Invest Clin 65(2): 134 - 142, 2024 https://doi.org/10.54817/IC.v65n2a01

Corresponding author: Jing Hao, Department of Traditional Chinese Medicine, Beijing Children’s Hospital, Capi-

tal Medical University, National Center for Children’s Health, No. 56 Nanlishi Road, West District, Beijing, China.

Tel: 010-59616363. E-mail:bchhaojing@163.com

Surgical complications in children with IgA

vasculitis: clinical analysis of 28 cases.

Jing Mu, Jing Hao, Xiaofang Zhen, Xin Mo, Yan Sheng

Department of Traditional Chinese Medicine, Beijing Children’s Hospital, Capital

Medical University, National Center for Children’s Health, Beijing,China.

Keywords: intussusception; intestinal perforation; Henoch-Schönlein purpura;

enterectomy; enterostomy; D-dimer; C-reactive protein.

Abstract. Intussusception and intestinal perforation are surgical severe

complications in children with IgA vasculitis (IgAV). Early diagnosis and timely

appropriate surgical intervention can reduce damage to the children. We an-

alyzed retrospectively the clinical characteristics, auxiliary examinations, di-

agnosis, treatment, and prognosis of 28 children with IgAV accompanied by

surgical complications (including intussusception in 21 patients and intesti-

nal perforation in seven patients) who were admitted to the Beijing Children’s

Hospital of the Capital Medical University from May 2016 to December 2020.

Within one year after the data was collected, the parents of the children were

interviewed by telephone about their treatment. Increased peripheral leuko-

cytes were observed in 60.7% of children. Serum C-reactive protein (CRP) and

D-dimer were elevated in 53.3% and 75% of children, respectively. Gastroin-

testinal bleeding was identified in 39% of children. Of the children with intus-

susception, the symptoms subsided spontaneously in four children and after air

enema in another four. Four children underwent laparotomy and manual reduc-

tion. Nine patients underwent enterectomy and anastomosis. Enterectomy and

anastomosis were performed in the seven patients with intestinal perforation,

two of whom underwent enterostomy concurrently. Increased inflammation in-

dexes, elevated D-dimer, and persistent abdominal pain without relief may be

risk factors for surgical complications in children with IgAV.

Surgical complications in children with IgA vasculitis 135

Vol. 65(2): 134 - 142, 2024

Complicaciones quirúrgicas en niños con vasculitis IgA:

análisis clínico de 28 casos.

Invest Clin 2024; 65 (2): 134 – 142

Palabras clave: intususcepción; perforación intestinal; púrpura de Henoch-Schönlein;

enterectomía; enterostomía; dímero D; proteína C-reactiva.

Resumen. La intususcepción y la perforación intestinal son complicacio-

nes quirúrgicas graves en niños con vasculitis por IgA (IgAV). El diagnóstico

temprano y la intervención quirúrgica adecuada y oportuna pueden reducir el

daño de esta enfermedad a los niños. Analizamos retrospectivamente las carac-

terísticas clínicas, exámenes auxiliares, diagnóstico, tratamiento y pronóstico

de 28 niños con IgAV acompañada de complicaciones quirúrgicas (incluida in-

tususcepción en 21 pacientes y perforación intestinal en siete pacientes) que

ingresaron en el Hospital Infantil de Beijing de la Universidad Médica de la Ca-

pital, de mayo de 2016 a diciembre de 2020. Un año después de la recopilación

de datos, los padres de los niños fueron entrevistados telefónicamente sobre su

tratamiento. Se observó un aumento de leucocitos periféricos en el 60,7% de

los niños. La proteína C reactiva (PCR) y el dímero D séricos estaban elevados

en el 53,3% y el 75% de los niños, respectivamente. Se identificó hemorragia

gastrointestinal en el 39% de los niños. De los niños con intususcepción, los sín-

tomas desaparecieron espontáneamente en cuatro niños y después de un enema

de aire en otros cuatro. Cuatro niños fueron sometidos a laparotomía y reduc-

ción manual. Nueve pacientes fueron sometidos a enterectomía y anastomosis.

Se realizó enterectomía y anastomosis en los siete pacientes con perforación

intestinal, dos de los cuales fueron sometidos a enterostomía al mismo tiempo.

El aumento del índice de inflamación, el dímero D elevado y el dolor abdominal

persistente sin alivio pueden ser factores de riesgo de complicaciones quirúrgi-

cas en niños con IgAV.

Received: 20-01-2023 Accepted: 18-03-2024

INTRODUCTION

IgA Vasculitis (IgAV), formerly known

as Henoch-Schönlein purpura (HSP), is a sys-

temic small vasculitis syndrome that usually

occurs in children. In 1802, Heberden first

described this syndrome. He reported a child

with joint pain and subcutaneous edema, ab-

dominal pain, vomiting, blood in the stool

and blood in the urine, as well as “blood

spots” on the skin of the legs

. Initially,

Schönlein used the term “rheumatic purpu-

ra” to describe the association between joint

pain and purpuric skin lesions in children.

In 1874, Henoch described the syndrome of

purpura, severe abdominal cramps, and me-

lena. Later, Henoch referred to nephritis as

a complication of the syndrome

The disease can cause purpuric skin le-

sions and is accompanied by various symp-

toms, including gastrointestinal symptoms,

arthritis, and nephritis. Approximately 50%

136 Mu et al.

Investigación Clínica 65(2): 2024

to 75% of IgAV children present with gastro-

intestinal symptoms, such as abdominal pain

with various degrees, from mild abdominal

pain to severe intestinal colic. Some children

may have serious surgical complications, in-

cluding intussusception, intestinal perfora-

tion, intestinal necrosis, intestinal obstruc-

tion, significant gastrointestinal bleeding,

severe pancreatitis, and testicular torsion.

Delayed management and surgical interven-

tion are associated with serious consequenc-

es and even death. We should carefully watch

for surgical complications in children with

severe abdominal pain and gastrointestinal

bleeding. Complete examinations should be

performed promptly to identify early com-

plications, such as intussusception and in-

testinal perforation. Appropriate and timely

treatment should be given accordingly. There

are many reports on the use of corticoste-

roids and immunosuppressive drugs

. These

specific treatments are still controversial,

and their efficacy remains to be evaluated.

If the complications are severe, including se-

vere gastrointestinal complications or prolif-

erative glomerulonephritis, steroids or/and

immunosuppressive drugs may be needed,

including colchicine, dapsone, montelukast,

corticosteroids and azathioprine and other

drugs

4-6

There have been relatively few clinical

and epidemiological studies on childhood

HSP in the past few years. To investigate this

syndrome further, we retrospectively ana-

lyzed the clinical data of 28 children with

intussusception or intestinal perforation

among 7374 children with IgAV.

METHODS

From May 2016 to May 2020, 7374

children with IgAV were treated in the De-

partment of Traditional Chinese Medicine,

Beijing Children’s Hospital, Capital Medi-

cal University. Surgical complications were

reported in 28 children, including intussus-

ception in 21 children and intestinal perfo-

ration in seven children. We retrospectively

analyzed the clinical characteristics, aux-

iliary examinations, diagnosis, treatment,

and prognosis of all 28 children with IgAV.

All data were collected from our Hospital’s

electronic medical record system.

Inclusion criteria

Patients with primary cutaneous vascu-

litis were classified as HSP or allergic vascu-

litis

, according to the American College of

Rheumatology (ACR) standards and the stan-

dards proposed by Michel et al. If the patient

met the following three or more criteria, the

patients were classified as HSP: 1) palpable

purpura, 2) intestinal colic, 3) gastrointes-

tinal bleeding, 4) hematuria (macroscopic

or microscopic hematuria), 5) disease onset

age ≤ 20 years, and 6) there was no history

of drug treatment before the onset of vascu-

litis. Patients who met less than three cri-

teria were classified as allergic vasculitis. In

addition, if children were diagnosed with pri-

mary skin vasculitis or other diseases, such

as connective tissue diseases and infections,

especially meningitis

, they were excluded

from the study. Because electronic medical

records have only been used recently in our

Hospital, only no more than three years of

medical records were collected. The inci-

dence of IgA vasculitis with surgical com-

plications is not high, so only 28 cases were

collected. A pre-existing anatomical factor

as the starting point of the invagination, an

accidental finding, and where the intussus-

ception was spotted in such cases as to be

taken as criteria for inclusion or exclusion.

Treatment methods

Surgical treatment: Of the 21 children

with intussusception, the symptoms subsid-

ed spontaneously in four children and after

air enema in four. Four children underwent

laparotomy and manual reduction. Nine

patients underwent enterectomy and anas-

tomosis due to intestinal necrosis (one of

them underwent enterostomy). Enterecto-

my and anastomosis were performed in the

seven patients with intestinal perforation

Surgical complications in children with IgA vasculitis 137

Vol. 65(2): 134 - 142, 2024

(two of them underwent enterostomy con-

currently).

Conservative treatment methods: Ac-

cording to the specific conditions of the

children, the children were treated with fast-

ing, gastrointestinal decompression, enteral

or parenteral nutrition support, glucocor-

ticoids, proton pump inhibitors, or gamma

globulin, as needed.

Follow up

During the follow-up of all 28 children,

three children had adhesive intestinal ob-

struction and underwent secondary surgery.

Four children had multiple hospitalizations

due to IgAV nephritis, and one child was re-

admitted due to abdominal pain.

Statistical analysis

The data are expressed as mean ± stan-

dard deviation. The differences between the

groups were analyzed using the chi-square

test. P<0.05 was considered statistically sig-

nificant. All statistical analysis is performed

using SPSS software (SPSS 19, IBM, USA).

RESULTS

General data

Twenty-eight of the 7,374 children with

IgAV were accompanied by surgical compli-

cations, a rate of 0.38%. Twenty-one children

had intussusception, and seven children had

intestinal perforation. Of the 28 children, 10

were males and 18 were females. The age at

onset ranged from 3 to 13 years (mean age:

7.2 years). The time from onset of purpura to

occurrence of surgical complications ranged

from 1 to 37 days (mean: 11 days), and the

time from onset of abdominal pain to occur-

rence of surgical complications ranged from

1 to 40 days (mean: 9.8 days). Of these pa-

tients, 6 (6/28) children complained of ab-

dominal pain before purpura appeared.

Clinical manifestations

All 28 children had symptoms of skin

purpura. Seven children (7/28) had joint

swelling and pain, 11 (11/28) had gastroin-

testinal bleeding, and five (5/28) had IgAV

nephritis (two cases with nephrotic syndrome

and three cases with hematuria and protein-

uria). All patients presented with apparent

abdominal pain. Tenderness and rebound ten-

derness were noted on abdominal examina-

tion. An abdominal mass was palpated in four

patients. The severity of the complications

in patients was different, and four patients

developed shock (hemorrhagic shock in one

patient and septic shock in three patients).

Convulsions occurred in two children (due to

IgAV involving the cerebrovascular system).

Three patients were complicated with pneu-

monia. After surgery, venous thrombosis oc-

curred in three patients, renal dysfunction

in one patient, pancreatic damage in one pa-

tient, thrombocytopenia in one patient, and

coagulation disorders in one patient.

Auxiliary examination

A routine blood count showed increased

leukocyte count in 17 children (17/28), with

the highest value being 33.11 × 10

/L (nor-

mal range: 4-10×10

/L). Elevated CRP was

noted in 15 children (15/28), with the high-

est value being > 160 mg/L (average value

< 8 mg/L). Elevated D-dimer was noted in

21 children (21/28), with the highest value

being 7.565 mg/L (normal limits: 0-0.243

mg/L). Four children tested positive in the

C-urea breath test. Serum influenza B virus

IgM was positive in six children. Serum in-

fluenza A virus IgM was positive in two chil-

dren. Serum mycoplasma pneumoniae IgM

was positive in two children. According to

abdominal B-mode ultrasonography or surgi-

cal exploration in some patients, 12 children

were diagnosed with ileo-ileal intussuscep-

tion, six children with ileo-colonic intussus-

ception, two children with jejunal-jejunal

intussusception, and one child with colon-

colonic intussusception. Of the seven chil-

dren with intestinal perforations, six were

diagnosed with ileal perforation (including

a child accompanied by colonic perforation)

and a child with jejunal perforation.

138 Mu et al.

Investigación Clínica 65(2): 2024

DISCUSSION

IgAV is a common small-blood-vessel al-

lergic disease occurring during childhood; it

is most common in children aged three to

eight years

, with a predominance in males.

Skin purpura is most commonly accompanied

by digestive tract, joint, or kidney lesions. Ab-

dominal symptoms are reported in 50% to

75% of IgAV children, including gastrointesti-

nal bleeding, gastric ulcers, pancreatitis, cys-

tic effusion, and protein-losing enteropathy.

Some rare surgical complications include in-

tussusception, intestinal obstruction, intesti-

nal fistula, and intestinal perforation

3,9

Intussusception is the most common

condition for surgery in IgAV children, with

an incidence of 3% to 4%

. Intestinal per-

foration is the second-leading condition for

surgery, with an incidence of approximately

0.38%

. In the past three years, we have

treated 7,374 patients with IgAV; surgical

complications were reported in 28 patients.

The incidence of intussusception was 0.28%,

and the incidence of intestinal perforations

was 0.09%. Due to our Hospital’s late use of

electronic medical records, no more than

three years of case data have been counted.

If the statistical years were longer, the data

may be more meaningful. The pathogenesis

of intussusception may be due to the aseptic

inflammation of small blood vessels in the

intestinal wall, which increases the perme-

ability of the intestinal wall blood vessels

10,12

to result in extravasation of blood com-

ponents and segmental bleeding under the

serosa and mucosa. These lead to uneven

peristaltic movement, local slow peristalsis,

spasms of the intestinal loop, or even intus-

susception

.As the disease progresses, tis-

sue hypoxia and hypoperfusion may occur.

Intestinal wall edema, ischemia, and hypoxia

lead to intestinal necrosis and even intesti-

nal perforation

. In the acute phase of IgAV,

increased blood viscosity results in slow lo-

cal blood flow and exacerbates intestinal

ischemia and hypoxia.The circulating D-di-

mer level in children with IgAV is significant-

ly increased

. This reflects the presence of

hypercoagulable states and the formation of

thrombi. Another study

found that IgAV

caused a significant increase in serum CRP

in children with abdominal surgical compli-

cations, suggesting that the occurrence of

this complication may be closely related to

infectious factors. Furthermore, serum CRP

level has been positively correlated with the

occurrence of these complications. This fur-

ther indicates that infectious factors play

an essential role in the occurrence of surgi-

cal complications.We summarized the clini-

cal data of all 28 children in this study and

found that the average time from the onset

of abdominal pain to the first surgical com-

plication was 9.8 days, and the longest time

was 40 days. Increased peripheral leukocyte

count was observed in 60.7% of children. Se-

rum CRP and D-dimer levels were elevated

in 53.3% and 75% of children, respectively.

Gastrointestinal bleeding was identified in

39%. Increased inflammation index, elevat-

ed D-dimer, and persistent abdominal pain

without relief may be risk factors for surgical

complications in children with IgAV. For this

child population, we should carefully watch

for surgical complications. Early and precise

diagnosis and timely, appropriate treatment

can reduce the harm to these children.

Intussusception in IgAV patients usu-

ally originates from the ileum (90%) or jeju-

num (7%)

. The common sites of intussus-

ception are ileo-ileal (51.4%), ileo-colonic

(38.6%), and jejunal-jejunal (7.0%). In IgAV,

colo-colonic intussusception is extremely

rare, with only a few cases reported

. The

most common site of intestinal perforation

is the small intestine, especially the ileum,

followed by the jejunum. This may be due

to the intestinal wall swelling in the small

intestine in IgAV patients. Children with

IgAV also often have infection, which can

enlarge the aggregated lymphoid nodules

in the ileum. Therefore, the local intestinal

wall thickens and even protrudes into the

intestinal lumen to form a starting point of

intussusception

. Among the children with