Multidisciplinary Aspects of 104 Patients with Pierre Robin Sequence

Abstract

Objective

To describe Pierre Robin sequence patients with a cleft palate from a multidisciplinary perspective.

Patients

A total of 104 individuals with Pierre Robin sequence and cleft palate, born between 1980 and 2010.

Method

Data were collected retrospectively and compared with large control groups.

Results

Of 104 patients, 19 (18.3%) were treated with a nasopharyngeal or oropharyngeal tube, continuous positive airway pressure, and/or a tracheotomy. The mean weight percentile for newborns with Pierre Robin sequence was 30.9. It decreased to 29.9 at the time of cleft palate repair (mean age, 13.7 months) (P = .78). Of 87 patients, 30 (34.5%) developed normal speech after cleft palate repair. Of 93 nonsyndromic Pierre Robin sequence patients, 31 (33.3%) had or are having surgery for velopharyngeal insufficiency, a rate that is significantly higher when compared with a control group of cleft palate–only patients (19.4%; P = .004). Of 31 patients, 25 (80.6%) developed normal resonance after surgery for velopharyngeal insufficiency. There was no significant difference in the rate of syndromes between the Pierre Robin sequence patients and a control group of cleft palate patients without Pierre Robin sequence (P = .25). Seven of 39 boys (17.9%) with Pierre Robin sequence had a diagnosis of autism spectrum disorder.

Conclusion

Even though the mean weight percentile for newborns with Pierre Robin sequence was low, the patients did not show a growth spurt during the first year of life. The high rate of velopharyngeal insufficiency after cleft palate repair in patients with Pierre Robin sequence needs further investigation. Also, the high rate of autism spectrum disorder among boys with Pierre Robin sequence prompts further investigation.

Keywords

cleft palate fistula growth Pierre Robin sequence psychology speech VPI

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References

Abyholm

D'Antonio

Davidson Ward

S.L.

Kjøll

Saeed

Shaw

Sloan

Whitby

Worhington

Wyatt

, VPI Surgical Group. Pharyngeal flap and sphincterplasty for velopharyngeal insufficiency have equal outcome at 1 year postoperatively: results of a randomized trial. Cleft Palate Craniofac J. 2005; 42: 501–511.

Anderson

I.C.

Sedaghat

A.R.

McGinley

B.M.

Redett

R.J.

Boss

E.F.

Ishman

S.L.

Prevalence and severity of obstructive sleep apnea and snoring in infants with Pierre Robin sequence. Cleft Palate Craniofac J. 2011; 48: 614–618.

Anderson

K.D.

Cole

Chuo

C.B.

Slator

Home management of upper airway obstruction in Pierre Robin sequence using a nasopharyngeal airway. Cleft Palate Craniofac J. 2007; 44: 269–273.

Andersson

E.M.

Sandvik

Abyholm

Semb

Clefts of the secondary palate referred to the Oslo Cleft Team: epidemiology and cleft severity in 994 individuals. Cleft Palate Craniofac J. 2010a; 47: 335–342.

Andersson

E.M.

Sandvik

Tordal

I.B.

Abyholm

Pharyngoplasty after primary repair of clefts of the secondary palate. Scand J Plast Reconstr Surg Hand Surg. 2010b; 44: 26–30.

Breugem

C.C.

Mink van der Molen

A.B.

What is “Pierre Robin sequence”?

J Plast Reconstr Aesthet Surg. 2009; 62: 1555–1558.

Brugha

McManus

Meltzer

Smith

Scott

F.J.

Purdon

Harris

Bankart

Autism Spectrum Disorders in Adults Living in Households Throughout England—Report From the Adult Psychiatric Morbidity Survey 2007. Leeds: NHS Information Centre; 2009.

Bush

P.G.

Williams

A.J.

Incidence of the Robin anomalad (Pierre Robin syndrome). Br J Plast Surg. 1983; 36: 434–437.

Caouette-Laberge

Bayet

Larocque

The Pierre Robin sequence: review of 125 cases and evolution of treatment modalities. Plast Reconstr Surg. 1994; 93: 934–942.

10.

Carey

J.C.

Fineman

R.M.

Ziter

F.A.

The Robin sequence as a consequence of malformation, dysplasia, and neuromuscular syndromes. J Pediatr. 1982; 101: 858–864.

11.

Chapman

Willadsen

The development of speech production in children with cleft palate. In: Howard

Lohmander

, eds. Cleft Lip and Palate: Speech Assessment, Analysis and Intervention. Oxford: Wiley; 2011: 23–40.

12.

Ehlers

Gillberg

The epidemiology of Asperger syndrome. A total population study. J Child Psychol Psychiatry. 1993; 34: 1327–1350.

13.

Emerson

Spencer-Bowdage

Bates

Relationships between self-esteem, social experiences and satisfaction with appearance: standardisation and construct validation of two cleft audit measures. Presented at The Craniofacial Society of Great Britain and Ireland's Annual Scientific Conference, “Setting a Positive Agenda in Cleft and Craniofacial Care”; April 2004; Bath, U.K.

14.

Evans

K.N.

Sie

K.C.

Hopper

R.A.

Glass

R.P.

Hing

A.V.

Cunningham

M.L.

Robin sequence: from diagnosis to development of an effective management plan. Pediatrics. 2011; 127: 936–948.

15.

Feragen

K.B.

Stock

N.M.

When there is more than a cleft: psychological adjustment when a cleft is associated with an additional condition. Cleft Palate Craniofac J. 2014; 51: 5–14.

16.

Feragen

K.B.

Stock

N.M.

Rumsey

Toward a reconsideration of inclusion and exclusion criteria in cleft lip and palate: implications for psychological research. Cleft Palate Craniofac J. 2014; 51: 569–578.

17.

Filip

Matzen

Aagenaes

Aukner

Kjoll

Hogevold

H.E.

Tonseth

Autologous fat transplantation to the velopharynx for treating persistent velopharyngeal insufficiency of mild degree secondary to overt or submucous cleft palate. J Plast Reconstr Aesthet Surg. 2013; 66: 337–344.

18.

Glynn

Fitzgerald

Earley

M.J.

Rowley

Pierre Robin sequence: an institutional experience in the multidisciplinary management of airway, feeding and serous otitis media challenges. Int J Pediatr Otorhinolaryngol. 2011; 75: 1152–1155.

19.

Goudy

Ingraham

Canady

The occurrence of velopharyngeal insufficiency in Pierre Robin sequence patients. Int J Pediatr Otorhinolaryngol. 2011; 75: 1252–1254.

20.

Han

Seruya

Preciado

Airway management in patients with Robin sequence. In: Berkowitz

, ed. Cleft Lip and Palate. New York: Springer Heidelberg; 2013: 279–286.

21.

Hanson

J.W.

Smith

D.W.

U-shaped palatal defect in the Robin anomalad: developmental and clinical relevance. J Pediatr. 1975; 87: 30–33.

22.

Izumi

Konczal

L.L.

Mitchell

A.L.

Jones

M.C.

Underlying genetic diagnosis of Pierre Robin sequence: retrospective chart review at two children's hospitals and a systematic literature review. J Pediatr. 2012; 160: 645–650 e642.

23.

Johnsen

S.L.

Rasmussen

Wilsgaard

Sollien

Kiserud

Longitudinal reference ranges for estimated fetal weight. Acta Obstet Gynecol Scand. 2006; 85: 286–297.

24.

Juliusson

P.B.

Roelants

Eide

G.E.

Moster

Juul

Hauspie

Waaler

P.E.

Bjerknes

Growth references for Norwegian children [in Norwegian]. Tidsskr Nor Laegeforen. 2009; 129: 281–286.

25.

Kandel

D.B.

Davies

Epidemiology of depressive mood in adolescents: an empirical study. Arch Gen Psychiatry. 1982; 39: 1205–1212.

26.

Klinto

Salameh

E.K.

Svensson

Lohmander

The impact of speech material on speech judgement in children with and without cleft palate. Int J Lang Commun Disord. 2011; 46: 348–360.

27.

Lavigne

J.V.

Faier-Routman

Psychological adjustment to pediatric physical disorders: a meta-analytic review. J Pediatr Psychol. 1992; 17: 133–157.

28.

Lohmander

Surgical intervention and speech outcomes in cleft lip and palate. In: Howard

Lohmander

, eds. Cleft Palate Speech. West Sussex: John Wiley & Sons, Ltd.; 2011: 55–85.

29.

The Norwegian Directorate of Health. Markestad

Halvorsen

Faglige retningslinjer for oppfølging av for tidlig fødte barn. Oslo: Sosial og helsedirektoratet. Available at http://www.helsedirektoratet.no/vp/multimedia/archive/00018/IS-19_18204a.pdf. Accessed January 7, 2015.

30.

Markestad

Halvorsen

Faglige retningslinjer for oppfølging av for tidlig fødte barn. In: Helsedirektoratet

, ed. Oslo: Seyfarth trykkerias; 2007.

31.

Martinez

Carter

J.S.

Legato

L.J.

Social competence in children with chronic illness: a meta-analytic review. J Pediatr Psychol. 2011; 36: 878–890.

32.

Meyer

A.C.

Lidsky

M.E.

Sampson

D.E.

Lander

T.A.

Liu

Sidman

J.D.

Airway interventions in children with Pierre Robin sequence. Otolaryngol Head Neck Surg. 2008; 138: 782–787.

33.

Nyberg

Westberg

L.R.

Neovius

Larson

Henningsson

Speech results after one-stage palatoplasty with or without muscle reconstruction for isolated cleft palate. Cleft Palate Craniofac J. 2010; 47: 92–103.

34.

Printzlau

Andersen

Pierre Robin sequence in Denmark: a retrospective population-based epidemiological study. Cleft Palate Craniofac J. 2004; 41: 47–52.

35.

Reid

Kilpatrick

Reilly

A prospective, longitudinal study of feeding skills in a cohort of babies with cleft conditions. Cleft Palate Craniofac J. 2006; 43: 702–709.

36.

Robin

A fall of the base of the tongue considered as new cause of nasopharyngeal respiratory impairment. Bull Acad Natl Med. 1923; 89.

37.

Robin

Glossoptosis due to atresia and hypotrophy of the mandible. Am J Dis Child. 1934; 48: 541–547.

38.

Rognerud

Strand

B.H.

Dalgard

O.S.

Mental health in Norway 1998. I. Socioeconomic differences in mental health and life style. Norsk Epidemiologi. 2002; 12: 239–248.

39.

Rohrich

R.J.

Byrd

H.S.

Optimal timing of cleft palate closure. Speech, facial growth, and hearing considerations. Clin Plast Surg. 1990; 17: 27–36.

40.

Singer

Sidoti

E.J.

Pediatric management of Robin sequence. Cleft Palate Craniofac J. 1992; 29: 220–223.

41.

Sommerlad

B.C.

Henley

Birch

Harland

Moiemen

Boorman

J.G.

Cleft palate re-repair—a clinical and radiographic study of 32 consecutive cases. Br J Plast Surg. 1994; 47: 406–410.

42.

Stransky

Basta

Solot

Cohen

Low

D.W.

Larossa

Jackson

Do patients with Pierre Robin sequence have worse outcomes after cleft palate surgery?

Ann Plast Surg. 2013; 71: 292–296.

43.

Tambs

Moum

How well can a few questionnaire items indicate anxiety and depression?

Acta Psychiatr Scand. 1993; 87: 364–367.

44.

Troland

Personality inventory for children (PIC): “Barne–MMPI”: norsk oversettelse og pilot-utprøving. Bergen: K. Troland; 1988.

45.

von Langenbeck

Die Uranoplastik mittelst Ablösung des mucös-periostealen Gaumenuberzuges. Berlin: Verlag von August Hirschwald, Unter den Linden; 1861.

46.

Wagener

Rayatt

S.S.

Tatman

A.J.

Gornall

Slator

Management of infants with Pierre Robin sequence. Cleft Palate Craniofac J. 2003; 40: 180–185.

47.

Wilhelmi

B.J.

Appelt

E.A.

Hill

Blackwell

S.J.

Palatal fistulas: rare with the two-flap palatoplasty repair. Plast Reconstr Surg. 2001; 107: 315–318.

48.

Wing

Sex ratios in early childhood autism and related conditions. Psychiatry Res. 1981; 5: 129–137.

49.

Wirt

Multidimensional Description of Child Personality. A Manual for the Personality Inventory for Children. Los Angeles: Western Psychological Services; 1981.

50.

Witt

P.D.

Myckatyn

Marsh

J.L.

Grames

L.M.

Dowton

S.B.

Need for velopharyngeal management following palatoplasty: an outcome analysis of syndromic and nonsyndromic patients with Robin sequence. Plast Reconstr Surg. 1997; 99: 1522–1529, discussion 1530–1524.