and cervical and oropharyngeal teratomas. The latter condition is potentially lifethreatening as teratoma may cause significant fetal airway obstruction. In fact, giant fetal neck masses are the most common head and neck indication for an EXIT procedure. One study suggested a mortality rate of 20% if a fetus with a teratoma is delivered in the standard fashion [16]. Steigman et al. reported overall postnatal survival of 93% in 15 cases of congenital neck masses in which the fetuses were delivered by EXIT [17]. Teratomas in particular are reported to be nearly 5 times more likely to be associated with congenital high airway obstruction syndrome (CHAOS) and require surgical manipulation of the airway when compared with cervical lymphatic malformations [18]. Our study reported 3 cases of cervical neck masses that were managed with EXIT in order to assure safe delivery of the fetus. In this series there was a statistically significant difference in the gestational age of consultation for children with cleft lip and palate compared to those with a neck mass, with clefts presenting in the second trimester and fetal neck masses not soliciting consultation until well into the third trimester. This is a troubling trend, as late presentation of an EXIT candidate leaves less time to mobilize resources and prepare for delivery. In these cases, preterm labor may precede multidisciplinary planning, catching the delivery team completely off guard. Suspicion of micrognathia through ultrasound has both airway and systemic implications. A number of congenital syndromes such as Stickler, Treacher Collins, oculo-auriculo-vertebral spectrum, and velocardiofacial syndrome are associated with micrognathia. Additionally chromosomal abnormalities such as trisomy 18 may present with fetal micrognathia along with abnormalities in circulating fetal DNA or certain protein levels detected during fetal non-invasive testing protocols of maternal blood. There are several sonographic criteria that may be used to make the diagnosis of micrognathia. Perhaps the two most common methods are the inferior facial angle (IFA) [19] and the jaw index [20]. Both methods were shown to be effective and accurate in diagnosing micrognathia, with sensitivity and specificity of 100% and 98.9% for the IFA and 100% and 98.7% for the jaw index, respectively. Nevertheless these indices are not usually reported in clinical practice as they have not been validated in large studies nor have they become widely accepted. In cases of suspected fetal micrognathia, prompt genetics consultation allows for chromosomal analysis to be done in a timely fashion. Additionally amniotic fluid index may offer a clue as to the degree of potential airway obstruction [21]. A 2012 retrospective study of 123 newborns with micrognathia suggested that premature labor, intrauterine growth restriction, the presence of a neurologic abnormality or syndromic features on ultrasound are associated with a need for airway intervention in the first 24 h of life [22]. A.R. Scott et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 679–683 A brief comment should be made about our one case of fetal macroglossia. This is an excellent example of how improvements in fetal imaging are outpacing our understanding of the significance of high resolution imaging findings. In this case, serial ultrasounds were performed and routine delivery was undertaken after a normal amniotic fluid index was confirmed. The child had no problems with airway obstruction during infancy and was ultimately diagnosed with Beckwith Wiedeman syndrome. A number of limitations for this study should be noted. First, prenatal referral patterns vary widely across regions and tertiary referral centers due to a number of factors. For example, there is a known correlation between orofacial clefts and certain cardiac and conotruncal anomalies, especially noteworthy in velocardiofacial syndrome. Therefore, regional centers that specialize in repair of complex congenital heart disease are likely seeing a higher volume of cleft lip and palate patients who are referred to these centers based on fetal echocardiogram findings rather than the identification of an incidental orofacial cleft. Conversely, centers with established referral patterns for cleft and craniofacial care independent of cardiac expertise will see a higher volume of newborns that are referred or self-referred after birth. These patients may or may not have received prenatal consultation for a fetal cleft elsewhere but ultimately present to established craniofacial programs for definitive repair (see Fig. 2). Finally, some tertiary pediatric referral centers service a greater proportion of underserved populations, either rural or urban, who have limited access or inclination to pursue specialized (if any) prenatal care. These centers will likely see a lower proportion of prenatal consultations as a regular referral source for congenital head and neck anomalies. 5. Conclusion Advances in prenatal imaging have been effective in diagnosing cervicofacial anomalies during the prenatal period. These consultations comprise a small but significant volume of new referrals to our two pediatric otolaryngology practices, however the cleft patients identified prenatally may make up a significant fraction of the referral source for a developing craniofacial program. This study would suggest that orofacial clefting and craniofacial disorders comprise the majority of prenatal consultations pertaining to the head and neck area. Those pediatric otolaryngologists who routinely care for children with craniofacial disorders are best poised to capture this demographic and provide complete and meaningful prenatal assessment. More importantly, pediatric otolaryngologists in particular may identify any potential airway complications that might develop in the peripartum period. Prompt referral of cervical masses in particular is essential, as some masses pose the threat of airway obstruction upon delivery of the fetus. Ironically, our data would suggest that such neck masses may be presenting to the otolaryngology clinic later in pregnancy, well into the third trimester, thus leaving providers less time to mobilize resources and prepare for delivery. Conflicts of interest None declared. 683 Financial disclosure Andrew Scott is a paid consultant for Advance Medical – expert second opinions; James Sidman is a paid consultant for Medtronic, Inc. Funding source None. References [1] T.M. Crombleholme, M. D’Alton, M. Cendron, B. Alman, M.D. Goldberg, G.T. Klauber, et al., Prenatal diagnosis and the pediatric surgeon: the impact of prenatal consultation on perinatal management, J. Pediatr. 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