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Congenital cricopharyngeal achalasia: a rare cause of dysphagia in an infant

The Turkish Journal of Pediatrics April 1, 2007 | Sari, Sinan; Eminoglu, Fatma Tuba; Belen, Fatma Burcu; Dalgi?, Buket; Et al Dysphagia secondary to congenital cricopharyngeal achalasia (CCA) is a rare condition in pediatric patients. We report a case of CCA in a 10-month-old boy presented with dysphagia, choking and nasal reflux. The diagnosis was made by barium studies. The patient was successfully treated by cricopharyngeal myotomy. site arnold chiari malformation

Key words: cricopharyngeal achalasia, dysphagia, children.

Oropharyngeal and esophageal disorders produce dysphagia. Oropharyngeal dysphagia is caused by abnormalities affecting the neuromuscular mechanism of the oral cavity, pharynx, and upper esophageal sphincter (UES). The cricopharyngeus muscle is the major component of the UES. Primary cricopharyngeal achalasia resulting from failure of relaxation of the cricopharyngeus muscle is a rare cause of dysphagia in children1. We herein present the case of a 10-month-old boy with cricopharyngeal achalasia treated by myotomy.

Case Report A 10-month-old boy was referred to our hospital because of cyanosis and dysphagia. The patient had been delivered full-term, through spontaneous vaginal delivery after an uncomplicated pregnancy. He had been hospitalized several times because of cyanosis, dysphagia, nasopharyngeal reflux and repeated episodes of aspiration pneumonia since birth and required tube feeding.

On admission, the patient weighed 6300 g (<3rd percentile). He had peroral cyanosis. Physical examination was unremarkable except systolic murmur heard along the second right intercostal space with radiation to the back. Choking, nasopharyngeal reflux and cyanosis persisted at every feeding during his hospitalization. Serum electrolyte analysis and thyroid functions were normal, as were the remainder of his laboratory tests. The patient was diagnosed as tetralogy of Fallot and major aortopulmonary collateral arteries after cardiologie evaluation. Cine-esophagogram with fluoroscopic observation of the swallowing showed regular posterior narrowing on the posterior wall of the esophagus at the level of 4th-5th cervical vertebrae with enlargement of the hypopharynx (Fig. 1). Therefore, he underwent cricopharyngeal myotomy and pharyngeal plexus neurectomy. He tolerated feeding very well on the 3rd postoperative day and was discharged after two weeks. He is presently 20 months old, has no feeding problems and an operation to correct congenital heart disease is planned.

Discussion Congenital cricopharyngeal achalasia (CCA) is an important but relatively seldom cause of dysphagia resulting from a functional narrowing at the level of the UES1,2. This is a rare entity in childhood and fewer than 50 pediatric cases have been reported10.

Symptoms of CCA include failure to thrive, regurgitation of food, choking, cyanosis, nasal reflux, coughing and recurrent aspiration pneumonia. These symptoms may develop weeks after birth. The age of initial presentation ranges from birth to six months1-6.

There may be delay in the diagnosis of disease, probably related to the fact that the condition of CCA is not widely recognized and the significance of early feeding difficulties in an affected child may be misinterpreted. In most cases, the symptoms are thought to be related to tracheoesophageal fistula, vascular ring or laryngeal cleft. A thorough history and observation of the patient while feeding are the essential points for diagnosis.

Congenital cricopharyngeal achalasia can be associated with Arnold-Chiari malformation, meningomyelocele or cerebral palsy. In children, however, isolated CCA is an unusual condition4.

The diagnostic workup includes an upper gastrointestinal series and manometric study. If there is a clinical suspicion of CCA, the radiologist must be informed. Cine-radiography can show posterior indentation (posterior bar) Aspiration and nasal reflux may be noted. Manometric study reveals the presence of a hypertensive UES, but it is difficult to perform in neonates and infants because the distance from pharynx to cricopharyngeal muscle area is short and the manometric catheter is prone to slip out at swallowing1,3,7,8. For these reasons, video fluoroscopy is sufficient to show disturbance of passage at pharynx and posterior bar in a patient with dysphagia.

Once the diagnosis of CCA is established, definitive treatment can be instituted. Treatment options include bougienage or balloon dilatation, endoscopic botulinum toxin injection or surgical myotomy1″6?·8-13. Apparently, no consensus exists among authors for the best management option for CCA. Some authors suggest that myotomy should be kept only for patients who have severe symptoms and/or respiratory compromise or who are unresponsive to other treatment modalities such as balloon dilatation or bougienage. However, others state that with the safe and very effective cricopharyngeus myotomy available, these alternatives cannot be recommended in children because these procedures have to be performed using sedation and general anesthesia and have to be repeated frequently1-5,9-111 Surgical myotomy was performed in our patient. On the third day of the post-operative period, dysphagia and nasopharyngeal reflux had improved dramatically. in our site arnold chiari malformation

[Sidebar] SUMMARY: San S, Eminoglu FT, Belen FB, Dalgi?§ B, Hasanoglu A, Boyunaga ?–L, K?¶ybasioglu A. Congenital cricopharyngeal achalasia: a rare cause of dysphagia in an infant. Turk J Pediatr 2007; 49: 193-195.

[Reference] REFERENCES 1. Skinner MA, Shorter NA. Primary neonatal cricopharyngeal achalasia: a case report and review of the literature. J Pediatr Surg 1992; 27: 1509-1511.

2. Korakaki E, Hatzidaki E, Manoura A, et al. Feeding difficulties in a neonate with primary cricopharyngeal achalasia treated by cricopharyngeal myotomy. Int J Pediatr Otorhinolaryngol 2004; 68: 249-253.

3. Muraji T, Takamizawa S, Satoh S, et al. Congenital cricopharyngeal achalasia: diagnosis and surgical management. J Pediatr Surg 2002; 37: E12.

4. Brooks A, Millar AJ, Rode H. The surgical management of cricopharyngeal achalasia in children. Int J Pediatr Otorhinolaryngol 2000; 56: 1-7.

5. Mahomed AA. Primary cricopharyngeal achalasia in infancy-myotomy treatment of choice. S Afr J Surg 2000; 38: 28-30.

6. Salib SA, Aubert D, Valioulis I, de Billy B. Cricopharyngeal achalasia – a cause of major dysphagia in a newborn. A case report. Eur J Pediatr Surg 1999; 9: 406-408.

7. Narla LD, Hingsbergen EA, Jones JE. Adult diseases in children. Pediatr Radiol 1999; 29: 244-254.

8. De Caluwe D, Nassogne MC, Reding R, de Ville de Goyet J, Clapuyt P, Otte JB. Cricopharyngeal achalasia: case reports and review of the literature. Eur J Pediatr Surg 1999; 9: 109-112.

9. Davis D, Nowicki M, Giles H. Cricopharyngeal achalasia responsive to balloon dilation in an infant. South Med J 2005; 98: 472-474.

10. Sewell RK, Bauman NM. Congenital cricopharyngeal achalasia: management with botulinum toxin before myotomy. Arch Otolaryngol Head Neck Surg 2005; 131: 451-453.

11. Blitzer A, Brin MF. Use of botulinum toxin for diagnosis and management of cricopharyngeal achalasia. Otolaryngol Head Neck Surg 1997; 116: 328-330.

12. Halvorson DJ. The treatment of cricopharyngeal dysmotility with a transmucosal cricopharyngeal myotomy using the potassium-titanyl-phosphate (KTP) laser. Endoscopy 1998; 30: 46-50.

13. Herberhold C, Walther EK. Endoscopic laser myotomy in cricopharyngeal achalasia. Adv Otorhinolaryngol 1995; 49: 144-147.

[Author Affiliation] Sinan Sari1, Fatma Tuba Eminoglu1, Fatma Burcu Belen1, Buket Dalgi?§1, Alev Hasanoglu1, ?–znur Leman Boyunaga2, Ahmet K?¶ybasioglu3 Departments of 1 Pediatrics, 2 Radiology, and 3 Otolaryngology, Gazi University Faculty of Medicine, Ankara, Turkey Sari, Sinan; Eminoglu, Fatma Tuba; Belen, Fatma Burcu; Dalgi?, Buket; Et al

BARGAIN HUNTER STOCK UP ON OFFICE GOODS.(VALLEY INC.)

Daily News (Los Angeles, CA) February 11, 2008 Byline: JULIA SCOTT DEPOT DEAL: Get $5 off $25 or more at Office Depot through Saturday. Print out a coupon at www.insidesocal.com/bargain for the discount.

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LINCOLNISH LUNCH THAI: Little Orchids Restaurant has a $5.95 lunch special, $1 more than a Lincoln Lunch. That’s pretty good for Thai food. It includes rice, an entree and one side (salad, soup or two pinkie-size egg rolls). Extra sides are $1.

I had red chicken curry with a generous ball of rice, and it was delicious. The super-crispy won tons were so hot I almost burned my tongue.

My friend’s chicken in curry sauce with green beans was also very tasty.

The fare is consistently good and comes quickly. Service can be curt, but the place is clean.

Get the lunch special Tuesdays through Saturdays before 3 p.m. It’s at 21614 Ventura Blvd. in Woodland Hills. Call 818-883-4848.

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Before the discount, nonmember prices are $20 for “Brokeback Mountain,” $15 for “Lost in Translation” and $15 for “Ray.” (As a comparison, “Brokeback” is $30 at Circuit City.) Many other HD DVDs fall in the $25 to $35 range. It’s unclear how long the sale lasts.

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When it comes to being maid of honor, three’s a crowd

The Record (Bergen County, NJ) June 17, 2009 | AMY DICKINSON AMY DICKINSON The Record (Bergen County, NJ) 06-17-2009 When it comes to being maid of honor, three’s a crowd AMY DICKINSON Date: 06-17-2009, Wednesday Section: BETTER LIVING Edtion: All Editions Column: ASK AMY Q. My very good friend just got engaged. I always thought I would be selected as her maid of honor, and the bride had hinted at it too.

Instead of me, the bride chose her cousin and another very good friend of ours, “Rhonda,” to be her joint maids of honor.

The bride said that she felt Rhonda very much wanted the role of maid of honor and that she didn’t want to deal with any potential anger from her had she chosen me.

I had always wanted to be chosen, but I never said it outright because I didn’t want to pressure the bride.

I feel as if my friend always gives in to Rhonda to keep her happy.

The bride said it was a very tough decision for her, and she still wants me involved in all of the wedding planning.

Should I tell the bride my feelings are hurt, or should I keep my mouth shut?

Should I actively involve myself in the planning, or should I wait for an invitation from the maids of honor? go to web site maid of honor

M.D.

Your friend is already gumming up her wedding by choosing two maids of honor, one of whom she appears to be afraid of.

(I’m already working on the script of this scenario for a film I’m tentatively calling “Maids of Horror.”) Use of the singular form in the designation “maid of honor” implies that only one maid will receive the honor of pushing the bride down the aisle.

The bride in question already made her choice and explained it to you. After choosing two maids, her second mistake was to ask a third person (you) to be involved in the wedding planning.

Don’t go there. Rhonda got there first, and life is too short to tangle with bridesmaid bullies over party planning.

Let your friend assign you to be keeper of the guestbook. This is an underappreciated honor, and a great way to meet guys.

Q. Recently, I took my 10-year-old niece to the mall. We were getting in the car when I noticed a little boy of around 4 or 5 years old get out of a car and stand by the bushes.

He was in plain view.

His mother walked over to the boy and pulled his pants down (all the way) and he started urinating.

I could not believe my eyes.

I said to her, “Don’t you think that’s rude? There is a bathroom right inside the store to the left.” The grandpa started yelling at me, cussing me out and calling me rude words.

The mother said I was a pervert for watching.

I replied that I had my 10-year-old niece in the car and she does not need to see that. The mother and the grandpa kept yelling at me.

I was so shocked!

Was I wrong to speak?

Still Shocked Let’s stipulate that 5-year-old boys don’t always give much notice when they have to “go.” But I completely agree with you that this family displayed poor manners. They are also volatile.

It would have been fine for you merely to let the family know there was a bathroom just inside the store (this might have been their first visit). this web site maid of honor

In addition to their rudeness, it was ill-mannered of you to call them rude to their faces and in front of their child especially when you could have highlighted their violation through your own polite concern.

It’s also a little scary to tangle with volatile strangers, especially when you have a young child in the car with you.

Dealing with this sort of behavior is what mall cops are for.

Q. On the topic of families “partying” at cemeteries: This Memorial Day at Arlington National Cemetery, I observed several family groupings around tombstones.

It was raining and they had put up lawn chairs and proceeded to have a family reunion of sorts.

They were very orderly some food was passed around, but no alcoholic beverages. I think this kind of gathering is fine but no beer and no sitting on tombstones.

Dorothy in Virginia I agree.

*** Send questions to askamy@tribune.com or write to Ask Amy, Chicago Tribune, TT500, 435 N. Michigan Ave., Chicago, IL 60611.

AMY DICKINSON

THIS ELECTRONIC VERSION MAY DIFFER SLIGHTLY FROM PRINTED VERSION. . If a …

Post-Tribune (IN) June 3, 2005 THIS ELECTRONIC VERSION MAY DIFFER SLIGHTLY FROM PRINTED VERSION . . If a man has enough horse sense to treat his wife like a thoroughbred, she will never turn into an old nag.

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When are parents going to stop defending their alcohol- and drug-addicted adult children? They need to accept responsibility for their actions before they can quit. If you bail them out every time they just continue the cycle.

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I am a liberal conservative. My husband is a conservative liberal. We have no idea what to argue about.

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Has anyone else noticed how politicians seem to be able to enact a new tax or raise the percentage of an old one faster than the speed of light? On the other hand, when it comes time to pay back some overpaid taxes, it suddenly becomes “tricky.” Does anyone else remember how a county income tax was supposed to provide property tax relief? I sure don’t remember the word “tricky” being used. howtogetridofpimplesovernightnow.com how to get rid of pimples overnight

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Before people start tying up the Quickly column with the Hoosier comments, there are 100 different tales, so just take the ones you like the best and that’s it.

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I would like to know if anyone has any idea how to get rid of rabbits in your yard. They are eating all my bushes and hopping five in a row in my yard. My husband wants to destroy them. Would someone please give me a better and safe way to get rid of them.

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The Futures Golf Tournament was great entertainment. It provided an opportunity to watch over 200 young women, many of whom will be future stars, playing to earn a spot on the LPGA golf tour. see here how to get rid of pimples overnight

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We share the same planet, the same country and the same neighborhood with many others. It is important to realize that our actions reflect on our neighbors. Starting today, try to be a better, more considerate neighbor. I will, too.

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How is it immoral to do stem cell research? If anything, it’s immoral not to do stem cell research. Think about the millions of lives that could be saved in the future.

Desire to help unquenchable: Gunman killed professor, but his research will live on.

The Columbus Dispatch (Columbus, OH) May 1, 2007 Byline: Kathy Lynn Gray May 1–Biomechanics professor Kevin Granata died a hero at Virginia Tech after saving a classroom full of students.

But that final selfless act is far from his only legacy. web site lower back exercises

His research into how to ease the lives of the sick and injured continues, including at his alma mater, Ohio State University.

Deborah Heiss, interim director of Ohio State’s division of physical therapy, is about to begin the second phase of a study on lower-back pain that she and Granata were leading when he and 31 other students and professors were killed by a student.

Granata was in his third-floor office in Virginia Tech’s Norris Hall when Seung-Hui Cho began his attack April 16. Granata directed 20 students into his office and had them lock the door before he went downstairs to try to help.

That’s when Cho shot him.

He was mourned not only in Blacksburg but also at Ohio State, where he had received his undergraduate degree and then his doctorate in biomechanics in 1993. He worked as a senior research engineer at Ohio State for four more years and had continued to collaborate with OSU staff members after he left.

He and Heiss were trying to figure out whether lower-back exercises they’d devised could strengthen the deep muscles enough to prevent recurring injuries. To test that strength, Granata and his lab assistants at Virginia Tech had designed two contraptions that Heiss now has in her physical therapy lab. The devices measure whether deep back muscles truly have recovered after an injury. in our site lower back exercises

“Once you have a back problem, what’s really important is that you’re fully rehabilitated so the injury doesn’t reoccur,” Heiss said.

Statistics show that a high percentage of people with back pain suffer reoccurrences within the first year because their muscles haven’t fully healed and strengthened, she said.

The back study evolved after Heiss met Granata at a grant-writing conference in 2003 and he agreed to be her mentor. Granata was excited about the study, and the two won a $250,000 National Institutes of Health grant for the work.

“My story with Kevin is not unique,” Heiss said. “When you walked into his office, he said, ‘How can I help you?’ I don’t know how he accomplished all that he did when he was so generous with his time.” Another Columbus resident with ties to Granata is hoping to show the researcher’s family how much he was appreciated.

Jerry Greenspan and Granata worked together in the OSU biomechanics laboratory for 3 1/2 years in the 1990s while Granata studied for his doctorate and Greenspan for his master’s degree. They ate lunch, hung out after work and endlessly talked about biomechanics.

“I saw Kevin progress from Kevin the bachelor, to Kevin the married man, to Kevin the married man with dog, to Kevin the married man with family,” said Greenspan, owner of Exercise Equipment Experts and Columbus Fitness Consultants in Upper Arlington.

They had lost touch the past few years, but when Greenspan heard about his friend’s death, he wanted to do something.

Greenspan has set up the Kevin P. Granata Memorial Fund through U.S. Bank and hopes to raise $50,000 for Granata’s wife, Linda, and their three children. He’s promised to donate 25 percent of gross profits from his two companies between April 16 and May 31 and has persuaded six exercise equipment manufacturers to donate $13,000 in equipment that he’ll raffle for the fund.

He’s also hoping that people in central Ohio will donate to the fund at U.S. Bank branches or by sending contributions made out to the fund to his office at 1640 W. Lane Ave., Upper Arlington 43221.

kgray@dispatch.com — Virginia governor closes loophole on guns A3 Distributed by McClatchy-Tribune Information Services.

Car plunges 7 stories off US parking deck

AP Worldstream May 31, 2011 BETHLEHEM, Pennsylvania (AP) ?ˆ” Authorities say a 21-year-old man drove his car off the top of a Pennsylvania casino parking deck and fell seven stories to his death.

The man died at a hospital Tuesday afternoon, hours after the vehicle fell from the top of the Sands Casino Resort garage in Bethlehem. go to website easton express times go to website easton express times

The car crashed through a railing of steel bars and cables. Police Capt. David Kravatz tells the Easton Express-Times that there were no brake marks.

Lehigh County Coroner Scott Grim ruled the death a suicide but says no note was found.

Sands spokeswoman Jennifer Reese told The Morning Call of Allentown that she won’t comment on whether the driver had been gambling there.

Bethlehem is about 45 miles (72 kilometers) northwest of Philadelphia.

Contract Notice: City of Round Rock Issues Request for Proposal for Library Services Strategic Master Plan (Texas)

US Fed News Service, Including US State News March 19, 2012 ROUND ROCK, Texas, April 17 — City of Round Rock has posted a Request for Proposal on March 16 for Library Services Strategic Master Plan. see here city of round rock

Contract, Tender Notice Type: Request for Proposal The Agency Requisition number is 12-007. web site city of round rock

Open Date and Time: April 17, 3:00 p.m.

Agency: City of Round Rock (M2462) Solicitation Type: 14 Days or more for entire solicitation package For any query with respect to this article or any other content requirement, please contact Editor at htsyndication@hindustantimes.com