Bilateral congenital pseudoarthrosis of the clavicles in a newborn Ram R. Kalagiri, MD, Vinayak Govande, MD, Martha Hemingway, DNP, NNP-BC, and Madhava R. Beeram, MD
Bilateral congenital pseudoarthrosis of the clavicles is extremely rare. We report a case of this entity presenting in the neonatal period. We highlight the importance of the differential diagnosis when clavicular fracture shows no evidence of healing or occurs bilaterally.
F
racture of the clavicle in the newborn is not unusual and invariably is unilateral and heals well (Figure 1). In contrast, congenital pseudoarthrosis is nontraumatic but may be confused with the more common traumatic clavicular fracture (Figure 2). In congenital pseudoarthrosis of the clavicle, the two primary ossification centers in the developing clavicle fail to unite in utero during embryogenesis. The etiology is unknown. The two portions of the clavicle are connected by a fibrous bridge that is contiguous with the periosteum, and a synovial membrane develops. Most commonly this disorder is seen on the right side and presents with a clavicular mass/protuberance often beyond the newborn period. Bilateral clavicular pseudoarthrosis is very rare (1) and is often associated with other congenital malformations, e.g., trisomy 22. We report a case of bilateral pseudoarthrosis of the clavicle diagnosed in the nursery soon after birth. Recognition of this condition and its nontraumatic etiology is important since the workup and outcome are very different from traumatic clavicular fracture (2). CASE HISTORY A 34-week 2.11-kg female preterm infant was born to a white, married (nonconsanguineous) 21-year-old G1, HIV-, syphilis- and hepatitis B–negative mother who had an uncomplicated pregnancy except for the premature onset of labor. No antenatal steroids were given. The family history was noncontributory. Delivery was vaginal without forceps, and Apgar scores were 9 and 9. Minimal resuscitation in the delivery room was required. The infant had immediate onset of respiratory distress. Her birth weight was 2110 g; length, 47 cm; and head circumference, 29 cm. All measurements were appropriate for the gestational age. Vital parameters were stable. There was a 1 × 2 cm cystic, nontender mass in the left anterior neck that caused no airway obstruction. The rest of the physical examination was unremarkable. Proc (Bayl Univ Med Cent) 2016;29(4):387–388
Figure 1. Chest x-ray showing left-sided clavicular fracture. Case courtesy of Dr. David Cuete; reprinted from http://radiopaedia.org/cases/birth-fracture-ofthe-clavicle.
The baby was admitted to the neonatal intensive care unit secondary to prematurity and respiratory distress. A chest radiograph (Figure 2a) performed for respiratory distress revealed findings of respiratory distress syndrome along with findings consistent with bilateral pseudoarthrosis of the clavicles. The infant was treated with surfactant and oxygen therapy. An osseous survey revealed 11 rib-bearing thoracic vertebral bodies and 6 lumbar-type vertebral bodies. Renal and brain sonograms were unremarkable. An ultrasound of the neck showed a cystic lesion with possible diagnosis of third branchial cleft cyst versus thyroglossal duct cyst. The cyst did not cause feeding or respiratory difficulty. The neck mass gradually became smaller and was unrecognizable at the time of discharge. The baby was discharged home after a few weeks. A genetic evaluation showed normal chromosomes. There is a high likelihood of congenital bilateral pseudoarthrosis of the clavicles, as there was no evidence of fracture healing on sequential imaging (Figure 2b). From Department of Pediatrics, Texas A&M Health Science Center College of Medicine, McLane Children’s Hospital, and Baylor Scott & White Health, Temple, Texas. Corresponding author: Vinayak Govande, MD, Department of Pediatrics, Baylor Scott & White Health, 2401 S. 31st St., Temple, TX 76508 (e-mail:
[email protected]). 387
a
b
Figure 2. (a) Initial chest x-ray obtained in November 2016 showing bilateral clavicle deformities (pseudoarthrosis). (b) Follow-up x-ray a month later showing persistent bilateral clavicle deformities, or nonhealing deformities.
DISCUSSION Congenital pseudoarthrosis is an uncommon congenital anomaly of the clavicles (1), right-sided being the most common (3–5). It is often asymptomatic (6). Our case of bilateral pseudoarthrosis of the clavicles presenting in the neonatal period is very rare. The baby was admitted to the neonatal intensive care unit due to respiratory distress; otherwise, this diagnosis would have been missed. It was associated with possible branchial cleft cyst versus thyroglossal duct cyst, 11 rib-bearing thoracic vertebral bodies, and 6 lumbar-type vertebral bodies. We did not find cervical ribs, cranio-cleido dysostosis, or dextrocardia, as described by Lloyds-Roberts and colleagues (7). The clavicle is the first bone to ossify in the fetal period. There are two ossification centers in the clavicle, a medial and a lateral. A bridge forms between them, which gets ossified to form the clavicle (8). As per Cadilhac and colleagues, failure of ossification of the bridge that connects these two ossification centers may lead to pseudoarthrosis (3). While fracture of the clavicle and congenital pseudoarthrosis have a similar radiological appearance, they have different etiologies and outcomes. One should consider a diagnosis of pseudoarthrosis if the clavicular fracture does not show evidence of healing. Table 1 shows key differences between clavicular fracture and unilateral and bilateral pseudoarthrosis of the clavicle. A fractured clavicle heals well without any sequelae, while pseudoarthrosis persists
throughout life. Often corrective surgeries are done at a later age (1). Surgical repair is indicated when symptoms limit daily activities and for aesthetic reasons. Open reduction and internal fixation result in good outcomes (9). 1.
2. 3.
4.
5. 6.
7.
8.
9.
Sung TH, Man EM, Chan AT, Lee WK. Congenital pseudarthrosis of the clavicle: a rare and challenging diagnosis. Hong Kong Med J 2013;19(3): 265–267. Uhing MR. Management of birth injuries. Clin Perinatol 2005;32(1):19– 38. Cadilhac C, Fenoll B, Peretti A, Padovani JP, Pouliquen JC, Rigault P. Congenital pseudarthrosis of the clavicle: 25 childhood cases. Rev Chir Orthop Repar Appar Mot 2000;86:575–580. Nieto Gil A, Gómez Navalón A, Zorrilla Ribot P. Bilateral congenital pseudarthrosis of the clavicle. A clinical case. Rev Esp Cir Ortop Traumatol 2015 May 5 [Epub ahead of print]. Owen R. Congenital pseudarthrosis of the clavicle. J Bone Joint Surg Br 1970;52(4):644–652. Persiani P, Molayem I, Villani C, Cadilhac C, Glorion C. Surgical treatment of congenital pseudarthrosis of the clavicle: a report on 17 cases. Acta Orthop Belg 2008;74(2):161–166. Lloyd-Roberts GC, Apley AG, Owen R. Reflections upon the aetiology of congenital pseudarthrosis of the clavicle. With a note on cranio-cleido dysostosis. J Bone Joint Surg Br 1975;57(1):24–29. Ogata S, Uhthoff HK. The early development and ossification of the human clavicle—an embryologic study. Acta Orthop Scand 1990;61(4): 330–334. Galvin JW, Dannenbaum JH IV, Grassbaugh JA, Eichinger JK. Pseudarthrosis of the clavicle. Orthopedics 2014;37(5):295–350.
Table 1. Key differences between clavicular fracture and pseudoarthrosis of the clavicle Condition Clavicular fracture
Incidence
Treatment
History of birth trauma
Heals well without sequelae
Unilateral pseudoarthrosis of clavicle Rare; exact incidence unknown
Nonhealing fracture on one side, without antecedent trauma
Surgical repair if symptomatic
Bilateral pseudoarthrosis of clavicle
Bilateral clavicular fractures without signs of healing on sequential radiographs
Surgical repair if symptomatic
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0.3% to 2.9% (2)
Key features
Extremely rare
Baylor University Medical Center Proceedings
Volume 29, Number 4
