Spontaneous pneumorrhachis in adolescents: illustrative case

Abstract

BACKGROUND

Spontaneous pneumorrhachis, defined as the presence of air in the spinal canal without trauma or iatrogenic intervention, is rare in adolescents. The clinical course remains anecdotal, and no standardized management guidelines exist. The authors performed the first systematic review focused on adolescent spontaneous pneumorrhachis and describe an illustrative case.

OBSERVATIONS

A 16-year-old previously healthy female presented with facial crepitus, intractable vomiting, fever, and streptococcal pharyngitis. Imaging revealed extensive pneumomediastinum, subcutaneous emphysema, and posterior epidural pneumorrhachis spanning from C7 to T8. Given a neurointact examination, conservative management was pursued. A systematic review of the literature assessed predisposing conditions, presenting symptoms, associated radiographic findings, and clinical management.

LESSONS

Pneumorrhachis should be considered in adolescents presenting with crepitus following vomiting or respiratory illness, particularly when accompanied by facial or cervical swelling. While the clinical course is typically benign, neurosurgical evaluation is valuable in distinguishing this condition from other causes of intraspinal air and to avoid neurological compromise. We propose a focused approach for the management of spontaneous pneumorrhachis in adolescence focused on treating the underlying condition, conservative management when no neurological deficits are present, and close neurological monitoring.

https://thejns.org/doi/10.3171/CASE26332

Pneumorrhachis is a rare radiological finding characterized by the presence of air within the spinal canal, in either the epidural or intradural spaces. The majority of reported cases occur in adults and result from traumatic or iatrogenic mechanisms.¹ Common causes include blunt or penetrating trauma, spinal or neuraxial procedures such as epidural anesthesia or lumbar puncture, and postoperative complications.¹ In contrast, spontaneous pneumorrhachis occurs in the absence of trauma or procedural intervention and is rare, especially in pediatric and adolescent populations. Spontaneous cases are typically associated with conditions or maneuvers that acutely increase intrathoracic pressure, such as forceful vomiting, intense coughing, asthma exacerbations, Valsalva maneuvers, strenuous exercise, or drug use.^2–4 Elevated intrathoracic pressure can lead to alveolar rupture with air dissecting into the mediastinum, subsequently tracking through cervical fascial planes and neural foramina into the posterior epidural space, which offers the “path of least resistance.”^5–9

Pneumorrhachis is typically discovered incidentally on CT imaging performed for suspected pneumomediastinum or subcutaneous emphysema.¹⁰ Pneumorrhachis is frequently asymptomatic; however, neurological symptoms such as pain, paresthesia, or motor weakness are rare and typically occur in the context of traumatic or iatrogenic etiologies.^10–12 Management involves treating the underlying cause such as perforated viscus, infection, or barotrauma,¹⁰ and for neurologically asymptomatic patients, conservative management and supportive care typically result in spontaneous resolution on serial imaging in the majority of patients.¹ However, the clinical course remains anecdotal, and no standardized management guidelines exist.

Here, we describe a case of spontaneous pneumorrhachis spanning the cervical/thoracic spine with extensive pneumomediastinum and subcutaneous emphysema in a previously healthy adolescent female who presented with intractable vomiting secondary to streptococcal pharyngitis. In addition, we present a systematic review of the literature on spontaneous pneumorrhachis in the adolescent, describe common clinical and radiographic features, explore potential risk factors, and propose a clinical management algorithm for this patient population.

Illustrative Case

A 16-year-old, otherwise healthy female presented to the emergency department with 1 day of bilateral facial swelling and 3 days of intractable vomiting and fever secondary to strep pharyngitis, for which she was being treated with oral amoxicillin. The patient’s vomiting episodes were forceful and repetitive, consistent with a Valsalva-type mechanism contributing to elevated intrathoracic pressure. Subjectively, both the patient and her mother denied any evidence of neurological complaints. Physical examination revealed notable crepitus of the bilateral neck and face, and neurological examination revealed no focal deficits. On presentation, vital signs were within normal limits, including a respiratory rate of 20 breaths per minute, heart rate of 82 beats per minute, and oxygen saturation of 100% on room air. Laboratory studies were notable for leukocytosis (white blood cell count 21.7 × 10³/µL), reflecting her underlying infection, and mild electrolyte abnormalities consistent with recent vomiting, including hypokalemia (3.4 mmol/L) and hypochloremia (96 mmol/L). Hemoglobin and hematocrit were elevated (17.0 g/dL and 49.4%, respectively), likely due to dehydration in the setting of repeated emesis.

Chest radiographic imaging demonstrated significant pneumomediastinum continuing to extensive subcutaneous emphysema of the anterior chest wall extending superiorly into the supraclavicular regions, neck, and shoulders bilaterally (Fig. 1). CT with contrast was performed to rule out Boerhaave syndrome. No esophageal tear was identified; however, CT demonstrated pneumomediastinum, subcutaneous air, and the presence of air within the posterior spinal epidural space spanning from C7 through T8 and with extension to the extraforaminal space at several levels (Fig. 2). MRI was not obtained given the absence of neurological deficits.

FIG. 1.

A: Chest radiograph demonstrating subcutaneous emphysema extending in the supraclavicular regions and neck bilaterally (arrows). B: Lateral chest radiograph demonstrating subcutaneous emphysema throughout the anterior chest wall (red arrow) and pneumomediastinum (yellow arrow).

FIG. 2.

A: Axial CT scan of the thoracic spine showing air in the spinal canal (red arrow) communicating through the neuroforamen to the paravertebral extraforaminal space at the T5 level (yellow arrow). B: Sagittal CT scan of the thoracic spine demonstrating air in the dorsal epidural space of the spinal canal from C7 to T8 (arc).

Conservative management with fluid resuscitation and antiemetics was pursued for symptom management, and observation of the pneumorrhachis given no neurological deficits. On hospital day 2, a chest radiograph was obtained and demonstrated improvement of the pneumomediastinum and subcutaneous emphysema. After progressive clinical improvement, the patient was discharged on hospital day 4. Advanced imaging was planned for the 6-week follow-up; however, the patient was lost to follow-up despite multiple attempts to contact her by both medicine and neurosurgical services.

Informed Consent

The necessary informed consent was obtained in this study.

Discussion

Observations

Spontaneous pneumorrhachis occurs without any direct injury or procedural trigger and is rare, especially in the adolescent age group. It has been associated with conditions that elevate intrathoracic pressure, such as asthma exacerbations,^13,14 respiratory tract infections,^13,15–19 forceful vomiting,^13,20–23 and strenuous exercise,^24,25 or, in some cases, has no clearly identifiable cause.^4,20,26,27 Although spontaneous pneumorrhachis is most often benign and self-limited, its radiological appearance frequently prompts consultation with the neurosurgery team due to concern for potential spinal cord compression, infection, or other structural pathology. However, no formal guidelines exist for the management of pneumorrhachis, and decision-making is largely based on clinical judgment and radiological interpretation.

We conducted a literature review using the PubMed and Scopus databases, focusing on adolescents aged 10–19 years, in accordance with PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines (Fig. 3). We used the search terms (“pneumorrhachis” OR “pneumatorrhachis” OR “spinal canal pneumatosis” OR “epidural pneumatosis” OR “intradural pneumatosis”) AND (“pediatric” OR “child” OR “children” OR “adolescent” OR “teenager”). From 85 unique articles identified, we excluded those with traumatic or iatrogenic causes, non–English-language publications, and papers that did not describe new cases. Of the remaining 46 articles describing spontaneous pneumorrhachis, 34 articles included patients between 10 and 19 years of age, accounting for a total of 38 adolescents (Table 1).^4,13–45 Study selection and data extraction were reviewed by two authors, with high agreement (94%), and discrepancies were resolved through discussion.

FIG. 3.

PRISMA flow diagram for systematic review of spontaneous pneumorrhachis in adolescents.

TABLE 1.

Reported cases of spontaneous pneumorrhachis in adolescents in the literature

Authors & Year	Age (yrs)	Sex	Past Medical History	Precipitating Event	Presenting Symptoms & Physical Exam Findings	Radiological Findings on CT	Management	Outcome*
Angi et al., 202028	17	M	Allergic asthma	Cough	Dry cough for 3 days, shortness of breath, wheezing, chest pain, palpable crepitus on rt neck	Extensive subcutaneous emphysema, pneumomediastinum, pneumorrhachis at C6–9	Supplemental oxygen, bronchodilators, systemic steroids, & antibiotics	Discharged after 5 days; full recovery
Behr et al., 201829	17	M	None	Cough	Cough w/ post-tussive emesis	Extensive pneumomediastinum, soft tissue emphysema, pneumorrhachis	—	Discharged; full recovery
Bernaerts et al., 200330	17	M	None	Ecstasy snorting	Pleuritic chest pain w/ associated shortness of breath	Pneumomediastinum, subcutaneous emphysema, pneumorrhachis	Observation & monitoring w/ no additional therapy	Discharged; full recovery
Bhadane et al., 201131	14	M	Bronchial asthma	Unknown	Sudden onset of shortness of breath, nonproductive cough, & wheezing for 2 days; systolic crunching sound over the chest	Pneumorrhachis, pneumomediastinum, pneumopericardium, subcutaneous emphysema	Antibiotics, bronchodilators, systemic corticosteroids, & high-flow oxygen	Discharged; full recovery
Buonsenso et al., 202115	17	M	None	SARS-CoV-2	Bilat chest & cervical pain, subcutaneous emphysema in neck & chest	Bilat apical pneumothoraces, subcutaneous emphysema, pneumorrhachis	Close clinical observation	Discharged; full recovery
Campos et al., 202432	18	M	Asthma, smoking history	Nonproductive cough	Dyspnea, stabbing chest pain in hemithorax radiating to back	Subcutaneous emphysema, pneumomediastinum, pneumorrhachis, bilat parenchymal hypodensities & scattered atelectatic bands	Supportive care including conservative oxygen therapy	Discharged; full recovery
Carolan et al., 201320	16	M	Asthma	Unknown	Fever, cough, vomiting w/ significant thoracic & cervical pain	Subcutaneous emphysema, pneumomediastinum, epidural pneumorrhachis	Symptomatic therapy w/ close observation	Discharged after 2 days; full recovery
Chang et al., 202313	16	M	Asthma	Asthma exacerbation	—	Subcutaneous emphysema, pneumorrhachis at C3–L2	Treatment of asthma exacerbation & supplemental oxygen therapy	Discharged
	18	F	None	Type A influenza	—	Subcutaneous emphysema, pneumorrhachis at C2–T3	Conservative management w/ supportive measures	Discharged
	16	M	None	Common cold	—	Pneumomediastinum w/ associated intraspinal air tracking along the posterior epidural space at T1–6	Conservative management w/ supportive measures	Discharged
	15	M	Anorexia nervosa	Anorexia nervosa, self-induced vomiting	—	Subcutaneous emphysema of the chest wall, pneumomediastinum, pneumoretroperitoneum, epidural pneumorrhachis at C3–T8	Conservative management w/ supportive measures	Discharged
	18	M		Cough	—	Subcutaneous emphysema & pneumoretroperitoneum at C4–T6	Conservative management w/ supportive measures	Discharged
Dontu & Kramer, 200733	10	M	None	Biking	Acute dyspnea & anterior neck & throat pain while biking	Small bilat pneumothoraces, pneumomediastinum, subcutaneous emphysema, air in the epidural space	IV fluids & observation	Discharged after 3 days; full recovery
Drevelengas et al., 199434	16	F	None	Cough	Severe parasternal chest pain & significant dyspnea	Pneumomediastinum, extensive subcutaneous emphysema, epidural pneumorrhachis	Supportive observation	Discharged; full recovery
Drolet et al., 200726	18	M	Type 1 diabetes	Unknown	Isolated headache w/o respiratory complaints	Mediastinal & spinal epidural air accumulation	Expectant management w/ serial imaging & observation	Discharged; full recovery
Eesa et al., 200614	18	M	Asthma	Asthma exacerbation	Cervical & upper thoracic swelling, marked crepitus of the neck & upper chest	Pneumomediastinum, air in the cervical subcutaneous tissues, pneumorrhachis	Supplemental oxygen, inhaled bronchodilators, & empiric antibiotics	Discharged; full recovery
Emiralioğlu et al., 201516	12	M	None	RSV	Persistent cough, nasal congestion & rhinorrhea, crepitus over the arms	Pneumomediastinum, subcutaneous emphysema, epidural air	Oxygen therapy	Discharged; full recovery
Hudson et al., 202135	14	M	None	Unknown	Mental status changes, chest pain, decreased oral intake & urinary output, fatigue, coughing, choking while brushing teeth	Pneumomediastinum, soft tissue emphysema, pneumoretroperitoneum, & pneumorrhachis	Supportive care	Discharged; full recovery
Jasmani et al., 202236	14	M	Bronchial asthma	URI	Acute onset of shortness of breath, chest pain, URI symptoms for 3 days	Tracheal perforation w/ pneumomediastinum, pneumopericardium, pneumorrhachis, subcutaneous emphysema	Oxygen therapy, close monitoring, & frequent nebulizers	Discharged after 3 days; full recovery
Kakel & Kennedy, 201124	16	M	None	Influenza, strenuous exercise	Generalized chest tightness & fatigue postexertion	Subcutaneous emphysema & air w/in the posterior epidural space	Management strategy not detailed in report; presumed supportive	Discharged; full recovery
Kelly & Van Opstal, 201922	16	M	Asthma	Marijuana use	Epigastric & periumbilical abdominal pain, chest pain radiating to back, neck swelling, air under the skin, emesis following marijuana use	Pneumorrhachis, subcutaneous emphysema, pneumomediastinum	Conservative management w/ supportive measures	Discharged; full recovery
Liao & Wang, 201525	19	M	None	Strenuous exercise	Shortness of breath & chest pain after exercise w/ swelling & crepitus over neck & shoulders; common cold 1 wk prior	Subcutaneous emphysema, pneumomediastinum, air in epidural space	Conservative management w/ supportive measures	Discharged after 2 days; full recovery
Liu & Liao, 201917	18	M	None	Common cold	Dyspnea & substernal chest pain secondary to viral illness, swollen neck, crepitus	Subcutaneous & epidural air	Supportive care including conservative oxygen therapy	Discharged; full recovery
Manden & Siddiqui, 200937	17	M	Bronchial asthma	Asthma attack	Shortness of breath, nonproductive cough, wheezing, Hamman’s sign	Subcutaneous emphysema, pneumomediastinum, pneumopericardium, epidural pneumorrhachis	Antibiotics, bronchodilators, systemic corticosteroids, & supplemental oxygen	Discharged; full recovery
Nonaka et al., 200938	17	M	None	Unknown	Shortness of breath, neck swelling, subcutaneous emphysema of neck, rt anterior chest wall, & rt axillary region	Pneumomediastinum, subcutaneous emphysema in the cervical, axillary, & chest wall regions; pneumorrhachis at C7–T1; intrapulmonary air w/ rt pulmonary artery dissection	Conservative management w/ supplemental oxygen	Discharged after 7 days; full recovery
Oshimizu et al., 202139	19	M	UC	Acute exacerbation of UC	No neurological or respiratory symptoms including cough	Pneumomediastinum, diffuse subcutaneous emphysema, epidural pneumorrhachis	Subtotal colectomy for UC exacerbation using neuraxial & spinal epidural anesthesia	Discharged; full recovery
Ramses Bedolla-Pulido et al., 201940	18	M	Asthma	Cold weather & saw dust exposure	Thoracic pain, chest tightness, paroxysmal coughing, nasal obstruction, rhinorrhea	Abdominal, mediastinal, & subcutaneous emphysema, epidural air btwn C6 & C7 vertebrae extending over the spinal canal	Bronchodilators, corticosteroids, & empiric antibiotics	Discharged; full recovery
Ronald et al., 202021	15	M	None	Vaping of THC	Fever, chills, cough, & vomiting w/ respiratory distress & hypoxia	Bilat pneumothoraces, mediastinal air, epidural pneumorrhachis	IV corticosteroids w/ supplemental oxygen therapy	Discharged; full recovery
Sadarangani et al., 200941	17	M	None	Weightlifting	Sudden onset of retrosternal chest & neck pain, nonproductive cough, sore throat, dyspnea	Cervical subcutaneous emphysema extending to superior mediastinum, bilat interstitial emphysema, epidural air	Oxygen supplementation, analgesics, antibiotics, & conservative clinical monitoring	Discharged; full recovery
Sandhya et al., 201227	10	F	Dermatomyositis	Unknown	10 mos of intermittent fever, skin rash, joint pain, dry cough, crepitations over infra-axillary & infrascapular areas	Pneumomediastinum, subcutaneous emphysema, interstitial lung disease, epidural emphysema	Prednisolone & conservative management	Discharged; full recovery
Sethi & Cooper, 201823	16	M	None	Alcohol & smoking cannabis	Projectile vomiting following cannabis inhalation, progressive discomfort in the neck & throat, voice alteration	Pneumorrhachis, pneumomediastinum, minor lt pneumothorax	Noninvasive oxygen therapy & supportive care	Discharged; full recovery
Shilpa et al., 201842	16	M	Cerebral germinoma	Unknown	Altered sensorium, swelling & stiffness of the neck & chest, continuous straining, subcutaneous crepitations	Pneumomediastinum, pneumothorax, surgical emphysema, pneumorrhachis, pneumoretroperitoneum	Conservative management	Discharged; full recovery
Showkat et al., 201343	14	M	ALL	Cough	Facial & thoracic edema, crepitus on the chest wall & parotid area, neuropathic pain & hyperreflexia of lower extremities	Pneumomediastinum, subcutaneous emphysema, epidural pneumorrhachis	High-flow oxygen w/ conservative supportive management	Died
Song et al., 20094	18	M	None	Unknown	Dyspnea, productive cough, crepitus on rt shoulder & neck, bilat lower extremity paresthesias	Pneumomediastinum & spinal air	Antitussive therapy for cough suppression	Discharged; full recovery
Sriganesh et al., 201718	18	M	Partially thrombosed venous sac in 4th ventricle, posterior fossa AVF	Lower respiratory tract infection (coagulase-negative staphylococci)	Severe respiratory distress	Rt-sided pneumothorax, pneumopericardium, extensive epidural air	Antibiotics, pleural drainage, & invasive ventilatory support	Died
Tavare et al., 201844	16	F	None	Sniffing of ketamine & inhaling nitrous oxide	Dyspnea, dysphonia, swelling of face, neck & upper torso w/ palpable crepitus	Pneumomediastinum, bilat subcutaneous emphysema, epidural & pericardial air	Supportive oxygen therapy	Discharged; full recovery
Willenbring et al., 202445	16	M	None	Viral URI	Fever, severe cough, post-tussive emesis, acute onset of neck & chest pain	Pneumomediastinum, pneumopericardium, subcutaneous emphysema, pneumorrhachis at C7–L2, gas tracking along left intercostal fascial planes	Nebulized inhalers, antitussives, & supplemental oxygen	Discharged after 4 days; full recovery
Zhuang et al., 202319	12	M	None	Mycoplasma pneumonitis	Sore throat, fever, severe dry cough, swelling & tenderness in neck, face, chest, back, & proximal upper limbs	Pneumomediastinum, pulmonary consolidations, air w/in neck, axillae, paraspinal & abdominal walls, thoracic inlet, & epidural spinal canal	Nasal catheter oxygen, inhalation, antibiotics, & symptomatic care	Discharged in 9 days; full recovery
Present case	16	F	None	Streptococcal pharyngitis	3 days of vomiting & fever, 1 day of bilat facial swelling, crepitus of neck & face	Pneumomediastinum; subcutaneous emphysema of anterior chest wall, supraclavicular regions, neck, & shoulders; pneumorrhachis at C7–T8	Fluid resuscitation, antiemetics, & supportive care	Discharged in 4 days; full recovery

AVF = arteriovenous fistula; IV = intravenous; UC = ulcerative colitis; URI = upper respiratory tract infection; — = not available.

* Hospital length of stay is reported when available; when not reported, only discharge status (discharged, recovered, or died) is reported.

We explored common predisposing features present across adolescents with spontaneous pneumorrhachis. Of the 38 adolescent cases, 34 (89.5%) were male and 4 (10.5%) were female, suggesting a strong male predominance, with a mean age of 16 years. The male predominance observed may reflect behavioral and physiological factors. Adolescent males may be more likely to engage in activities associated with sudden increases in intrathoracic pressure, such as strenuous exertion or inhalational drug use. Additionally, differences in respiratory mechanics or force generation during coughing or vomiting may contribute, although the underlying drivers remain unclear. Most patients were previously healthy, although several had comorbidities such as asthma,^{13,14,20,22,28,31,32,36,37,40} type 1 diabetes,²⁶ or a history of vaping or cannabis use.^21,23,44 Precipitating factors were identified in most cases, with the most frequent triggers being forceful vomiting, upper respiratory infections including severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) or respiratory syncytial virus (RSV), and asthma exacerbation or coughing fits (Table 1). A few cases were due to strenuous exertion or exercise.^24,25,33,41 A small subset of cases were due to inhaled recreational substances, including ecstasy, tetrahydrocannabinol (THC), ketamine, and nitrous oxide.^22,23,44

Various symptoms and physical examination findings were frequently encountered in adolescents with spontaneous pneumorrhachis. Dyspnea and shortness of breath were the most common physical examination findings (17 cases, 45%); however, chest pain (13 cases, 34%), neck or facial swelling (8 cases, 21%), and crepitus or subcutaneous emphysema (15 cases, 39%) were also commonly observed (Table 1). Crepitus and/or subcutaneous emphysema were key clinical findings that prompted further imaging. Despite the presence of air in the spinal canal, the majority of cases were clinically benign. Only 1 case reported neurological symptoms, which included lower extremity neuropathic pain and hyperreflexia.⁴³

Common radiographic features are present within adolescents who develop spontaneous pneumorrhachis. Mediastinal involvement was present in all cases and frequently accompanied by subcutaneous emphysema. The posterior epidural space was involved in most cases. Among cases in which spinal levels were specified, epidural air most commonly accumulated in the cervical and upper thoracic regions, with only a few cases identifying air in the lumbar region.^13,45 In many cases, including our patient, air traversed through the neural foramina, consistent with the pathophysiological cascade described by the Macklin effect. The Macklin effect involves alveolar rupture secondary to increased intrathoracic pressure, with air dissecting along the bronchovascular sheaths into the mediastinum.^5,6 From there, air may follow fascial planes in the neck and enter the epidural space through the neural foramina,^7,8 particularly favoring the posterior spinal canal, which offers less anatomical resistance than the anterior compartment due to its looser areolar tissue and lack of dense vascular structures.⁹

Management was conservative in 37 of 38 cases (97%), consisting of oxygen therapy, bronchodilators, analgesics, and observation. One patient who presented with an acute exacerbation of ulcerative colitis and was found to have pneumorrhachis on preoperative imaging underwent a colectomy.³⁹ No adolescents underwent surgical decompression or laminectomy. Whereas most adolescent cases of spontaneous pneumorrhachis followed a benign clinical course, 2 patients experienced fatal outcomes. Showkat et al. reported on a patient with a history of B-cell acute lymphoblastic leukemia (ALL) who developed respiratory arrest resulting in death.⁴³ Sriganesh et al. described a patient with underlying neurological pathology awaiting endovascular intervention who developed acute respiratory distress syndrome, leading to rapid deterioration and death.¹⁸ In both cases, death was attributed to complications of underlying disease rather than to pneumorrhachis. The remaining patients (36 cases, 95%) recovered fully without neurological sequelae after conservative management. While only 8 studies reported hospital length of stay, the duration ranged from 2 to 9 days. Length of stay appeared to correlate with underlying etiology and comorbidities, with most patients discharged within 5 days.

Few studies reported follow-up intervals after discharge or specified whether repeat imaging was obtained. One study described ­follow-up at 6 months, which demonstrated no abnormalities.¹⁹ For the other studies that disclosed interval imaging, follow-up imaging was typically performed within 1–2 months and consistently demonstrated complete radiographic resolution in clinically stable patients. Reported modalities included CT at 2 months and chest radiography or ultrasound at 1 month.^24,28,36,39

Based on the findings from our systematic review, we propose a clinical framework to guide the evaluation and management of spontaneous pneumorrhachis in the adolescent population (Fig. 4). In adolescents presenting with neck or chest crepitus, swelling, or respiratory symptoms following forceful vomiting, coughing, or infection, initial imaging with chest radiography and CT should be obtained to assess for pneumomediastinum and pneumorrhachis. If air is identified in the spinal canal, a thorough neurological examination is indicated and neurosurgical consultation is warranted. In patients with focal neurological deficits such as motor weakness, paresthesia, or hyperreflexia, MRI should be obtained to evaluate for cord compression, infection, or other contributory structural pathology. In neurologically intact patients, particularly when air is localized to the posterior epidural space, conservative management is appropriate. Supportive care with supplemental oxygen, analgesics, antiemetics, and close observation is typically sufficient. Patients should be monitored for red-flag symptoms including worsening neck or facial swelling, progressive crepitus, or the emergence of any neurological signs. Repeat imaging should be considered in cases of clinical worsening or atypical radiographic features. Concerning new radiographic features such as anterior epidural air or extensive foraminal tracking should prompt neurosurgical reevaluation. Patients who remain clinically stable may be discharged on symptom improvement, with outpatient MRI and neurosurgical follow-up in 4–6 weeks to confirm pneumorrhachis resolution, consistent with the timelines reported in the literature.

FIG. 4.

Proposed clinical management algorithm for spontaneous pneumorrhachis in adolescents.

While spontaneous pneumorrhachis generally follows a benign course, its alarming radiographic appearance and rare but serious complications underscore the need for a systematic approach guided by symptomology and imaging in emergency settings. Although developed for adolescent cases, our pneumorrhachis management framework may extend to adult patients, in whom spontaneous pneumorrhachis similarly demonstrates a benign natural history with favorable outcomes under conservative treatment.¹

Lessons

Spontaneous pneumorrhachis is a rare but increasingly recognized entity, particularly in adolescents presenting with forceful vomiting, respiratory infections, or other causes of elevated intrathoracic pressure. Although its radiographic appearance can raise significant concern, the condition is typically benign and self-limiting, with conservative management proving effective in nearly all reported cases. However, vigilance should remain high as rare cases have resulted in neurological compromise or death, underscoring the need for careful clinical assessment. Our systematic review and resulting management framework offer a structured, stepwise approach to evaluation and management. Clinical features such as crepitus, neck/facial swelling, subcutaneous emphysema, or chest symptoms should raise early suspicion and prompt appropriate imaging to identify air in the mediastinum and/or epidural space. Patients without neurological deficits can be safely managed expectantly. Those with neurological deficits should receive urgent MRI and neurosurgical evaluation to rule out cord compression, infection, or other complications. This case adds to the limited body of literature on adolescent spontaneous pneumorrhachis and proposes guidelines to aid in triage and management, emphasizing the role of symptomatology, air distribution, and multidisciplinary evaluation in guiding decision-making.

Disclosures

The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

Author Contributions

Conception and design: all authors. Acquisition of data: Paul. Analysis and interpretation of data: all authors. Drafting the article: Paul, Mietus, Honzel. Critically revising the article: all authors. Reviewed submitted version of manuscript: Paul, Mietus, Honzel. Approved the final version of the manuscript on behalf of all authors: Gutting. Statistical analysis: Paul. Administrative/technical/material support: Gutting. Study supervision: Mietus.

Correspondence

Stephen Gutting: University of Massachusetts Chan Medical School, UMass Memorial Health, Worcester, MA. stephen.gutting2@umassmemorial.org.