Utility of sonographic assessment of the position of the third part of the duodenum using water instillation in intestinal malrotation: a single-center retrospective audit

Background

Intestinal malrotation and particularly volvulus are potentially devastating conditions. Upper gastrointestinal (UGI) contrast studies have been considered the gold standard for diagnosis. However the use of ultrasonography (US) has been increasingly described. We describe a method for delineating the duodenal anatomy with US as a means to exclude malrotation.

Objective

To report our experience using US to assess intestinal rotation.

Materials and methods

We conducted a retrospective audit of US scans performed at a tertiary referral centre to exclude malrotation for paediatric surgery between 2008 and 2011.

Results

One hundred thirty-nine infants were included, of whom 114 had a normal US scan. Of the 114, nine had subsequent upper gastrointestinal contrast studies that confirmed the initial results; there were no false-negatives. There were abnormal US scans in four infants associated with midgut volvulus and malrotation; there were no false-positives. The other 21 US scans were equivocal, and 11 of these had a confirmatory UGI contrast study; only one required surgery to correct malrotation.

Conclusion

US has been a safe and effective tool in the assessment of intestinal rotation at our institution. The main advantages of US imaging are its lack of ionising radiation and its rapid and accurate diagnosis of volvulus.     

The burden of excluding malrotation in term neonates with bile stained vomiting

Purpose

To determine the number of term infants with bilious vomiting (BV) referred to a neonatal surgical centre for exclusion of malrotation by upper gastrointestinal contrast (UGI) examination.

Methods

Retrospective review of term (>37/40) neonates <28 days of age undergoing UGI for exclusion of malrotation between Jan 2010 and Dec 2014 in a neonatal network with 30,000 term deliveries annually. Only infants with BV in the absence of alternative clinical/radiological diagnosis were included.

Results

One hundred and sixty-six infants met the inclusion criteria. Fourteen (9 %) infants had malrotation diagnosed by UGI and confirmed at laparotomy. Only 1 of 110 infants referred at 0–2 days of age had positive UGI compared to 13 of 56 infants referred after this age (p < 0.01). An increase in referrals followed the death of an infant from midgut volvulus and as a result one in 500 term infants are currently being referred.

Conclusion

Increasing awareness of the potential consequences of bilious vomiting appears to have resulted in increased referrals with no increase in detection of malrotation. Prospective studies are required to determine whether investigation of all infants with unexplained bilious vomiting is required and if it is possible to select cases for surgical referral.

     

Disorders of midgut rotation: making the correct diagnosis on UGI series in difficult cases

Background

In some series of malrotation small numbers of children are described in whom the position of the duodenojejunal flexure was considered to be normal on straight anteroposterior (AP) view of an upper gastrointestinal (UGI) series.

Objective

The purpose of this study was to illustrate children with disorders of midgut rotation in whom the diagnosis was difficult because on the straight AP view of the UGI series the duodenojejunal flexure was either not clearly depicted or was projected to the left of the midline close to its expected normal position at or close to the level of the duodenal cap.

Materials and methods

We reviewed 111 children with malrotation to determine the frequency that duodenojejunal flexure was not clearly depicted or was close to normal position.

Results

Seven patients had close to normal position of duodenojejunal flexure on AP view. The correct diagnosis was made on initial UGI series in four patients based on other features on AP and lateral views. In two of the other three patients, a repeat UGI series facilitated the correct diagnosis. In the final patient, an abnormal position of a nasojejunal tube suggested the correct diagnosis.

Conclusion

Accurate diagnosis of anomalies of midgut rotation requires careful assessment of the entire duodenal sweep on both AP and lateral views to avoid false-negative interpretations.     

Heterotaxy syndromes and abnormal bowel rotation

Background

Bowel rotation abnormalities in heterotaxy are common. As more children survive cardiac surgery, the management of gastrointestinal abnormalities has become controversial.

Objective

To evaluate imaging of malrotation in heterotaxy with surgical correlation and provide an algorithm for management.

Materials and methods

Imaging reports of heterotaxic children with upper gastrointestinal (UGI) and/or small bowel follow-through (SBFT) were reviewed. Subsequently, fluoroscopic images were re-reviewed in conjunction with CT/MR studies. The original reports and re-reviewed images were compared and correlated with surgical findings.

Results

Nineteen of 34 children with heterotaxy underwent UGI, 13/19 also had SBFT. In 15/19 reports, bowel rotation was called abnormal: 11 malrotation, 4 non-rotation, no cases of volvulus. Re-review, including CT (10/19) and MR (2/19), designated 17/19 (90%) as abnormal, 10 malrotation (abnormal bowel arrangement, narrow or uncertain length of mesentery) and 7 non-rotation (small bowel and colon on opposite sides plus low cecum with probable broad mesentery). The most useful CT/MR findings were absence of retroperitoneal duodenum in most abnormal cases and location of bowel, especially cecum. Abnormal orientation of mesenteric vessels suggested malrotation but was not universal. Nine children had elective bowel surgery; non-rotation was found in 4/9 and malrotation was found in 5/9, with discrepancies (non-rotation at surgery, malrotation on imaging) with 4 original interpretations and 1 re-review.

Conclusion

We recommend routine, early UGI and SBFT studies once other, urgent clinical concerns have been stabilized, with elective laparoscopic surgery in abnormal or equivocal cases. Cross-sectional imaging, usually obtained for other reasons, can contribute diagnostically. Attempting to assess mesenteric width is important in differentiating non-rotation from malrotation and more accurately identifies appropriate surgical candidates.     

Sonographic assessment of the retroperitoneal position of the third portion of the duodenum: an indicator of normal intestinal rotation

Background

The diagnosis of intestinal malrotation is based on an upper gastrointestinal contrast series (UGI), which is considered the imaging reference standard. It may however be challenging even for experienced paediatric radiologists.

Objective

The purpose of this study was to demonstrate the agreement between UGI and US in assessing the position of the third portion of the duodenum (D3) and to show that a retroperitoneal duodenum indicates normal forgut rotation.

Materials and methods

In a prospective study, US assessment of the duodenum and the superior mesenteric vessels was performed in consecutive children who were referred for clinically indicated UGI at a single institution.

Results

Eighty-five children, 5?months to 14?years old, were studied. In 82/85 (96%), both US and UGI suggested normal forgut rotation. In three children, US demonstrated a normal position of the D3 whereas UGI showed an abnormal position of the duodeno-jejunal junction.

Conclusion

US is a non-invasive, easily performed technique for excluding malrotation. UGI may be reserved for situations where US does not demonstrate a normal position of the D3.     

Comparison of upper gastrointestinal contrast studies and pH/impedance tests for the diagnosis of childhood gastro-oesophageal reflux

Background

The upper gastrointestinal (UGI) contrast study is used in the assessment of children with gastro-oesophageal reflux (GOR) and for detection of structural anomalies. The pH study is more sensitive than the UGI study for the diagnosis of GOR. The pH study has been replaced by the pH/impedance test, which detects both acid and nonacid reflux.

Objective

To compare the UGI contrast study with the pH/impedance test for the diagnosis of GOR in children.

Materials and methods

We retrospectively reviewed consecutive records of children investigated for GOR from October 2008 to February 2010, and compared the findings of UGI studies with those of pH/impedance tests.

Results

The UGI studies revealed GOR in 116 of 579 children (20%). Of the children undergoing a UGI study, 66 also underwent a pH/impedance test. Using the pH/impedance tests as the reference for GOR, UGI had a sensitivity of 42.8% and a negative predictive value of 24%. There was no significant correlation (P?>?0.05) between the reflux index and the number of reflux episodes in the pH/impedance tests and height of reflux in the UGI study. There were low incidences of malrotation (0.9%), hiatus hernia (1%) and delayed gastric emptying (0.4%).

Conclusion

The UGI study had low sensitivity for the diagnosis of GOR and low yield for the diagnosis of structural anomalies.     

CT appearance of the duodenum and mesenteric vessels in children with normal and abnormal bowel rotation

Background

Demonstration of the third duodenal segment (D3) in retroperitoneal location has been recently proposed as a method for excluding malrotation.

Objective

This study was performed to determine whether a retroperitoneal third duodenal segment can reliably exclude malrotation.

Materials and methods

CTs of 38 patients with proven malrotation and 100 patients without malrotation were evaluated for the location of the duodenum/proximal small bowel, and the relationship of the superior mesenteric vein (SMV) to superior mesenteric artery (SMA).

Results

The D3 segment was in normal retroperitoneal location in 100% of control patients, compared to 2.5% or (1 of 38) of patients with malrotation. Nine of 11 patients (91%) with malrotation imaged prior to surgery had the proximal bowel in an abnormal location, while all 100 control patients had it in a normal location. The SMV was in normal relationship to the SMA in 11/38 patients (29%) with malrotation, compared to 79% of normal controls. In 10 controls, a branch of the SMV was partially wrapped around the SMA, potentially mimicking partial mesenteric volvulus.

Conclusion

A retroperitoneal location of the D3 segment makes the diagnosis of malrotation unlikely but not impossible. Additional imaging of the duodenojejunal junction or cecum may be necessary to reliably exclude intestinal malrotation.     

Characterization of acoustic noise in a neonatal intensive care unit MRI system

Background

To eliminate the medical risks and logistical challenges of transporting infants from the neonatal intensive care unit (NICU) to the radiology department for magnetic resonance imaging, a small-footprint 1.5-T MRI scanner has been developed for neonatal imaging within the NICU. MRI is known to be noisy, and exposure to excessive acoustic noise has the potential to elicit physiological distress and impact development in the term and preterm infant.

Objective

To measure and compare the acoustic noise properties of the NICU MRI system against those of a conventional 1.5-T MRI system.

Materials and methods

We performed sound pressure level measurements in the NICU MRI scanner and in a conventional adult-size whole-body 1.5-T MRI system. Sound pressure level measurements were made for six standard clinical MR imaging protocols.

Results

The average sound pressure level value, reported in unweighted (dB) and A-weighted (dBA) decibels for all six imaging pulse sequences, was 73.8 dB and 88 dBA for the NICU scanner, and 87 dB and 98.4 dBA for the conventional MRI scanner. The sound pressure level values measured on the NICU scanner for each of the six MR imaging pulse sequences were consistently and significantly (P?=?0.03) lower, with an average difference of 14.2 dB (range 10–21 dB) and 11 dBA (range 5–18 dBA). The sound pressure level frequency response of the two MR systems showed a similar harmonic structure above 200 Hz for all imaging sequences. The amplitude, however, was appreciably lower for the NICU scanner, by as much as 30 dB, for frequencies below 200 Hz.

Conclusion

The NICU MRI system is quieter than conventional MRI scanners, improving safety for the neonate and facilitating siting of the unit within the NICU.     

Effective dose estimation for pediatric upper gastrointestinal examinations using an anthropomorphic phantom set and metal oxide semiconductor field-effect transistor (MOSFET) technology

Background

There is a need for updated radiation dose estimates in pediatric fluoroscopy given the routine use of new dose-saving technologies and increased radiation safety awareness in pediatric imaging.

Objective

To estimate effective doses for standardized pediatric upper gastrointestinal (UGI) examinations at our institute using direct dose measurement, as well as provide dose-area product (DAP) to effective dose conversion factors to be used for the estimation of UGI effective doses for boys and girls up to 10 years of age at other centers.

Materials and methods

Metal oxide semiconductor field-effect transistor (MOSFET) dosimeters were placed within four anthropomorphic phantoms representing children ≤10 years of age and exposed to mock UGI examinations using exposures much greater than used clinically to minimize measurement error. Measured effective dose was calculated using ICRP 103 weights and scaled to our institution’s standardized clinical UGI (3.6-min fluoroscopy, four spot exposures and four examination beam projections) as determined from patient logs. Results were compared to Monte Carlo simulations and related to fluoroscope-displayed DAP.

Results

Measured effective doses for standardized pediatric UGI examinations in our institute ranged from 0.35 to 0.79 mSv in girls and were 3–8% lower for boys. Simulation-derived and measured effective doses were in agreement (percentage differences <19%, T?>?0.18). DAP-to-effective dose conversion factors ranged from 6.5?×10^?4?mSv per Gy-cm² to 4.3?×?10^?3?mSv per Gy-cm² for girls and were similarly lower for boys.

Conclusion

Using modern fluoroscopy equipment, the effective dose associated with the UGI examination in children ≤10 years at our institute is <?1 mSv. Estimations of effective dose associated with pediatric UGI examinations can be made for children up to the age of 10 using the DAP-normalized conversion factors provided in this study. These estimates can be further refined to reflect individual hospital examination protocols through the use of direct organ dose measurement using MOSFETs, which were shown to agree with Monte Carlo simulated doses.     

Pre- and postoperative imaging of adolescents undergoing laparoscopic adjustable gastric banding surgery

Background

As childhood obesity rates rise, laparoscopic adjustable gastric banding (LAGB) is being investigated as a bariatric surgical option in adolescents.

Objective

To examine pre- and postoperative imaging in adolescents undergoing LAGB, describe the most common abnormal preoperative imaging findings, and illustrate the typical appearance and variants on postoperative upper-gastrointestinal (UGI) examinations.

Materials and methods

A retrospective chart review was performed of all adolescents from 2008 to 2010 undergoing LAGB at a single tertiary-care pediatric hospital. The picture archiving and communication system was queried for all imaging obtained before and after surgery. Postoperative UGI studies were analyzed for common patterns.

Results

Twenty-seven obese adolescents who underwent LAGB were identified. Twenty-five had preoperative imaging, most commonly a UGI study (81.5%). Eight UGI studies were abnormal but did not impact surgery. Preoperative chest and neck radiographs were also common. Intraoperative imaging was rare. Seventy-three postoperative UGI studies were performed on 22 children (range, 2–12 studies). A common postoperative imaging pattern was observed in 19/22 (86%) children. No complications were observed.

Conclusion

The most common pre- and postoperative imaging studies in adolescents undergoing LAGB are UGI studies. Pediatric radiologists should be familiar with the imaging of LAGB as this procedure becomes increasingly common.     

NICU Admissions and Maternal Stress Levels

Objective

To determine the stress levels among mothers of babies admitted in Neonatal Intensive Care Unit (NICU) and to identify demographic parameters that influence their stress levels.

Methods

Stress levels were assessed using Parental Stressor Scale: Neonatal Intensive Care Unit (PSS: NICU) questionnaire among 100 NICU mothers by doctors between 6 and 8 d of admission. Maternal stress was quantified using Likert scale as low (1–2.9), medium (3–3.9) and high (4–5). The data was analyzed using SPSS Ver.16.

Results

The mean scores for the subscales sights and sounds, looks and behaviour and alteration in the parental role were 2.55, 4.1 and 4.12 respectively. Increased maternal age, prematurity of baby, longer NICU stay and inability to directly breastfeed the baby were associated with higher stress levels.

Conclusions

NICU mothers are under significant stress and appropriate counseling targeted towards specific stressors is required.     

Non Benign Neonatal Arrhythmias Observed in a Tertiary Neonatal Intensive Care Unit

Objective

To analyze non benign neonatal arrhythmias (NA) observed in a tertiary neonatal intensive care unit (NICU).

Methods

From June 2006 through July 2011, newborns admitted to the NICU for NA or diagnosed as NA after hospitalization were evaluated retrospectively. The newborns with non benign NA were included in the study.

Results

During the study period, the incidence of non-benign NA was 0.7 % (n?=?55/7880). The mean age at diagnosis was 16.7?±?1.8 d ranging from 1 d to 90 d. The most common type was supraventricular arrhythmia (SVT) with an incidence of 0.3 %. Univariate analyses showed that there were significant differences between the survived and died infants according types of congenital heart disease (CHD), electrolyte imbalance, and arrhythmias. The mortality rates were higher among infants with obstructive type left-to right shunt and common mixing type CHD. The most dangerous type of electrolyte imbalance was hyperkalemia.

Conclusions

Many arrhythmias could not be noticed at neonatal period even in NICU, implying that it is increasingly important for the physician to be aware of the etiology, development, and natural history of these arrhythmias.     

Diagnostic performance of the upper gastrointestinal series in the evaluation of children with clinically suspected malrotation

Background Malrotation is a congenital disorder of abnormal intestinal rotation and fixation that predisposes infants to potentially life-threatening midgut volvulus. Upper gastrointestinal tract (UGI) examination is sometimes equivocal and can lead to inaccurate diagnosis. Objective To determine the diagnostic performance of UGI examinations in children who subsequently underwent a Ladd procedure for suspected malrotation or volvulus. Materials and methods We reviewed all children up to 21 years old who had undergone both a UGI examination and a Ladd procedure for possible malrotation across 9 years. Children were excluded if they had not undergone either a UGI examination or a Ladd procedure and if congenital abdominal wall defects were present. Results Of 229 patients identified, 166 (59% male, median age 67 days) were included. Excluded were 47 without a UGI series, 12 with omphalocele or gastroschisis, 1 without verifiable operative data, 1 who had not undergone a Ladd procedure, and 2 older than 21 years. Of the 166 patients, 40% were neonates and 73% were <12 months old, and 31% presented with bilious vomiting and 15% with abdominal distention. Of 163 patients with surgically verified malrotation, 156 had a positive UGI examination, a sensitivity of 96%. There were two patients with a false-positive UGI examination and seven with false-negative examination. Jejunal position was normal in six of the seven with a false-negative examination and abnormal in the two with a false-positive examination. Of 38 patients with surgically verified volvulus, 30 showed volvulus on the UGI series. Five required bowel resection and three died. Conclusion Jejunal position can lead to inaccurate UGI series interpretation. Meticulous technique and periodic assessment of performance will help more accurately diagnose difficult or equivocal cases.     

Ultrasound diagnosis of midgut volvulus: the “whirlpool” sign

The authors present their US findings in 24 patients with proved complicated midgut malrotation: volvulus in 18 and occlusive Ladd's bands in 6. All the 24 patients have had US examination prior to surgery. Contrast examinations were performed in only 9 patients, always after US and before surgery. The sonographic whirlpool pattern of the superior mesenteric vein and mesentery around the superior mesenteric artery was detected in 15 of the 18 patients with midgut volvulus, and was best seen using Doppler color. Embryological signification of midgut malrotation is discussed.Presented at the IPR meeting in Stockholm, May 1991. Selected for publication by an International Group of the ESPR     

Mortality and Morbidity in Extremely Low Birth Weight (ELBW) Infants in a Neonatal Intensive Care Unit

Objective

To determine the morbidity and mortality in ELBW babies till discharge from a Neonatal Intensive Care Unit (NICU).

Methods

This study was a prospective observational study conducted in a 40 bed well equipped level III care NICU between 01.12.2006 and 30.04.2008. All ELBW babies admitted during this period were assessed for morbidities and interventions required during NICU stay and for their outcome like survival or death.

Results

The survival rate of 87 ELBW babies admitted during this period was 56.1 %. Pulmonary hemorrhage was the commonest cause of death (25 %) followed by respiratory distress syndrome (22.5 %), intraventricular hemorrhage (22.5 %) and sepsis (20 %). Significantly higher number of non-survivors were <750 g at birth (p?=?0.0001) and <28 wk gestation (p?=?0.0001). Small for gestational babies had better chances of survival compared to those appropriate for gestational age (p?=?0.005). RDS (67.8 %), probable sepsis (62.1 %) and hyperbilirubinemia (59.8 %) were the most frequent morbidities. Conventional ventilation (72.4 %) and nasal CPAP(48.3 %) were the commonest respiratory interventions. Surfactant replacement therapy was required in 47.1 % babies.

Conclusions

ELBW babies have a major contribution to mortality in a NICU. Babies with birth weight <750 g and gestation <28 wk have poor survival. RDS, pulmonary hemorrhage, IVH and sepsis are the common causes of death while RDS, sepsis and hyperbilirubinemia are the most common morbidities.     

Coloboma and anorectal malformations: a rare association with important clinical implications

Purpose

In a patient with an anorectal malformation (ARM), the presence of a coloboma is commonly associated with other serious anomalies.

Methods

Our database and the world literature were reviewed searching for associated defects in patients with ARM and coloboma.

Results

Of 2,482 ARMs in our database, 11 had coloboma (0.4 %): 2 were females. No specific associated type of ARM was identified. Six patients were developmentally delayed. Eight had a cardiac anomaly (3 had TAPVR, 2 VSD, 3 ASD), five required a cardiac operation. Five had a gastrointestinal anomaly (3 malrotation, 1 biliary and 1 duodenal atresia). Six had eye and seven had ear anomalies. Five had a genetic abnormality. In the literature, 71 patients with ARM and coloboma were found: 65 % were females. 24 % died prematurely. 74 % were developmentally delayed. 70 % had a cardiac malformation (35 % had TAPVR, 38 % required an operation). 57 % had gastrointestinal anomalies (31 % malrotation, 31 % biliary atresia, 17 % Hirschsprung disease). Eye and ear anomalies were present in 80 and 97 % of patients, respectively. 81 % had a genetic abnormality.

Conclusions

An ocular inspection in patients born with ARM is crucial. The finding of a coloboma should increase awareness to evaluate for a developmental, cardiologic or gastrointestinal anomaly.     

Improvement of survival in infants with congenital diaphragmatic hernia in recent years: effect of ECMO availability and associated factors

Background/Purpose

Survival of patients with congenital diaphragmatic hernia (CDH) depends both on non-modifiable congenital conditions and on modifiable pre and postnatal management. ECMO improves survival up to 80% in neonates with CDH in the best ECMO centers worldwide. The first Neonatal ECMO Program in Chile was started in our University in 2003. Our objective is to determine the impact of a Neonatal ECMO Program in a level III NICU on newborns with CDH.

Methods

Data of all newborns with CDH admitted to our NICU was separated into two groups: pre ECMO (1996–2003) and ECMO (2003–2007). Crude and adjusted odds ratios for 24 months survival were estimated by logistic regression.

Results

Data of 46 newborns with CDH was analysed, 20 in the pre ECMO and 26 in the ECMO period. Patient characteristics were similar in both groups; however, 24-month survival increased significantly from 25% (5/20) in the pre ECMO period to 77% (20/26) in the ECMO period (P = 0.001). Adjusted odds ratios for 24-month survival were 26.98 for OI ≤ 40, 7.58 for 5 min Apgar ≥ 7 and 17.5 for ECMO availability.

Conclusions

The establishment of an ECMO program was associated with a significant increase in long-term survival for infants with CDH.     

Small bowel volvulus with a jejunal trichobezoar

Background

Small bowel volvulus caused by a jejunal trichobezoar is an extremely rare and life-threatening emergency in children.

Case characteristics

An 8-year-old girl with abdominal pain and persistent bilious vomiting.

Observation

The abdominal computed tomography scan showed a solitary intraluminal mass and a whirl sign, suggesting the small bowel volvulus. Emergency laparoscopic exploration revealed the rotated segment of small bowel loops by a jejunal trichobezoar.

Outcome

Satisfactory recovery after surgery.

Message

Trichobezoars should be considered in the differential diagnosis of abdominal pain and projectile vomiting in children.     

Effect of Multisensory Stimulation on Neuromotor Development in Preterm Infants

Objective

To investigate the effect of Auditory, Tactile, Visual and Vestibular stimulus (ATVV) on neuromotor development in preterm infants.

Methods

Fifty preterm infants born at 28–36 wk with a birth weight ranging from 1,000–2,000 g were recruited for the study. They were block randomized into a control group (n?=?25) and study group (n?=?25). New Ballard score was used for the baseline measurement of neuromaturity in both groups. In neonatal intensive care unit (NICU), the study group received multisensory stimulation for 12 min per session, 5 sessions per wk along with routine NICU care either from 33 wk corrected gestational age for infants born at 28–32 wk or from 48 h of birth for infants born at 33–36 wk until discharge from the hospital. The control group received the routine NICU care. At term age the preterm infants were assessed using Infant Neurological International Battery (INFANIB) and the groups were compared using independent t test.

Results

The multisensory stimulated infants showed higher neuromotor score (p?=?0.001) compared to the control group. The french angle components of INFANIB including heel to ear (p?=?0.016) and popliteal angle (p?=?0.001) were statistically significant between the groups.

Conclusions

Multisensory stimulation appears to have a beneficial effect on the tonal maturation in preterm infants. However, further studies are warranted to investigate the long-term effects of multisensory stimulation on neurodevelopmental outcome in preterm infants.     

Isolated intestinal neuronal dysplasia Type B (IND-B) in Japan: results from a nationwide survey

Purpose

Intestinal neuronal dysplasia Type B (IND-B) has been proposed to be an allied disorder of Hirschsprung’s disease (ADHD). The original histological criteria included hyperganglionosis, giant ganglia, ectopic ganglion cells and an increased AChE activity in the lamina propria. The criteria for IND-B have been gradually revised. The present diagnostic criteria are [1] more than 20 % of the submucosal ganglia contain nine or more ganglion cells and [2] the patient is older than 1 year. To clarify the current status of IND-B in Japan, a nationwide retrospective cohort study was performed.

Methods

Questionnaires were sent to 161 major institutes of pediatric surgery and gastroenterology in Japan.

Results

A total of 355 cases of ADHD were collected, including 18 cases of IND-B (5 %). Based on original criteria, 13 out of 18 cases were diagnosed as IND-B. However, only four cases met the current criteria. Three of the four patients (75 %) required pull-through operation. All of the patients exhibited giant ganglia and ganglioneuromatosis-like hyperplasia of the myenteric plexus.

Conclusions

IND-B cases matching the current criteria are thought to be quite rare and they are associated with marked hyperplasia of the myenteric plexus. “True” IND-B is a rare and intractable disease.