PSU Volume 67 No 02 AUGUST 2026
Kaposiform Hemangioendothelioma
Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive
vascular tumor that primarily affects infants and young children,
though it can occasionally present in adults. First distinguished as a
separate entity in 1993, KHE occupies an intermediate position in the
spectrum of vascular neoplasms — neither fully benign nor overtly
malignant — and is characterized by its capacity for locally
invasive and infiltrative growth. Its estimated annual incidence is
approximately 0.071 per 100,000 children, though the true prevalence is
likely higher, as small or atypical lesions may be misclassified as
other vascular anomalies. A slight male predominance has been noted in
larger case series.
The underlying cause of KHE remains incompletely understood. Most cases
arise sporadically without an identifiable trigger, though rare reports
describe worsening after trauma, surgery, infection, or vaccination,
suggesting that inflammatory stimuli may aggravate the disease. At the
molecular level, somatic mutations — including activating
variants in the GNA14 gene — have been identified in a subset of
specimens, implicating the MAPK/ERK signaling pathway. Whether these
mutations are causative or secondary is still debated. Dysregulation of
both angiogenesis and lymphangiogenesis are central to KHE's pathology.
Elevated vascular endothelial growth factor-C and its receptor
(VEGFR-3), along with high angiopoietin-2 levels, have been implicated
in driving abnormal vessel formation and contributing to the
consumptive coagulopathy that defines the disease's most dangerous
complication.
On histopathology, KHE is recognized by infiltrating, rounded,
confluent nodules composed of spindle-shaped endothelial cells that
form malformed lymphatic channels and slit-like vascular lumina.
Microthrombi, hemosiderin deposits, and eosinophilic hyaline bodies are
frequently observed. Immunohistochemical staining shows positivity for
vascular markers CD31 and CD34, and lymphatic markers D2-40, VEGFR-3,
and Prox-1, while staining is negative for GLUT-1 — a feature
that distinguishes KHE from infantile hemangioma — and for HHV-8.
KHE exhibits considerable clinical heterogeneity. Lesions are
classified by depth of involvement into three morphological types:
superficial (confined to skin and subcutaneous tissue), mixed
(involving both superficial and deep structures including muscle, bone,
or joint), and deep (no cutaneous involvement, affecting internal
organs, retroperitoneum, mediastinum, or bone). A further clinically
important subgroup is intracavitary KHE — tumors arising
primarily within body cavities such as the thoracic or abdominal
cavity, retroperitoneum, or pelvic space, where the bulk of the lesion
is contained within the cavity and may involve serosal surfaces,
mesentery, or cavity-specific structures. The mixed type is the most
common overall, while deep and intracavitary lesions account for a
smaller proportion of cases but carry the greatest diagnostic challenge
and the most severe clinical burden.
Cutaneous KHE typically presents as erythematous, violaceous, or
darkened patches, plaques, or firm nodules. When associated with the
Kasabach-Merritt phenomenon (KMP), lesions become tense, engorged,
purpuric, hot to the touch, and painful. Deep lesions without skin
involvement may present with nonspecific symptoms such as abdominal
distension, jaundice, respiratory distress, or unexplained
thrombocytopenia and coagulopathy, often resulting in significant
diagnostic delay. Intracavitary lesions in particular may compress
adjacent organs or critical structures as they enlarge, leading to
pleural effusion, pericardial effusion, peritoneal effusion,
obstructive jaundice, gastrointestinal bleeding, or airway compromise.
Approximately 90% of cases become evident within the first year of
life, and about half of cutaneous lesions are detectable at birth.
Unlike infantile hemangioma, KHE does not undergo spontaneous
involution and tends to progress without treatment.
The most feared complication of KHE is the Kasabach-Merritt phenomenon,
which occurs in 42–71% of patients. KMP is defined by profound
thrombocytopenia — with median platelet counts around 21 ×
10?/L at presentation — combined with consumptive coagulopathy
and hypofibrinogenemia. It is critical to understand that KMP does not
occur with infantile hemangioma; it is exclusively associated with KHE
and the related tufted angioma. KMP carries a mortality rate estimated
between 10–30%, and deaths are most commonly attributable to
hemorrhage in vital organs. Intracavitary KHE carries a
disproportionately high KMP burden — studies have documented KMP
rates approaching 80% in this subgroup, compared to roughly 30% in
non-intracavitary cases — a disparity that may reflect the
anatomical microenvironment of body cavities, which lack the soft
tissue buffering present in superficial or extremity lesions,
facilitating early compression of vascular structures, local blood
stasis, and amplification of platelet trapping. The rich chylous pools
and subserosal lymphatic networks within the thoracic and abdominal
cavities may further intensify the local accumulation of coagulant
substances and increase susceptibility to KMP.
Risk factors for KMP include young age at presentation, large tumor
size (particularly greater than 8 cm), and anatomic location —
intrathoracic and retroperitoneal lesions are disproportionately
associated with KMP. Patients with KMP tend to have larger tumors and
present at a younger age than those without.
Beyond KMP, KHE causes significant morbidity through musculoskeletal
involvement. The tumor's infiltrative and destructive growth can erode
bone, invade joints, and alter the mechanical properties of surrounding
connective tissue, leading to decreased range of motion, chronic pain,
joint contractures, and progressive scoliosis — particularly with
thoracic or retroperitoneal lesions. Tumor stimulation, inflammatory
cell infiltration, and fibrin deposition near vertebral structures can
promote local fibrosis, and during skeletal development, contractures
may mechanically pull on bone, eventually driving progressive thoracic
deformity. These musculoskeletal complications are more common in older
children and can persist even after hematologic parameters normalize.
Lymphedema is a recognized long-term sequela, especially with lesions
involving the proximal extremities near lymph node basins.
Intracavitary KHE is additionally associated with a higher burden of
organ dysfunction — including jaundice and abnormal liver
function from bile duct or portal vein compression, gastrointestinal
hemorrhage from intestinal wall infiltration, and structural
deformities such as scoliosis from paraspinal involvement —
complications that are far less common in non-intracavitary disease and
that can in severe cases impair respiratory and circulatory function
and contribute to death.
Diagnosis of KHE requires integration of clinical, laboratory, imaging,
and pathological data. Ultrasound is useful for superficial or small
lesions, typically revealing heterogeneous, ill-defined, hypervascular
masses. However, MRI is the preferred modality and provides the most
comprehensive assessment of disease extent. On MRI, KHE
characteristically demonstrates iso-intensity relative to adjacent
muscle on T1-weighted imaging and heterogeneous hyperintensity on
T2-weighted imaging, with intense, heterogeneous gadolinium
enhancement. Lesions commonly show ill-defined margins, multiplanar
involvement, adjacent fat stranding, and bone or joint changes
including cortical destruction or remodeling. CT can complement MRI in
evaluating bony involvement and is more practical in critically ill
patients. Three morphological patterns have been described:
well-defined solid mass, solid central mass with surrounding
infiltrative regions, and purely infiltrative lesion without a distinct
solid core. Biopsy remains the gold standard for diagnosis and should
be obtained when clinically safe. In patients with classic KMP and a
compatible vascular mass on imaging, histologic confirmation may not
always be required before initiating treatment.
No FDA-approved therapy exists specifically for KHE. Management must be
individualized based on disease severity, the presence or absence of
KMP, lesion location, and patient age. For patients with KMP,
aggressive combination therapy is required — monotherapy is
generally insufficient. The mTOR inhibitor sirolimus, combined with
short-term corticosteroids, has emerged as the preferred first-line
regimen for KHE with KMP. Sirolimus works by inhibiting the
PI3K/AKT/mTOR pathway, which mediates downstream effects of both
VEGF-C/VEGFR3 and angiopoietin-2/Tie-2 signaling, and may additionally
suppress the proliferation of KHE endothelial cells through effects on
autophagy-related pathways. Response rates with sirolimus are high, and
angiopoietin-2 levels have been shown to fall significantly with
treatment.
For patients without KMP, sirolimus monotherapy is effective, and
randomized trial evidence demonstrates that low-dose sirolimus
(targeting trough concentrations of 5–8 ng/mL) is non-inferior to
high-dose sirolimus (10–15 ng/mL) at one year of treatment, with
fewer respiratory, skin, and mucosal adverse events. Low-dose sirolimus
therefore represents a safer long-term option for non-KMP patients
requiring prolonged therapy, though high-dose regimens retain a role
for severe cases and those complicated by KMP. Intracavitary KHE, even
when treated with sirolimus-based regimens, tends to show lower
sustained response rates at 6 and 12 months compared to
non-intracavitary disease, underscoring the need for vigilant systemic
monitoring and active complication management alongside tumor-directed
treatment; outcomes at 24 months, however, appear more comparable
between the two groups.
Vincristine — alone or combined with corticosteroids or
antiplatelet agents such as ticlopidine — remains an alternative
first-line option for KMP, with an overall response rate of
approximately 72%. Corticosteroids alone yield lower sustained response
rates and carry significant long-term side effects. Propranolol and
interferon-alpha have been used with variable and generally less
reliable results; interferon-alpha in particular carries a risk of
serious neurologic complications and is contraindicated in children
under one year of age. Topical sirolimus and tacrolimus ointments have
shown efficacy for superficial lesions, offering a way to avoid
systemic drug exposure in selected patients.
Surgical resection is reserved for cases where complete and safe
excision is achievable, as the infiltrative nature of most KHE makes
curative surgery impractical for the majority. Elective resection
during active KMP is generally discouraged due to the risk of worsening
coagulopathy and hemorrhage. Arterial embolization can serve as an
adjunct in cases with extensive, unresectable lesions or refractory
KMP, providing rapid reduction in tumor blood flow. Platelet
transfusions should be avoided unless the patient is actively bleeding
or being prepared for an invasive procedure.
KHE significantly impairs health-related quality of life in
affected children and their families. Both physical functioning —
particularly in children with activity-limiting musculoskeletal
complications — and psychosocial functioning are measurably
reduced compared to healthy peers. KMP and activity dysfunction are the
strongest risk factors for poor quality of life. Parents of children
with KHE consistently report high levels of worry, emotional burden,
and disruption to daily activities regardless of lesion location or
parental education level.
Complete tumor resolution is uncommon. Residual lesions after treatment
often persist as vascular staining, telangiectasia, soft tissue
fibrosis, or subcutaneous infiltrates. Long-term surveillance is
warranted, as untreated residual KHE can continue to infiltrate
surrounding tissue and cause progressive fibrosis and joint destruction
over time. Early and accurate diagnosis, multidisciplinary management,
and individualized treatment remain the cornerstones of improving
outcomes in this challenging disease.
References:
1- Ryu YJ, Choi YH, Cheon JE, Kim WS, Kim IO, Park JE, Kim YJ. Imaging
findings of Kaposiform Hemangioendothelioma in children. Eur J Radiol.
86:198-205, 2017
2- Schmid I, Klenk AK, Sparber-Sauer M, Koscielniak E, Maxwell R,
Häberle B. Kaposiform hemangioendothelioma in children: a benign
vascular tumor with multiple treatment options. World J Pediatr.
14(4):322-329, 2018
3- Ji Y, Chen S, Li L, Yang K, Xia C, Li L, Yang G, Kong F, Lu G, Liu
X. Kaposiform hemangioendothelioma without cutaneous involvement. J
Cancer Res Clin Oncol. 144(12):2475-2484, 2018
4- Dai S, Yang K, Qiu T, Zhou J, Zhang X, Chen S, Li L, Ji Y.
Health-Related Quality of Life in Children With Kaposiform
Hemangioendothelioma. Front Pediatr. 10:720611, 2022
5- Huo J, Chen S, Li J, Liu C. Retroperitoneal kaposiform
hemangioendothelioma with kasabach-merritt phenomenon in children: A
case report and review of the literature. Front Pediatr. 11:1138689,
2023
6- Zhou J, Lan Y, Qiu T, Zhang Z, Gong X, Zhang X, Yang C, Zhou Z,
Zhang Y, Yang M, Fu J, He C, Peng Q, Hu F, Xia C, Kong F, Chen S, Ji Y.
Efficacy and safety of high-vs low-dose sirolimus in patients with
kaposiform hemangioendothelioma: A randomized clinical trial. J Am Acad
Dermatol. 93(1):124-131, 2025
7- Zhou J, Ji Y. Kaposiform hemangioendothelioma. J Am Acad Dermatol. 12:S0190-9622(26)00396-8, 2026
Accessory Breast Tissue
Accessory breast tissue, also termed ectopic or supernumerary
breast tissue, refers to mammary tissue located anywhere on the body
outside its normal position on the anterior thoracic wall. It is a
congenital anomaly that originates during embryonic development. Around
the sixth week of gestation, paired mammary ridges, commonly called the
"milk lines," appear bilaterally on the ventral surface of the embryo
and extend from the axilla to the inguinal and vulvar region. Under
normal circumstances these ridges regress almost entirely, leaving only
a single pair of buds in the pectoral region that go on to form the
adult breasts. When regression fails or is incomplete at any point
along this line, residual mammary elements persist and may later
develop into accessory breast tissue. Because this represents the
reappearance of a structure typical of more primitive mammals, in which
multiple paired glands run the length of the trunk, the condition is
regarded as atavistic, a reversion to an ancestral pattern. Although
the milk line is the usual site, ectopic mammary elements have
occasionally been described well beyond it, including the neck, back,
face, thigh, and even the sole of the foot.
The spectrum of accessory mammary tissue is conventionally described by
a classification scheme dating from 1915 that divides it into eight
categories according to which components, glandular tissue, nipple, and
areola, are present. The most complete form contains all three elements
and constitutes a fully formed supernumerary breast. Subsequent
categories describe progressively incomplete combinations: glandular
tissue with a nipple but no areola; glandular tissue with an areola but
no nipple; glandular tissue alone, sometimes called mamma aberrata; a
nipple and areola without underlying glandular tissue, known as
pseudomamma; a nipple only, which is the classic supernumerary nipple
or polythelia; an areola only, termed polythelia areolaris; and finally
a patch of hair only, polythelia pilosa. In everyday practice two broad
terms dominate: polythelia, denoting supernumerary nipples generally
without associated breast tissue, and polymastia, denoting accessory
glandular tissue with or without a nipple. The distinction is
clinically meaningful because the presence or absence of true glandular
tissue determines whether the lesion can undergo the same physiological
and pathological changes as a normal breast.
Reported prevalence varies considerably depending on the population
studied and the definitions used, ranging broadly from about 0.22% to
6% of the general population. Polythelia tends to fall within the lower
portion of that range, while accessory glandular tissue is reported in
roughly 0.4% to 6% of women and 1% to 3% of men. Geographic and ethnic
differences exist, with higher frequencies described among Asian and
Japanese women and lower frequencies among those of Caucasian
background. Supernumerary nipples have historically been described as
somewhat more common in males, yet among patients who actually present
for clinical evaluation or excision, the great majority are female and
often young, likely because women more frequently seek correction for
cosmetic or symptomatic reasons. Lesions are usually solitary and
unilateral, though bilateral and multiple lesions occur; when
unilateral, a slight predilection for the right side has traditionally
been noted, although this is not invariable.
By anatomical site, the axilla is by far the most common location for
ectopic breast tissue, followed by other regions of the chest, the
inframammary fold, and the abdomen. The condition is most often
sporadic, but familial clustering is well recognized, including
instances of male-to-male transmission. The proposed inheritance
patterns are heterogeneous, encompassing autosomal dominant
transmission with incomplete penetrance, X-linked dominant, and
autosomal recessive modes, and somatic mutations arising early in
embryonic life may also contribute. Accessory mammary tissue has been
linked to congenital anomalies, most consistently of the renal and
urinary tract, and less frequently to cardiovascular malformations,
kidney tumors, and chromosomal conditions such as trisomy 21. These
associations, however, are inconsistent, and large cohorts composed
mainly of adults frequently identify no accompanying congenital anomaly
at all. Because most congenital anomalies are detected in early
childhood, the apparent rarity of associations in adult series may
partly reflect the age at which patients present. Ultrasonographic
screening of the kidneys and abdomen is sometimes recommended as part
of diagnostic follow-up, particularly in younger patients.
Clinically, accessory breast tissue is frequently asymptomatic and may
be noticed only as a cosmetic concern or a cutaneous protuberance,
often slightly more pigmented than surrounding skin. When functional
glandular tissue is present, it responds to the same hormonal stimuli
as orthotopic breast tissue, so the lesion may enlarge and become
symptomatic at puberty, during pregnancy, or while breastfeeding. Forms
consisting of glandular tissue alone, lacking an external nipple or
areola, are especially prone to going unrecognized until hormonal
stimulation makes them apparent. The characteristic complaint is a soft
axillary mass, commonly a few centimeters in size, accompanied by
cyclic pain coinciding with menstruation, swelling, tenderness, and
fluctuation in volume. Larger lesions may restrict shoulder movement or
cause irritation against clothing, and the cosmetic appearance often
provokes anxiety. In children and adolescents the diagnosis is uncommon
and may be delayed, yet it can occur even before menarche, and a
tender, periodically enlarging axillary mass in this age group should
raise suspicion.
The differential diagnosis is broad and accounts for frequent
misidentification. Accessory breast tissue has been mistaken for
lipoma, lymphadenopathy, hidradenitis suppurativa, sebaceous cyst,
vascular malformation, neurofibroma, lymphoma, and metastatic disease,
among others. Ultrasonography is the preferred first-line imaging
modality, demonstrating hypoechoic, septate, glandular-appearing tissue
analogous to normal breast, sometimes with duct ectasia. Bilateral
imaging is advisable to detect contralateral involvement, which may be
asynchronous. Mammography and magnetic resonance imaging serve as
useful adjuncts when the diagnosis is uncertain or when malignancy or
another neoplastic process must be excluded, and fine-needle aspiration
or core-needle biopsy can provide definitive confirmation.
Histologically, the tissue shows the architecture of normal breast,
with mammary lobules and lactiferous ducts in the dermis, surrounding
connective tissue stroma, and bundles of nipple-type smooth muscle,
often in proximity to cutaneous adnexal glands.
Because it is genuine mammary tissue, the ectopic gland is susceptible
to the full range of benign and malignant breast disease. Reported
benign processes include fibroadenoma, fibrocystic change, ductal
hyperplasia, duct ectasia, lactating adenoma, and intraductal or
intracystic papilloma, the last being distinctly uncommon in this
setting. Malignant transformation, while rare, is documented and
includes ductal carcinoma in situ and invasive carcinoma. Importantly,
tumors arise within glandular tissue rather than from a supernumerary
nipple in isolation; when malignancy or significant proliferative
disease is found in association with a supernumerary nipple, it
typically reflects underlying accessory glandular tissue. This
underscores the principle that wherever ectopic mammary tissue is
identified, the possibility of accompanying breast pathology should be
considered.
Management is largely conservative. Surgical excision is reserved for
symptomatic lesions, persistent cosmetic concern, diagnostic
uncertainty, or suspicion of neoplasm, and prophylactic removal is not
currently recommended. Excision through a small incision placed within
a natural axillary fold is generally safe and effective, with
liposuction reserved for larger lesions; reported complications include
hematoma, seroma, infection, residual tissue, contour irregularity, and
hypertrophic scarring. Operating before pregnancy is often favored
because reoperation rates are lower and patient satisfaction higher.
Overall, accessory breast tissue is a benign, usually innocuous
condition whose chief clinical importance lies in correct recognition,
distinction from other masses, and awareness of its capacity to develop
the same diseases as a normally situated breast.
References:
1- De la Torre M, Lorca-García C, de Tomás E, Berenguer
B. Axillary ectopic breast tissue in the adolescent. Pediatr Surg Int.
38(10):1445-1451, 2022
2- El Malih S, Ezzahi M, Haloua M, Tahiri L, Akammar A, El Bouardi N,
Alami B, Alaoui Lamrani MY, Maaroufi M, Boubbou M. Unusual intracystic
papilloma arising from ectopic axillary breast tissue: Case report.
Radiol Case Rep. 18(10):3414-3420, 2023
3- Class MM, McCoy K, Melin AA, Hafeez F, Abidi N, Krakowski AC.
Bilateral accessory axillary breast tissue in a premenarchal female.
Pediatr Dermatol. 41(4):704-706, 2024
4- Al Assaad M, Vulcain DR, Phan A, Boyraz B, Hoda SA. Polythelia
(Supernumerary Nipple): Clinicopathological Characterization of an
Atavistic Lesion. Int J Surg Pathol. 2025 33(8):1735-1741, 2025
5- Sag S, Sonmez Y, Gungormez EK, Canbaz FA, Gercel G, Yavuzer D, Yasar
E, Thomas DT. Pediatric Breast Pathologies: 5-Year Experience and
Proposal for a Risk-Based Management Algorithm. J Pediatr Adolesc
Gynecol. S1083-3188(26)00309-8, 2026
Endoscopic Functional Luminal Imaging Probe
For most of the history of gastroenterology, the esophagus has been
judged by what the eye could see and what a swallow could tell. An
endoscope reveals the mucosa; a barium column traces the path of liquid
downward; a manometry catheter records the pressures that ripple along
the muscular tube as a patient swallows on cue. Each of these tools
answers a real question, yet each leaves a gap. None of them measures
directly how the esophagus behaves as a mechanical object—how
readily its walls yield to stretch, how wide its narrowest point will
open under load, how the muscle answers when the lumen is filled rather
than when a bolus is swallowed. The functional luminal imaging probe,
almost always abbreviated to FLIP, was built to close that gap, and
over the past decade it has moved from a research curiosity into a
working clinical instrument, including, more recently, in children.
The principle behind FLIP is elegant in its simplicity. A catheter
carrying a cylindrical balloon is passed transorally and positioned
across a region of interest, most often the esophagogastric junction.
Inside the balloon sit sixteen impedance-planimetry sensors that
measure the electrical voltage between neighboring electrodes as the
balloon is filled with a conductive fluid. From those readings the
system reconstructs cross-sectional area at multiple points along the
balloon and pairing that geometry with a built-in pressure sensor
yields the central number of the field: the distensibility index, or
DI, calculated as the minimal cross-sectional area divided by the
intraballoon pressure at a set fill volume. Two versions of the device
exist. EndoFLIP uses a soft balloon for measurement alone, while
EsoFLIP carries a stiffer balloon that can dilate a narrowing while
reporting diameter changes in real time. The technology is performed
during sedated endoscopy, which is part of its appeal—it can be
done in the same sitting as the diagnostic exam, without the awake
catheter placement that manometry requires.
What FLIP can do has expanded well beyond a single measurement. Carlson
and colleagues, working from a cohort of more than seven hundred
subjects studied alongside high-resolution manometry, showed that the
device could be used not just to gauge junction opening but to classify
esophageal motility itself. Their approach, FLIP Panometry, watches how
the esophageal body contracts in response to sustained
distension—an assessment of secondary peristalsis that ordinary
manometry, focused on swallow-triggered primary peristalsis, simply
does not capture. By combining a contractile-response pattern with a
junction-opening category, they built a classification that paralleled
the Chicago Classification used for manometry. Patients with normal
junction opening and a normal contractile response almost always had
normal motility or ineffective motility on manometry, while those with
reduced opening and a weak contractile response overwhelmingly carried
a disorder of junction outflow, most often achalasia. The message was
not that FLIP should replace manometry, but that the two tests
illuminate different facets of esophageal function and can confirm,
complement, or clarify one another, particularly when an initial
manometric impression is inconclusive.
The pediatric story is younger and, in some ways, more revealing.
EndoFLIP was cleared in the United States in 2019 for the esophagus,
pylorus, and anal sphincters in children five years and older, and
off-label use has reached infants as young as ten months. Yet the
central difficulty in children is the absence of normative data.
Benitez and colleagues studied one of the largest pediatric achalasia
cohorts reported, measuring junction distensibility before and
immediately after balloon dilation and comparing the results to
non-achalasia controls. Treatment-naive children had markedly lower
distensibility and smaller junction diameters than controls, and
dilation produced a clear, immediate rise in DI together with improved
symptom scores. But the findings also carried a caution: only half of
treatment-naive children fell below the adult diagnostic threshold of
2.0 mm² per mmHg, meaning that adult cutoffs, applied
uncritically, could misclassify a substantial fraction of symptomatic
children. Pediatric esophagi are not simply smaller adult esophagi, and
the reference ranges borrowed from adults may flatter or mislead.
Two recent reports show how far the pediatric application now reaches
beyond achalasia. In eosinophilic esophagitis, where chronic
inflammation can quietly remodel the esophageal wall into fibrosis and
stricture, Berson and colleagues found that FLIP detected reduced
distensibility even in children who were in clinical and histologic
remission. Their cohort's average DI sat below the threshold often used
to mark abnormal rigidity, and the histologic eosinophil count did not
track with the distensibility number—an argument that biopsy and
symptom assessment alone can miss residual mechanical disease that FLIP
picks up. In a different vein, Hoskins and colleagues turned the probe
toward vascular compression of the esophagus, the kind produced by an
aberrant subclavian artery or a vascular ring. There, FLIP detected
narrowing more often than endoscopy did, sometimes flagging functional
restriction in a normal-looking esophagus, and just as usefully, it
helped exclude meaningful obstruction in children whose symptoms turned
out to stem from reflux or inflammation rather than the vessel. Paired
measurements at the compression site and the lower sphincter showed
convincingly reduced diameter and distensibility where the vessel
pressed.
None of these papers oversells the device. Each returns to the same
chorus of limitations: no standardized pediatric protocol, no validated
reference values indexed to age and size, balloon and catheter
constraints in the smallest patients, cost, and the concentration of
expertise in a handful of centers. FLIP does not diagnose by itself; it
is described, repeatedly, as a complementary or adjunctive tool, most
powerful when its real-time, objective numbers are read alongside
endoscopy, imaging, and manometry. Still, the trajectory is clear. A
technology that lets a clinician feel the esophagus quantitatively, at
the moment of endoscopy, is filling a space the older tools left
empty—and as pediatric normative data accumulate, that quiet rise
seems likely to continue.
References:
1- Benitez AJ, Budhu S, Burger C, Turco R, Ballester L, Shah A, Lynch
K, Fiorino K, Menard-Katcher C, Muir AB, Mamula P. Use of the
functional luminal imaging probe in pediatrics: A comparison study of
patients with achalasia before and after endoscopic dilation and
non-achalasia controls. Neurogastroenterol Motil. 33(12):e14133, 2021
2- Krasaelap A, Lerner DG. Advances in Endoscopic Procedures in Pediatric Patients. Pediatr Clin North Am. 68(6):1221-1235, 2021
3- Carlson DA, Gyawali CP, Khan A, Yadlapati R, Chen J, Chokshi RV,
Clarke JO, Garza JM, Jain AS, Katz P, Konda V, Lynch K, Schnoll-Sussman
FH, Spechler SJ, Vela MF, Prescott JE, Baumann AJ, Donnan EN, Kou W,
Kahrilas PJ, Pandolfino JE. Classifying Esophageal Motility by FLIP
Panometry: A Study of 722 Subjects With Manometry. Am J Gastroenterol.
116(12):2357-2366, 2021
4- Lerner DG, Mencin A, Novak I, Huang C, Ng K, Lirio RA, Khlevner J,
Utterson EC, Harris BR, Pitman RT, Mir S, Gugig R, Walsh CM, Fishman D.
Advances in Pediatric Diagnostic Endoscopy: A State-of-the-Art Review.
JPGN Rep. 3(3):e224, 2022
5- Berson J, Kota A, Levine J. Endoluminal functional lumen imaging
probe: a new modality in the evaluation of esophageal disorders in
children and preliminary use in eosinophilic esophagitis. Front
Pediatr. 13:1581225, 2025
6- Hoskins BJ, Bose P, Pitman RT. Pediatric vascular compression of the
esophagus: Endoluminal functional lumen imaging probe as a complement
to imaging and endoscopy. JPGN Rep. 7(2):192-199, 2026