Congenital portosystemic shunts are present in one in 30,000 children. Among the associated risks of severe complications are neonatal cholestasis, benign and malignant liver tumors, hepatopulmonary syndrome, portopulmonary hypertension, and encephalopathy. They can be detected on prenatal ultrasonograms, during the investigation of a positive galactosemia screening test in neonates or of a complication, or be found fortuitously on an abdominal ultrasound. Small intrahepatic shunts may resolve spontaneously within one year of age, but other shunts such as extrahepatic, persistent ductus venosus or persisting intrahepatic shunts, must be closed in one or two steps, by interventional radiology techniques or surgically. The plasticity of the intrahepatic portal system allows revascularization of the liver after shunt closure, even when no intrahepatic portal structures can be detected on imaging studies. This leaves little or no place for liver transplantation in the management of these children.

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Congenital Portosystemic Shunts in Children: Recognition, Evaluation, and Management

Cochlear implantation has become an accepted treatment for deafness. As the frequency of cochlear implantation has increased, requests for images have also increased in the work-up for candidates. An absent cochlear nerve (CN) is a contraindication to cochlear implantation. Therefore, MRI is performed to evaluate the CN in patients with sensorineural hearing loss. Recently, some authors have reported the relationship between cochlear nerve canal (CNC) stenosis and CN hypoplasia. To review the relationship between CNC and CN. During a period of 78 months, 21 children (42 ears) with unilateral or bilateral sensorineural hearing loss underwent both HRCT and MRI of the cochlear nerve. We retrospectively reviewed two factors: the evaluation of inner ear malformations and the relationship between CNC stenosis and CN hypoplasia. Inner ear malformations were recognized in ten ears. The mean CNC diameter was approximately 2 mm (ranging from 0.6 to 2.7 mm). CN hypoplasia was seen in eight of the 42 ears; all eight were associated with CNC stenosis (≤1.5 mm). Of the 34 ears with normal CN, 32 had CNC >1.5 mm in diameter and the remaining two ears, with incomplete partition type I, had CNC stenosis. Children with CNC stenosis had a high incidence of CN hypoplasia. CNC stenosis (≤1.5 mm) suggests CN hypoplasia. On the other hand, CN hypoplasia was not seen in children with CNC diameter >1.5 mm. Therefore, we conclude that children with CNC stenosis or malformations on HRCT should receive MR imaging of the CN.

CT and MR imaging for pediatric cochlear implantation: emphasis on the relationship between the cochlear nerve canal and the cochlear nerve

Objectives Hypoplasia of the auditory nerve (AN) refers to significant narrowing of the VIIIth cranial nerve which could compromise stimulation of the nerve by electrical pulses delivered from a cochlear implant (CI), thereby hindering activity in other parts of the auditory pathways. To compensate, high current levels or increased charge may be required to elicit auditory perception causing current to spread to other cranial nerves and potentially resulting in unwanted myogenic responses. Deficits in central auditory activity could reduce perception of speech and language. In the present study, we measured auditory brainstem responses in children with and without hypoplasia of the AN to answer the following questions. In children with hypoplastic ANs, (a) can CI stimulation evoke typical patterns of activity from the AN and brainstem?, (b) do brainstem responses change with CI experience?, (c) are evoked responses dependent on the size of the AN pathway?, and (d) does auditory development measured by behavioral tests of speech perception develop more slowly than in peers with normal AN diameter? Design Of 807 children using CIs in our program, 20 (2.5%) were identified as having AN hypoplasia using high-resolution computed tomographic scan and/or magnetic resonance imaging. An age-matched control group of children using CIs with normal AN diameter were recruited to compare electrophysiological and behavioral measures. Radiologic imaging was used to measure the diameter of the internal auditory canal (IAC), auditory nerve canal (ANC), and AN. Electrophysiological testing of the evoked compound action potential and auditory brainstem response was performed at CI activation and every 3 mo after initial testing up to 2 yr. Peak latency and waveform morphology were compared between study and control groups. Tests of speech perception and discrimination were attempted every 12 mo after device activation up to 10 yr. Results : Hypoplastic AN was identified as moderate to critical stenosis of the IAC, ANC, and AN. Initial electrically evoked compound action potential responses were mostly absent in children with AN hypoplasia. In the time window when electrically evoked auditory brainstem responses would be expected, some responses included single amplitude peaks at normal wave eV latencies, but the majority were abnormal, with peaks at atypical latencies or with no observable wave peaks. All evoked responses were inconsistent over time and did not reflect a typical pattern of auditory brainstem development. Speech perception scores were significantly poorer in the study group compared with controls and did not improve with CI experience. The type of abnormal evoked waveform response was independent of IAC, ANC, or AN diameter and also independent of behavioral outcome measures. Conclusions : Evoked responses recorded in CI children with AN hypoplasia indicate a high incidence of nonauditory activity with CI use. The range of abnormal responses was not predicted by the severity of the hypoplastic AN or associated structures. This, along with poorer auditory development compared with peers with normal AN diameters, suggests that children with hypoplasia of the AN are poor candidates for cochlear implantation.

Electrophysiologic and behavioral outcomes of cochlear implantation in children with auditory nerve hypoplasia.

Clinical features, images, complications, treatments, and prognosis of 10 children with congenital portosystemic shunt (CPSS) were reviewed. Nine children were diagnosed with intrahepatic shunts while one presented with extrahepatic shunt. CPSS was detected by prenatal ultrasonography in four infants. Three infants presented with galactosemia without an enzyme deficiency. Two children presented with mental retardation and attention deficit hyperactivity disorder. Pulmonary hypertension developed in two patients. Spontaneous closure occurred in four infants with intrahepatic shunts including patent ductus venosus. The shunts were closed using transcatheter embolizations in four patients with intrahepatic shunts. Conclusion: Intrahepatic shunts may close spontaneously. Transcatheter embolization is effective for the treatment of symptomatic intrahepatic shunts.

Clinical features of congenital portosystemic shunt in children

High prevalence of inner-ear and/or internal auditory canal malformations in children with unilateral sensorineural hearing loss

Purpose
The aim of this study was to evaluate the diameters of the various bony canals of the inner ear in patients with sensorineural hearing loss (SNHL) and establish criteria for detecting hypoplasia of the bony canal of the cochlear nerve

Computed tomographic features of the bony canal of the cochlear nerve in pediatric patients with unilateral sensorineural hearing loss

Background: Portosystemic shunt is one of the main causes of persistent hypergalactosemia without enzyme deficiency, but the diagnostic imaging strategy has not yet been established. The purpose of the present study was to establish a diagnostic imaging strategy.



Methods:  A retrospective investigation of the clinical and imaging findings of 10 children with persistent hypergalactosemia without enzyme deficiency detected by screening was undertaken.



Results:  Abnormal ultrasonography (US) findings were detected in all eight patients with liver disorders. In three patients with citrin deficiency, the combination of fatty liver on US and laboratory evidence of cholestasis led to the diagnosis. In three patients with portosystemic shunt, US on sedation clearly depicted the shunt vessels. The extent was more easily understood on contrast computed tomography (CT). Per-rectal portal scintigraphy with N-isopropyl-p-I-123 iodoamphetamine and lung perfusion scintigraphy with 99mTc macroaggregated albumin were useful for evaluation of portal shunt index and assessment of pulmonary arteriovenous shunt. One patient underwent transarterial coil embolization. In two patients with hepatic tumor, the lesions and its vascularity were clearly demonstrated on US and dynamic CT. In one patient, small shunt index on per-rectal portal scintigraphy suggested no need for treatment. The other patient was treated with a combination of steroid, radiation, and interventional radiology. The etiology remained unknown in two children.



Conclusions:  In the assessment of hypergalactosemia, US is the modality of choice. CT is a useful tool for more detailed evaluation of the abnormalities found on US. Per-rectal portal scintigraphy and pulmonary perfusion scintigraphy play an important role in the evaluation of portosystemic shunt. Interventional radiology is sometimes effective.

Hypergalactosemia in early infancy: diagnostic strategy with an emphasis on imaging.

Anarrow duplicated internal auditory canal (IAC) is a very rare congenital inner ear malformation, with only 5 cases (to our knowledge) reported in the literature. 1-5 A 14-year-old girl with unilateral sensorineural hearing loss (SNHL) showed 2 narrow 1 mm) bony canals in the inner ear portion of the left temporal bone on computed tomograms (CTs). The anterior canal continued to the bony canal of the facial nerve, and the posterior canal continued to the cochlea. Other than a lack of development of the canal for the cochlear nerve, the cochlea was normal. High-resolution magnetic resonance images (MRIs) revealed a single thin cranial nerve located in the anterior canal. However, a cranial nerve was not found in the posterior canal. Therefore, a diagnosis of a unilateral narrow duplicated IAC with a hypoplastic vestibulocochlear nerve and vestibular-semicircular canal malformation was made. Computed tomography plays an important role in the evaluation of the bony structure; however, assessment of the neural contents on MRIs is important for the appropriate diagnosis and treatment of patients with a narrow duplicated IAC. We report a case of a unilateral narrow duplicated IAC, with a particular emphasis on the imaging findings of thin-slice high-resolution CTs and MRIs.

Tatsuo Kono

Papers

Computed tomographic features of the bony canal of the cochlear nerve in pediatric patients with unilateral sensorineural hearing loss

Hypergalactosemia in early infancy: diagnostic strategy with an emphasis on imaging.

Narrow duplicated internal auditory canal: a rare inner ear malformation with sensorineural hearing loss.