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previously visited 061128 VIGOROUS ACHALASIA Random Case
Authored By: pedrad CSH, Radiologist, Chung Shan Medical University Hospital, Taiwan.
Patient: 3 year old male
History: Intermittent abdominal pain for days.
Vomit(+). Fever(-).
Constipation for long time.
History of umbilical hernia post surgery.
Images:small[medium]largeas-submittedimages only

Fig. 1: KUB

Fig. 2: UGI

Fig. 3: UGI

Fig. 4: UGI

Fig. 5: CT_Cor

Fig. 6: CT_Cor

Fig. 7: CT_Animator

Fig. 8: LGI

Fig. 9: LGI_Follow up

Fig. 10: Surgery

Fig. 11: Surgery
Images:small[medium]largeas-submittedimages only
Findings:

KUB:
 1. Much stool in colon.
 2. Scoliosis along L-spine with hemivertebrae in lower L-spine.

UGI(Barium swallow):
 1. Marked smooth dilatation of esophagus with air-contrast level
    due to lower esophagus segmental lumen narrowing and absence of
    esophageal peristalsis.

CT with IV contrast:
 1. Abnormal hight position of right kidney without hydronephrosis
    in right sub-phrenic region, in favor of renal ectopy.
 2. Scoliosis along L-spine with a right extra-hemivertebrae
    in L3/L4.

LGI(Barium enema):
 Well-distention of rectum without segmental narrowing.

Surgery:
 Myotomy and fundoplication.

DDx:

(1)Diffuse esophageal spasm (DES)
1. Uncoordinated contraction of esophagus
2. Barium swallow: multiple spasms with corkscrew appearance

(2)Nutcracker esophagus (another form of spasm)
1. High-amplitude coordinated contraction of esophagus.
2. Barium swallow: Nonspecific finding

(3)Achalasia (Classic achalasia):
1. Loss inhibitory nerves at lower esophageal sphincter with lower esophageal obstruction due to failure of sphincter to completely relax.
2. Usual presence of nonperistaltic isolated contractions or low-amplitude simultaneous contractions of the esophageal body in severer cases.
3. Barium swallow: lower esophageal obstruction with bird-beak and dilatation of proximal esophagus with stasis of barium (air-fluid level).

(4)Vigorous achalasia (VA, described in 1957 as a subset of achalasia or an early stage of classic achalasia):
1. Loss inhibitory nerves at lower esophageal sphincter with lower esophageal obstruction due to failure of sphincter to completely relax.
2. But presence of high-amplitude simultaneous contractions of the esophageal body.
3. Barium swallow: findings combining achalasia and DES.
4. Treatment: similar to classic achalasia.

(5)Esophageal stricture:
1. Acquired narrowing of esophagus caused by a variety of entities.
2. Barium swallow: lumen narrowing with mucosal change.

Diagnosis: 1. Vigorous achalasia
2. Right renal ectopy
3. Hemivertebrae, L-spine
Discussion: About esophagus:
1. Barium swallow revealed findings similar achalasia except a short segmental spasm / narrowing in lower esophagus.
2. Endoscopy (not showed) revealed smooth mucosa before obstruction, less likely esophageal stricture secondary to gastroesophageal reflux with esophagitis.
3. Esophageal manometry was not performed in our case.
4. The patient received surgery with myotomy and fundoplication smoothly.
Comments: post a comment

The pediatric doctors and surgeons help the little boy kindly. 
But this is not the end. 
There are still many congenital and acquired anomalies 
(but not a VACTERL) of the patient.
By Dr. Tsao

--pedrad CSH, 2006-12-04 06:03:16
Additional Details:

Case Number: 7445961Last Updated: 02-19-2007
Rating:

0 ratings
Anatomy: Gastrointestinal (GI)   Pathology: Other
Modality: CT, Conventional Radiograph, GI, Photograph, 3D ReconstructionAccess Level: Readable by all users
Keywords: vigorous achalasia, esophagusACR: 715.745Contained in: ahmeeed
Case has been viewed 1211 times.

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