Patient-Reported Experiences With Median Arcuate Ligament Syndrome: Resilience and Resources Required

Abstract

Objective:

Median arcuate ligament syndrome (MALS) is a vascular condition that is treated surgically. MALS is a diagnosis of exclusion and therefore often requires that pediatric patients undergo an exhaustive number of tests and potential experiences with misdiagnosis before receiving an accurate diagnosis and treatment. The purpose of the current mixed-methods study was to assess the pediatric patient- and parent-reported experience of (a) coping with and managing symptoms associated with MALS and (b) the diagnostic, treatment, and recovery process for pediatric MALS.

Methods:

Nine postsurgical pediatric patients (M age = 20.11 ± 3.59; all <18 at time of surgery) and 6 parents participated in semistructured focus groups and completed self-report questionnaires about their experiences and current functioning.

Results:

Four themes emerged: (a) the impact of MALS (physical and psychosocial), (b) uncertainties and feeling misunderstood, (c) extensive medical tests, and (d) mental health impacts, with an additional three themes related to specific querying (i.e., appreciation, recommendations, and interdisciplinary treatment team). With the exception of variable long-term physical impacts, patients and their parents reported fairly consistent experiences, and the content of these themes was consistent with self-reported quantitative data.

Conclusions:

The current study highlights the resilience and resources seemingly required for effective diagnosis and intervention for pediatric MALS. The need for increased knowledge about MALS, the inclusion of an interdisciplinary treatment team, and long-term follow-up for pediatric patients with MALS are implicated.

Chronic abdominal pain (CAP) is a common medical condition that can significantly impact an individual’s daily life and long-term well-being (Youssef et al., 2006). A possible cause of CAP for some pediatric patients is median arcuate ligament syndrome (MALS), a vascular compression syndrome occurring when the celiac artery is compressed by the median arcuate ligamen (Mak et al., 2016). Pediatric patients with MALS exhibit symptoms of postprandial epigastric pain, nausea, diarrhea, and weight loss, but the pathophysiological mechanisms of their pain are poorly understood (Duffy et al., 2009; Skelly et al., 2018). MALS is also associated with significant impacts on physical and psychosocial quality of life (QOL; Joyce et al., 2014; Mak et al., 2013, 2016).

As MALS is a diagnosis of exclusion and its symptom profiles are similar to other causes of pediatric CAP (Mak et al., 2016), patients must undergo an extensive series of appointments and tests to rule out other etiologies of CAP prior to receiving a diagnosis of MALS (Stiles-Shields et al., 2018). Even after diagnosis, surgeons might call for repeat testing, demonstrating how the ambiguity in the pathophysiologic mechanism of pain in patients with MALS complicates the diagnostic process. Once the medical team feels confident that the results are consistent with a diagnosis of MALS, they may approach the family with surgery as a possible means of treatment. However, the mechanisms for the pain associated with MALS are unclear, and the medical team therefore emphasizes that a decision to engage in surgical intervention should be done with care (Stiles-Shields et al., 2018). Indeed, surgery improves pain for most, but not all patients (Mak et al., 2013). The multipart diagnostic process, the gaps in understanding the mechanisms of pain, and the psychosocial comorbidities are possible contributions to MALS being frequently overlooked as a diagnosis.

While the majority of pediatric patients who undergo surgery report improvement in their overall QOL, 30% continue to suffer from CAP after surgery and remain at risk for long-term impairments (Mak et al., 2013; Stiles-Shields et al., 2018). Furthermore, around half of all presurgical pediatric patients meet criteria for at least one psychiatric disorder (e.g., anxiety, mood disorder), with no evidence to suggest that surgery impacts comorbid diagnoses (Mak et al., 2016; Stiles-Shields et al., 2018). Responsively, some institutions are adopting an interdisciplinary treatment team approach to MALS, which includes a general pediatric surgeon, vascular surgeon, psychologist, and pain specialist (Stiles-Shields et al., 2018). While this interdisciplinary approach is a data-informed response to the multifaceted needs of pediatric patients with MALS, it also further adds to the treatment burden for these patients and their families (e.g., additional appointments and assessments). To the best of our knowledge, no reports on the patient and parent perspective of seeking diagnosis and treatment for MALS exist. Such research could guide the interventions for this unique population, as well as provide more information for the providers who may encounter pediatric patients with MALS.

There is a growing body of research for the characterization and treatment of MALS in the pediatric population. However, large gaps remain in the understanding of the diagnostic, treatment, and recovery process of pediatric patients with MALS. Therefore, the purpose of the current mixed-methods study was to characterize the patient- and parent-reported needs for coping with and managing symptoms associated with MALS and to explore the impact of the diagnosis and surgical process. While this study was exploratory in nature, we suspected based on clinical experience that multiple adversities would be identified, as well as potential opportunities for improvement for providers.

Method

Participants

In compliance with the university institutional review board approval, informed consent was obtained from all participants. Participants meeting the following criteria were eligible to participate: (a) completed surgical release of the median arcuate ligament at Comer Children’s Hospital and (b) were under the age of 18 at the time of surgery. Parents (or legal guardians) of pediatric patients who had completed surgery were also invited to participate. Exclusion criteria included (a) difficulty reading, speaking, or understanding English and (b) a diagnosis for which participation in this study was inappropriate (e.g., active psychosis). Focus group participants were compensated for their time with a $25 gift card.

Procedure

In-person sessions were conducted in the Department of Psychiatry and Behavioral Sciences at the University of Chicago. To promote the inclusion of participants who may live far from the medical center, videoconference sessions were also conducted through the institution’s secure WebEx conferencing platform. All focus group sessions were an hour in length and were conducted by a licensed clinical psychologist (PhD). All groups were asked a standard set of questions about their needs and experiences (see online supplemental Table S1), with the option to follow up about specific feedback. All sessions were audio recorded; audio files were kept in a secure, password-protected electronic folder and used to generate direct transcriptions. Following completion of the focus groups, participants completed self-report questionnaires.

Measures

All self-report assessments were administered and managed via the REDCap (Research Electronic Data Capture) electronic data capture tools (Harris et al., 2009).

Psychological Symptoms

The Kessler Psychological Distress Scale (K10) is a 10-item self-report questionnaire designed and validated to measure distress in youth and adults (Kessler et al., 2002). Scores range from 10 to 50, with scores categorized as nonclinical (<20), mild (20–24), moderate (25–29), and severe (30+). The K10 was administered to surgical patients only and was included as distress is frequently endorsed by patients with MALS.

The Pediatric Quality of Life Inventory (PedsQL; child, parent, and adult versions) measures health-related QOL, assessing physical, emotional, social, and school/work functioning (Varni et al., 2001). Higher scores indicate higher QOL. The PedsQL was administered to all participants due to previous findings associating MALS with impacts to QOL (e.g., Joyce et al., 2014).

Medical Care

The Direct and Indirect Medical Care Impact of MALS Form was created for a larger study and assesses the frequency of medical care usage, travel, and missed days from work/school 1 year prior to surgery and since surgery. Service utilization totals were averaged for patients and parents who participated as dyads.

Data Analysis

A thematic analysis was conducted to analyze qualitative data from the focus groups. An inductive approach was used, such that coding was driven by the data, rather than existing theory (Patton, 1990). The second author, who did not lead any focus groups, conducted the initial coding and organized the codes into themes and subthemes. Following this, she confirmed themes and subthemes with the lead author (who conducted the majority of focus group sessions), and adjustments and consolidations of themes and subthemes were made accordingly. Focus groups were conducted until data saturation was reached according to consensus. Quantitative data collected via self-reported questionnaires were examined using descriptive analyses.

Results

Participants

All pediatric patients who underwent surgery at our institution and had consented for potential research contact were invited to participate (n = 66); 37 patients and/or parents responded to contact. Reasons for nonparticipation included (a) “no show” to a scheduled focus group session, (b) indicating that schedule was too busy to participate, (c) initial interest but eventual lack of response to study team, and (d) interference of pain. There was no evidence to suggest differences in the demographic characteristics or presurgical self-reported pain level of those who did not participate (ps ≥ .1).

A total of eight focus group sessions were conducted with 15 pediatric patients and parents, with the number of groups determined by participant availability. The sample consisted of nine postsurgical pediatric patients (ages 15–28, M = 20.11 ± 3.59, all < 18 at time of surgery; 100% female) and six parents (83.3% female). Four parent–child dyads participated; five pediatric patients and two parents participated independently. Table 1 displays the sample demographic and psychosocial characteristics.

Table 1.

Focus Group Demographic and Psychosocial Characteristics

Variable	Patient (n = 9)	Parent (n = 6)
Age, M (SD; range)	20.11 (3.59; 15–28)	—
Sex, n (%)
Male	—	1 (16.7)
Female	9 (100)	5 (83.3)
Race/ethnicity, n (%)
Caucasian	9 (100)	6 (100)
Hispanic/Latinx	1 (11.1)	—
Highest level of education, n (%)
Some high school	1 (11.1)	—
High school	4 (44.4)	1 (16.7)
College degree	4 (44.4)	4 (66.7)
Graduate degree	—	1 (16.7)
Currently employed, n (%)	5 (55.6)	4 (66.7)
K10, M (SD; range)	20.00 (5.9; 10–31)	—
PedsQL, M (SD; range)
Physical	62.50 (33.95; 12.5–100)	80.21 (30.02; 28.13–100)
Emotional	67.78 (18.89; 40–100)	76.67 (19.92; 50–100)
Social	81.67 (19.53; 40–100)	80.83 (24.17; 50–100)
School/work	62.78 (20.93; 40–100)	86.00 (12.94; 75–100)
Psychosocial	70.74 (17.89; 41.67–100)	85.00 (13.28; 65–100)
Total	67.87 (21.50; 31.25–100)	86.96 (14.44; 63.04–100)

Note. All former patients were <18 years of age at the time of their surgery. Parents were not asked to report their age, personal distress (K10), or their child’s current pain intensity. K10 = Kessler Psychological Distress Scale; PedsQL = Pediatric Quality of Life.

Themes

Four main themes emerged from pediatric patients and their parents. Each theme is detailed below and in online supplemental Table S2.

The Impact of MALS

The most frequently endorsed theme, mentioned by all participants, focused on the impact of MALS. These impacts were described by participants in terms of physical presurgical and postsurgical experiences.

Presurgical Physical Impacts: Pain, Disruptions.

The majority of impacts described by pediatric patients and their parents focused on the physical symptoms associated with MALS. Five described the symptoms as debilitating, with one pediatric patient (22 years old) articulating,

It was so bad … the only time I would sleep [wa]s if I was passed out from pain. I couldn’t sleep normally because I was constantly nauseous, constantly in pain. I used to not be able to stand long enough to take a shower. It was bad. I would shower maybe once a month, if I could. And even then, I almost passed out in the shower a handful of times.

Parents corroborated such reports, with one parent detailing,

She pretty much went to school and came home and crawled into bed. It hurt every time she ate and every time she tried to fall asleep. She would wake me up in the middle of the night—there were times she was afraid she was having a heart attack. She didn’t quite know what was happening; she just knew something had changed.

The disruption to typical routines and functioning due to the physical symptoms was also noted. Parents described changes in school attendance and mood in their children (e.g., “My daughter was missing two to three days of school [per week] … she fell into a depression”). However, multiple parents also reported that the impacts extended from the pediatric patient to the entire family (e.g., “There is a point where it changed our lives dramatically—and never to return to the way we were, any of us, prior to that whole experience.”).

Postsurgical Physical Impacts: Hospital Difficulties and Fears of Reoccurrence.

Participants described physical experiences following surgery that had both short- and long-term impacts. In the short term, seven detailed postsurgical pain that was resolved by the time of discharge (e.g., “It was quite painful. When I was discharged, I was relatively comfortable. I don’t remember feeling like I needed to stay there longer.” [Patient, 20]). Yet, five patients and two parents noted difficulties during the hospitalization, such as inflammation and discomfort from the intravenous (IV) therapy; one patient (21) noted long-term impact from her IV: “My elbow is still whacky. It got totally messed up with the IV being in there for too long.” Regarding long-term impacts, there was a highly variable experience described by the pediatric patients and their parents. Five patients and three parents described resuming normal functioning and activities (e.g., “I went from not being able to do anything at all to being able to get back into high school—and I came back swinging.” [Patient, 22]). Yet, four patients and two parents detailed lasting physical effects, such as difficulties with cardiovascular activities:

Obviously for having it compressed for so long, I still do experience some issues … I’m not very good when it comes to cardio things and I can’t do a whole lot when it comes to physical activity and stuff like that. I’m okay if I’m just walking but I can’t do too much more than that. (Patient, 22)

Continued abdominal pain and fears of reoccurrence were also endorsed, with participants describing a recognition of pain specific to MALS. For example, a parent stated,

[She] is occasionally having artery pain so— [we’re] fearful of possible recompression. It’s nowhere like it was—it’s not as often as it was. I remember telling [Provider] this— [she] would come out and say, “My artery hurts.” It sounds funny because you’d usually say, “My belly hurts,” but she’ll know exactly where the location is.

This variable experience of long-term impacts was also reflected in the extreme variability reported on the PedsQL, particularly for physical functioning (self-reported range: 12.5–100; parent-reported range: 28.13–100; see Table 1).

Uncertainties and Feeling Misunderstood

Uncertainty Prior to Diagnosis.

All participants also endorsed difficulties with the uncertainties surrounding the patients’ symptoms prior to the diagnosis of MALS. One parent described feelings of helplessness while being unable to reassure her child without a diagnosis:

My daughter … had the fear that she was going to die. “What is this? It won’t go away. I’m getting no relief. I’m going to die.” And truthfully, I couldn’t tell her that she wasn’t … being that I didn’t know what it was.

Given the consistent depictions of distress surrounding the experience of having MALS symptoms without a diagnosis, nearly all articulated feelings of relief upon receiving a diagnosis that felt congruent with their symptom experience. One patient (21) stated,

Having that reassurance that I knew something was wrong with my body … like, we know our bodies and we’re trying to tell you what’s going on with them, so to be listened to and told that there was something structural that was really affecting my digestive system … it mentally [gave] me so much relief.

Feeling Misunderstood by Medical Providers.

Relating to the uncertainties prior to diagnosis, five patients and one parent also described varying levels of frustration in response to being misunderstood and/or misdiagnosed by treatment providers. Patients and parents both endorsed experiences with inferences that psychological symptoms were the cause of the pain. For example: “It was really hard for me to be sick for so many years and have all of my doctors telling me that the pain was in my head, that I was making it up, that I was seeking attention” (Patient, 21). A parent also noted: “Basically he only did one test—had her jump up and down, and said, ‘It’s in your head. It’s stress, girl.’ She brought in her schoolwork … he just read that as, she’s a stressed high schooler and it’s stress or anxiety.” Multiple patients also reported experiences with providers suggesting that they had eating disorders, due to the symptoms of weight loss and an avoidance response to a pain trigger (i.e., food; “It was just crazy hearing doctors question if I had an eating disorder, rather than look further into it.” [Patient, 28]). Even following diagnosis and treatment, many participants reported continued difficulties with providers (“It’s crazy that not many know about MALS. I still go to doctors now … and it’s just funny when they are like, ‘What is MALS? I’ve never heard of that.’” [Patient, 28]). Indeed, multiple patients and parents described themselves as educating their providers about MALS.

Parent Advocacy.

In line with feelings of uncertainty and misunderstandings with providers, a subtheme emerged around the need for parents to advocate for their children’s care. One patient (22) articulated,

[My mother] was trying to do her own research since none of the doctors had been able to help us. I know that was extremely taxing for her because every waking moment that she wasn’t working, she was trying to figure out what was wrong with her youngest child.

A parent also described this work as a burden, noting, “The mental scenario started to get heavier and heavier because we were like, ‘Why do we have to meet all of these people?’” Many patients said that they believed they would not have received an accurate diagnosis or treatment without their parents’ advocacy on their behalf (e.g., “It still took a lot of advocating for myself and my family advocating for me to actually get the help that I needed.” [Patient, 21]). This advocacy was also described as a testament to the parents’ belief that the symptoms that the pediatric patients described were genuine (e.g., “My mom really had to fight for who I was as a person because everyone wanted to tell her that this was all my ploy to get extra attention.” [Patient, 21]).

Medical Tests

As MALS is a diagnosis of exclusion, all participants also described undergoing an exhaustive number of medical tests. Four patients attributed medical testing as a disruption to their school schedule. Many also described the burden that the testing placed upon their families in terms of disrupting their schedules (e.g., “She ran so many tests … it was ridiculous. She was missing school like crazy.” [Parent]) and in accessing care (e.g., “There isn’t a permanent [gastroenterologist] in [our] entire state, so things were really hard.” [Patient, 20]). Most notably, multiple participants described how the symptoms associated with MALS made these tests more difficult. One pediatric patient (22) stated,

They had me go through a bunch of other horrifying, horrifying tests that were extremely traumatic. The one I remember the most is the barium. I had to drink the barium and keep it down for two hours. With MALS, I was super nauseous all the time and I couldn’t eat or drink anything—I was like 90 pounds—so having to drink so much barium was horrifying, and I couldn’t do it so they ended up shoving a tube down my throat forcefully.

Another pediatric patient (22) went on to describe the experience of continuing to have medical tests performed despite her providers’ suspicions that she had MALS:

They said they found it and knew what the problem was most likely, but they still had me go through a bunch of tests to make sure that it wasn’t MALS, which to a point I do understand, but it was so much testing in such a short period of time, and so much of it was extremely traumatic for someone like me who can’t eat or drink anything.

In the year prior to surgery, the majority of participants reported multiple visits to their primary care provider (8.00 ± 6.10 visits), medical specialists (6.11 ± 4.12), and the emergency department (4.17 ± 3.06). Table 2 displays service utilization prior to and following surgery.

Table 2.

Service Utilization Prior to and Following Surgery

Variable	One year prior to surgery		Postsurgery
	n (%)	Visits, M (SD; range)	n (%)	Visits, M (SD; range)
Primary care provider	8 (72.7)	8.00 (6.10; 1–20)	6 (54.5)	16.17 (21.89; 3–60)
Medical specialist	9 (81.8)	6.11 (4.12; 3–15)	4 (36.4)	19.25 (16.50; 5–40)
School doctor/nurse	1 (9.1)	30	1 (9.1)	6
Psychiatrist	2 (18.2)	1.50 (.71; 1–2)	4 (36.4)	17.50 (28.40; 2–60)
Psychologist	3 (27.3)	2.00 (1.73; 1–4)	6 (54.5)	96.17 (98.23; 2–260)
Social worker	—	—	1 (9.1)	25
Physical therapist	1 (9.1)	2	2 (18.2)	85.00 (13.28; 65–100)
Dietitian	3 (27.3)	2.67 (2.08; 1–5)	—	—
Other specialty	1 (9.1)	6	—	—
Emergency department	6 (54.5)	4.17 (3.06; 2–10)	3 (27.3)	6.00 (2.65; 3–8)
Hospitalization (days)	3 (27.3)	7.67 (7.23; 3–16)	3 (27.3)	2.00 (1.73; 1–4)

Note Former pediatric patients and parents who completed focus groups as dyads had their reported totals averaged, resulting in a sample size of 11 for service utilization. Postsurgical service utilization was not time capped (i.e., participants may be reporting about service utilization for multiple years since their surgery).

Mental Health

The majority of participants described mental health conditions and/or symptom experiences throughout their MALS diagnostic and treatment experience. Despite many noting concern that treatment providers might conflate their symptoms from MALS with a mental health condition, many described experiencing symptoms of depression and anxiety prior to diagnosis. One patient (22) stated,

My primary care physician thought that it was depression. I was definitely depressed, but it wasn’t what was causing the problem—it was caused by the problem. The doctors didn’t believe me when I said that was the case; that I was perfectly fine before the pain.

However, nearly all pediatric patients endorsed some experience with mental health symptoms following surgery. One pediatric patient (18) reported that she received an eating disorder diagnosis after her MALS symptoms had resolved, explaining that “I wouldn’t eat from the MALS and after surgery I just never ate … I didn’t realize that what I am doing is really bad and I needed help.” The reported postsurgical experience with mental health conditions and/or symptoms was corroborated with former pediatric patients’ responses to the K10. Indeed, the average score on the K10 was consistent with being likely to have emotional distress/meet criteria for a psychiatric disorder (Kessler et al., 2003; see Table 1). Further, mental health service use increased following surgery (see Table 2).

Queried Themes

Three themes emerged that were determined to be specifically queried based upon the interests of the study team: appreciation, recommendations, and interdisciplinary treatment team.

Appreciation.

In querying what went well during the diagnostic and treatment process, positive experiences with providers were described (e.g., “I would have to say that we were blessed with a fantastic pediatrician who believed [her] because we definitely experienced doctors who did not believe her.” [Parent]). A patient (18) stated,

I honestly think we would have been 10 times worse if we had a different [physician assistant] that we didn’t connect with. The connection that he went out of his way to build with us made all the difference in the world.

Clear and open communication was also noted as appreciated by patients and their parents. One parent described,

She was also experiencing a lot of dizziness and I remember them saying they were not sure they will be able to help her with that … I like the fact that they were very honest about that. There were no promises of, “We’re going to fix everything. You’re gonna be great.”

Recommendations.

In querying recommendations for improvement, education was most frequently cited. Participants expressed a desire for medical communities to become more aware of MALS as a condition (e.g., “Educate … every vascular doctor, GI doctor, and every pediatrician … they need to know about MALS.” [Parent]). Multiple participants went on to explain that they hoped this education would limit (a) delays to diagnosis, (b) the number of medical providers who need to be seen, and (c) the amount of medical testing. For example, a patient (22) said,

The other reason it was so frustrating for me is because there were so many invasive tests that I probably didn’t truly need … some of them I understand, like checking the blood flow with the IV fluid. I just don’t know how many of the other tests were strictly necessary. I would like it if for other people, so they don’t have to suffer through some of the traumatizing experiences, if maybe there is a way to cut down on how many of those additional exams are actually needed.

Finally, multiple participants recommended increasing access to education about MALS for future patients. Suggestions ranged from connecting potential patients to postoperative families (e.g., “Just having a community of people who understand what you have been through would be helpful.” [Patient, 21]) to providing information about short- and long-term impacts of MALS (e.g., “I think it may be a good idea if there was a way to put together a packet or booklet about what could potentially happen due to having MALS for along time.” [Patient, 22]).

Interdisciplinary Treatment Team.

Participants were also queried about their reaction to being treated by a team. While some noted initial hesitation and/or worry (e.g., “A lot of people have told me that the pain is in my head, so I was nervous to see psych a bit because I was worried that was going to happen again.” [Patient, 20]), all participants described ultimately appreciating the interdisciplinary approach (e.g., “It was good to know they wanted to check every box before I actually did [the surgery].” [Patient, 18]). Multiple participants also referenced that the inclusion of a psychologist in pre- and postsurgical evaluations was of particular benefit:

I also think the psychology part is very important. Like, even after the surgery, my anxiety just got worse, so it was better to know earlier that I do have this. Who knows how I would have been if I didn’t have to go to the psychology evaluation. I probably would have never gone to see a psychologist. It’s helpful because some people may just think they’re stressed about daily stuff and not realize it is something more severe. (Patient, 19)

Another patient (21) detailed, “I think having the psychologist is really important because it is definitely a mindf*** to have pain when you eat food, which you need to survive.”

Discussion

The current mixed-methods study aimed to characterize the patient and parent experience across the diagnostic, treatment, and recovery process for pediatric MALS. With the exception of variable long-term physical impacts, pediatric patients and their parents reported fairly consistent experiences. Namely, themes regarding (a) the impact of MALS, (b) uncertainties and feeling misunderstood, (c) medical tests, and (d) mental health emerged, with an additional three themes related to specific querying (i.e., appreciation, recommendations, and interdisciplinary treatment team). The content of these themes was consistent with self-reported health-related QOL, distress, and pre- and postsurgical service utilization.

As anticipated, multiple adversities were described—from physical symptoms to extensive medical testing. That this sample overcame such adversities to receive a diagnosis and surgical intervention speaks both to their resiliencies and resources. The majority of pediatric patients and parents depicted remarkable persistence that lasted up to several years, including symptom management and scheduling appointments with multiple providers to pursue a diagnosis that was concordant with the symptom experience. Many also reported traveling across the country to seek specialty care and diagnostic evaluations. As such, the resiliency of these families was also bolstered by their resources. Indeed, they anecdotally reported having the insurance coverage and financial security to schedule multiple specialty appointments, travel for care, and the ability to miss school and work. These findings highlight the many strengths of the families of pediatric patients with MALS.

These findings also highlight potential health disparities. Indeed, this sample was composed of families of only female patients and was almost entirely non-Hispanic/Latinx Caucasian. This demographic makeup is consistent with previous research on pediatric MALS (Joyce et al., 2014; Mak et al., 2016; Stiles-Shields et al., 2018). Racial, ethnic, and socioeconomic disparities have been identified across multiple acute and chronic pediatric pain conditions, such that youth from minoritized populations are less likely to receive appropriate diagnosis, medication, and treatment (Goyal et al., 2015; Johnson et al., 2013; Mossey, 2011). This body of literature, combined with the resilience and resources described by the current sample, highlights a likely health disparity: There may be many cases of pediatric MALS that are going undiagnosed and/or misdiagnosed, particularly within minoritized populations.

To address such possible health disparities and to improve the diagnostic and treatment experience for future MALS patients, multiple targets were identified. First, increasing knowledge about MALS as a condition is imperative. Indeed, providers cannot screen for a condition that is unknown to them. Disseminating information about the typical symptom presentation of MALS to both specialty providers (e.g., gastrointestinal) and primary care providers is key. Primary care is included in this list of providers, as nearly all children—including those who are likely to be impacted by health disparities—attend at least one well-child visit annually (Bloom et al., 2011). Second, the patient experience is benefited by an interdisciplinary treatment team approach. While MALS is treated surgically (Mak et al., 2016), nearly all participants reported an impact to their mental health. These findings are consistent with Mishel’s (1988) uncertainty in illness theory, as individuals who experience illness-related uncertainty often have high levels of psychological distress. As such, individuals with chronic illnesses benefit from increased access to health and mental health care providers, who acknowledge this uncertainty and provide support in accepting and effectively coping with sources of uncertainty (Mishel, 1990). Connecting pediatric patients to interdisciplinary providers, including pediatric psychologists, is likely to best support their variable and complex needs with appropriate screenings and treatment referrals. Finally, long-term, interdisciplinary follow-up for pediatric patients with MALS is likely to benefit patients with less optimal postsurgical outcomes. The data collected with this sample are consistent with the pediatric MALS literature to indicate variable long-term outcomes following surgery (Joyce et al., 2014; Mak et al., 2013; Stiles-Shields et al., 2018), supporting that patients likely need ongoing assessments to ascertain whether additional services or referrals are necessary.

Given our role in medical systems, pediatric psychologists are ideally suited to support these patient-driven targets. First, pediatric psychologists may support earlier identification of MALS by disseminating the patient experience with MALS to colleagues who are likely to treat children with CAP. Indeed, beyond academic dissemination (e.g., Grand Rounds, professional conferences), pediatric psychologists may promote conversations about MALS with their medical colleagues and encourage integration of multiple disciplines in screening and treating children with CAP (e.g., Stiles-Shields et al., 2018). As a result, more timely diagnosis might occur, which could potentially reduce the burden described by former patients with MALS and also minimize the medicalization of CAP. Second, pediatric psychologists are trained to provide behavioral health support to pediatric patients and their families and can therefore assess, monitor, and provide intervention as needed to patients with MALS across the diagnostic and treatment course, including follow-up.

The current study should be considered in light of specific limitations. As previously noted, the sample was highly homogeneous (i.e., female, non-Hispanic/Latinx Caucasian). While possible reasons for this have already been discussed, future research should target more diversity in terms of sex, race/ethnicity, and socioeconomic status. Second, this sample was recruited from a single institution, perhaps limiting generalizability to potential regional constraints/differences and intervention with an interdisciplinary team. Third, participants completed their self-report measures after participating in the focus group, potentially influencing their responses. Fourth, an objective measure of time to diagnosis was not able to be obtained; this metric should be collected in future research with patients with MALS, as the current study would suggest increased time would be associated with more psychological distress. Fifth, this study examined the experience of pediatric patients who underwent surgical intervention. Future research should include comparisons between surgical and nonsurgical patients with MALS. Finally, while all patients and their parents who had previously participated in a prospective chart review study for pediatric MALS were invited to participate, it is possible that there were self-selection effects such that certain patient experiences were not reflected by the current sample. Ongoing research incorporating patient- and parent-reported experiences with pediatric MALS is warranted.

The current mixed-methods study identified recurrent themes for pediatric patients and their families that highlight specific future directions. Namely, information about MALS as a condition and its symptom cluster needs to be disseminated to promote earlier screening and identification, particularly for minoritized populations. Pediatric patients with MALS are also likely to benefit from an interdisciplinary treatment team approach and to have long-term follow-up following surgical intervention. Future research with more diverse patient groups with MALS is needed, as well as the ongoing engagement of patients and their families to continue to inform intervention approaches.

Implications for Impact Statement.

Median arcuate ligament syndrome (MALS) is a frequently overlooked cause of chronic abdominal pain. As a diagnosis of exclusion, pediatric patients must undergo exhaustive medical testing to receive care and are often told that their pain does not have a physical etiology. Patients and their families want for providers to become more knowledgeable of MALS and to demonstrate belief in patients’ reported symptom experience.