Introduction
Patients and methods
Retrospective cohort study
Baseline characteristics
Feeding and growth
Statistical analysis
Systematic literature review
Database | Search query |
---|---|
PubMed | (((“Pierre Robin Syndrome”[Mesh]) OR (pierre robin syndrome[tiab] OR pierre robin sequence[tiab] OR PRS[tiab] OR pierre robin[tiab] OR robin sequence*[tiab]))) AND (((“Feeding Behavior”[Mesh]) OR “Eating Disorders”[Mesh]) OR (feeding behavior*[tiab] OR feeding behaviour*[tiab] OR feed*[tiab] OR nutrition*[tiab] OR feeding difficult*[tiab] OR eating difficult*[tiab] OR feeding problem*[tiab] OR eating problem*[tiab] OR eating disorder*[tiab])) |
Embase | (((‘pierre robin syndrome’:ab,ti OR ‘pierre robin sequence’:ab,ti OR ‘prs’:ab,ti OR ‘pierre robin syndromes’:ab,ti OR ‘pierre robin sequences’:ab,ti) OR ‘pierre robin syndrome’/exp) AND ((‘feeding behaviour’:ab,ti OR ‘feeding behaviours’:ab,ti OR ‘feeding behavior’:ab,ti OR ‘feed’:ab,ti OR ‘feeding’:ab,ti OR ‘nutrition’:ab,ti OR ‘nutritions’:ab,ti OR ‘feeding difficulty’:ab,ti OR ‘feeding difficulties’:ab,ti OR ‘feeding problem’:ab,ti OR ‘feeding problems’:ab,ti OR ‘eating problem’:ab,ti OR ‘eating problems’:ab,ti OR ‘eating difficulty’:ab,ti OR ‘eating difficulties’:ab,ti OR ‘eating disorder’:ab,ti OR ‘eating disorders’:ab,ti) OR ‘feeding behavior’/exp. OR ‘child nutrition’/exp. OR ‘nutritional disorder’/exp. OR ‘feeding disorder’/exp)) AND [embase]/lim NOT [medline]/lim |
Cochrane library | Feeding behaviour* OR feeding behavior* OR feed* OR nutrition* OR feeding difficult* OR eating difficult* OR feeding problem* OR eating problem* OR eating disorder*:ti OR feeding behavior* OR feeding behavior* OR feed* OR nutrition* OR feeding difficult* OR eating difficult* OR feeding problem* OR eating problem* OR eating disorder*:ab AND pierre robin syndrome OR pierre robin sequence OR PRS OR pierre robin OR robin sequence*:ti OR pierre robin syndrome OR pierre robin sequence OR PRS OR pierre robin OR robin sequence*:ab |
CINAHL | (TI pierre robin syndrome OR pierre robin sequence OR PRS OR pierre robin OR robin sequence* OR AB pierre robin syndrome OR pierre robin sequence OR PRS OR pierre robin OR robin sequence*) AND (S1 AND S2) TI (pierre robin syndrome OR pierre robin sequence OR PRS OR pierre robin OR robin sequence*) OR AB (pierre robin syndrome OR pierre robin sequence OR PRS OR pierre robin OR robin sequence*) TI (Feeding behaviour* OR feeding behavior* OR feed* OR nutrition* OR feeding difficult* OR eating difficult* OR feeding problem* OR eating problem* OR eating disorder) OR AB (Feeding behaviour* OR feeding behavior* OR feed* OR nutrition* OR feeding difficult* OR eating difficult* OR feeding problem* OR eating problem* OR eating disorder) |
Google Scholar | pierre robin sequence OR pierre robin syndrome OR PRS AND feeding difficulties OR feeding problems OR nutrition |
Article | Population | Reported FD | Treatment n (%) | Duration | Growth/weight gain | Intervention | Effect on FD | ||
---|---|---|---|---|---|---|---|---|---|
NG-tube | Gastrostomy | Other | |||||||
Abadie et al. [40] | - N = 66 - Gl, RG, CP - iRS | 98 % | 34 (52 %) | – | – | 3 months | Normal in 97 % | Tr | 26 % (n = 5) bottle feeding after Tr |
Anderson et al. [35] | - N = 12 - Gl, MG, CP, ORD - iRS, sRS | 100 % | 12 (100 %) | – | – | nm | Mean of 28 g/day | NPA | nm |
Baujat et al. [17] | - N = 35 - Gl, RG, CP - iRS, sRS | 100 % | 30 (86 %) | – | – | 8.6 months (±1.7 months) | nm | nm | nm |
Butow et al. [24] | - N = 143 - Gl, MG/RG, CP - iRS, sRS | 52 % | 27 (19 %) | 2 (6 %) | Suction and drinking plate (n = 134, 94 %) | nm | nm | Suction and drinking plate | 26 % (n = 35) NG-tube feeding or gastrostomy needed |
Cruz et al. [41] | - N = 43 - Gl, MG, CP - iRS, sRS | Nm | 5 (11 %) | 12 (26 %) | – | NG-tube ≥3 months | nm | Tr/TLA | nm |
Daniel et al. [42] | - N = 39 - Gl, MG, ORD - iRS, sRS | 82 % | 32 (82 %) | – | – | nm | 3230 ± 590 g (birth) 8890 ± 1290 g (12 months) | MDO, NPA, CPAP | nm |
Evans et al. [43] | - N = 115 - Gl, RG/MG, CP, ORD - iRS, sRS | 95 % | 27 (23 %) | 49 (43 %) | Nipple (n = 40, 35 %) | nm | nm | NPA, Tr, TLA | nm |
Glynn et al. [30] | - N = 69 - Gl, MG, CP - iRS, sRS | 70 % | 48 (70 %) | 2 (3 %) | – | 3 months (3 weeks–6 months) | nm | NPA, Tr, TLA | nm |
Gozu et al. [44] | - N = 20 - Gl, MG, CP - iRS, sRS | 60 % | 12 (60 %) | – | – | nm | nm | MDO | nm |
Hamdi et al. [45] | - N = 30 - CP, ORD, FD - iRS, sRS | 100 % | 30 (100 %) | – | – | 57 days (1–120 days) | nm | TLA, Tr, removable palatal appliance | nm |
Li et al. [46] | - N = 82 - Gl, MG, CP - iRS, sRS | Nm | 46 (42 %) | – | Obturator plate/CP bottles (n = 36, 44 %) | nm | nm | NPA ,TLA, Tr | nm |
Lidsky et al. [47] | - N = 82 - Gl, MG, CP - iRS, sRS | Nm | – | 17 (25 %) | – | nm | nm | Tr, MDO | Reduction of need for feeding intervention in iRS |
Maas et al. [48] | - N = 151 - MG/RG and ORD/Gl/FD/snoring/hypoxemia/failure to thrive/syndrome or CP - iRS, sRS | 89 % | 76 (55 %); data only provided at time of discharge, after a mean of 19 (11–38) days | – | Feeding plate (n = 56, 37 %) | nm | Decrease of 0.59 (1.08–0.11) in SD score for weight, during admission | Orthodontic plate therapy, CPAP, Tr, TLA, mandibular traction, functional therapy (e.g., Castillo Morales) | Infants with pre-epiglottic baton plate demonstrated better weight gain during admission |
Meyer et al. [49] | - N = 74 - Gl, MG, CP - iRS, sRS | 50 % | 19 (26 %) | 18 (24 %) | – | nm | nm | Tr, MDO, NPA | nm |
Smith et al. [50] | - N = 60 - Gl, MG/RG, CP and/or ORD - iRS, sRS | 62 % | 17 (28 %) | 20 (33 %) | – | 26 % 0–3 months, 31 % 4–18 months, 43 % >18 months | Successful oral diet in 89 % of patients after 3 years FU | Tr or MDO | nm |
Stubenitsky et al. [51] | - N = 44 - Gl, MG/RG, CP, ORD - iRS | 100 % | 30 (68 %) | 1 (2 %) | Reflux therapy (n = 27, 61 %) | nm | Mean weight gain 722 g in 4 weeks | NPA | nm |
van den Elzen et al. [52] | - N = 74 - MG, CP, and/or Gl - iRS, sRS | 68 % | 30 (41 %) | 2 (3 %) | Central venous line (n = 1, 1 %) | nm | Significant number of infants (24 %, p = 0.002) with body weight < P10 at age 6–24 months compared with healthy controls | Tr | nm |
van Lieshout et al. [53] | - N = 59 - MG/RG and ORD - iRS, sRS | 47 % | 25 (34 %) | 3 (4 %) | – | nm | nm | Tr, MDO | nm |
Vatlach et al. [5] | - N = 82 - MG/RG with at least: ORD, Gl, FD, or CP - iRS, sRS | 83 % | 40 (49 %) | – | Haberman feeder (n = 16, 20 %); regular nipple (n = 21, 26 %) | nm | SDS of weight decreased from −0.72 at admission to −1.46 at discharge (p < 0.05) | Tr, MDO, CPAP, NPA, orthodontic plate therapy, TLA, functional therapy (e.g. Castillo Morales) | nm |
Wagener et al. [54] | - N = 22 - MG, Gl, ORD, CP - iRS, sRS | 100 % | 22 (100 %) | – | – | 4 months (1 week–11 months) | 56 % ↑ weight; 44 % ↓ weight | NPA | nm |
Results
Baseline characteristics
Variable | Study group (RS) n (%) | Control group (iCPO) n (%) |
p value* | |
---|---|---|---|---|
Total number of patients | 69 | 64 | ||
Sex | Male | 32 (46 %) | 23 (36 %) | 0.22 |
Female | 37 (54 %) | 41 (64 %) | ||
Gestational age | <37 weeks | 8 (12 %) | 7 (11 %) | 0.91 |
≥37 weeks | 61 (88 %) | 57 (89 %) | ||
Birth weight (g) | Mean = 3217 SD = 669 | Mean = 3302 SD = 556 | 0.44 | |
Grade of CPa
| 1. Submucous cleft or bifid uvula | 3 (4 %) | 9 (14 %) | <0.001 |
2. Soft palate only | 10 (15 %) | 27 (42 %) | ||
3. Soft palate and segment of hard palate | 38 (56 %) | 18 (28 %) | ||
4. Total palate up to incisive foramen | 17 (25 %) | 10 (16 %) | ||
Associated disorders | Isolated RS 32 (46 %) Non-isolated RS 37 (54 %) | 0.06 | ||
Stickler syndrome | 9 | |||
Treacher Collins syndrome | 2 | |||
Spondyloepiphyseal dysplasia | 1 | |||
4q deletion syndrome | 1 | |||
Van der Woude syndrome | 1 | |||
Osteopathia striata with cranial sclerosis | 1 | |||
Goldberg–Shprintzen syndrome | 1 | |||
Yunis–Varon syndrome | 1 | |||
Auriculo-condylar syndrome | 1 | |||
Hemifacial microsomia | 1 | |||
Other | 18 |
FD and NG-tube feeding
Variable | Study group (RS) | Control group (iCPO) |
p value* | |
---|---|---|---|---|
Total number of patients | 69 | 64 | ||
With
feeding difficulties
| 63 (91 %) | 38 (72 %) | 0.004 | |
Sex | Male | 27 (43 %) | 15 (40 %) | 0.74 |
Female | 36 (57 %) | 23 (61 %) | ||
Gestational age | <37 weeks | 7 (11 %) | 5 (13 %) | 0.76 |
≥37 weeks | 56 (89 %) | 33 (87 %) | ||
Birth weight (g) | Mean = 3237 SD = 644 | Mean = 3289 SD = 586 | 0.68 | |
Grade of CP | 1. Submucous cleft or bifid uvula | 2 (3 %) | 3 (8 %) | <0.001 |
2. Soft palate only | 9 (15 %) | 19 (50 %) | ||
3. Soft palate and segment of hard palate | 37 (60 %) | 10 (26 %) | ||
4. Total palate up to incisive foramen | 14 (23 %) | 6 (16 %) | ||
Associated disorders | Non-isolated RSb
| 36 (57 %) | NA | NA |
Isolated RS | 27 (43 %) | |||
With NG-tube feeding
| 55 (80 %) | 12 (19 %) | <0.001 | |
NG-tube feeding duration (days) | Median = 59.00 Mean rank = 33.13 | Median = 9.56 Mean rank = 9.72 | <0.001 | |
Sex | Male | 24 (44 %) | 7 (58 %) | 0.36 |
Female | 31 (56 %) | 5 (42 %) | ||
Gestational age | <37 weeks | 7 (13 %) | 4 (33 %) | 0.08 |
≥37 weeks | 48 (87 %) | 8 (67 %) | ||
Birth weight (g) | Mean = 3217 SD = 661 | Mean = 3039 SD = 733 | 0.41 | |
Grade of CPa
| 1. Submucous cleft or bifid uvula | 1 (2 %) | 0 (0 %) | 0.23 |
2. Soft palate only | 9 (17 %) | 5 (42 %) | ||
3. Soft palate and segment of hard palate | 32 (59 %) | 4 (33 %) | ||
4. Total palate up to incisive foramen | 12 (22 %) | 3 (25 %) | ||
Associated disorders | Non-isolated RSb
| 32 (58 %) | NA | NA |
Isolated RS | 16 (42 %) |
Growth
Variable effects on growtha
| EMM (g) | SE | 95 % CI (upper bound–lower bound) |
p value* | |
---|---|---|---|---|---|
Time points 1–9 (birth to 24 months of age) | iCPO | 5620 | 96 | 5263–5678 | 0.008 |
RS | 5261 | 95 | 5240–5581 | ||
Time points 1–4 (birth to 6 months of age) | iCPO | 3805 | 65 | 3676–3934 | 0.044 |
RS | 3619 | 65 | 3471–3746 | ||
Time points 5–9 (9 to 24 months of age | iCPO | 9833 | 143 | 9551–10,114 | 0.026 |
RS | 9390 | 138 | 9119–9661 | ||
Time points 1–9b (birth to 24 months of age) | iCPO | 5588 | 106 | 5380–5796 | 0.030 |
RS | 5268 | 112 | 5047–5490 | 5268 | |
Feeding difficultiesc, f
| Yes | 6902 | 87 | 6147–6488 | 0.467 |
No | 6767 | 192 | 6094–6849 | ||
NG-tube feedingc, f
| Yes | 6584 | 189 | −396–346 | 0.893 |
No | 6559 | 189 | −346–396 | ||
Grade of CPc, f
| 1. Submucous cleft or bifid uvula | 5468 | 237 | 5019–5953 | 0.308 |
2. Soft palate only | 5540 | 130 | 5283–5797 | ||
3. Soft palate and segment of hard palate | 5489 | 107 | 5278–5699 | ||
4. Total palate up to incisive foramen | 5198 | 146 | 4911–5486 | ||
Associated disordersd, f
| Isolated RS | 6479 | 151 | 6181–6777 | 0.517 |
Non-isolated RSe
| 6621 | 154 | 6317–6825 | ||
Intervention typed, f
| Surgical | 6902 | 203 | 6504–7301 | 0.052 |
Conservative | 6484 | 179 | 6132–6836 | ||
Surgical intervention subtyped, f
| MDO | 7965 | 188 | 7587–8344 | 0.007 |
TLA | 7720 | 336 | 7049–8391 | ||
Tr | 8765 | 223 | 8317–9213 | ||
TLA + Tr | 6423 | 752 | 4920–7927 | ||
MDO + Tr | 8383 | 412 | 7555–9210 |
Interventions in the study group
Conservative | Surgical |
p value* | Surgical intervention subtype | |||||||
---|---|---|---|---|---|---|---|---|---|---|
MDO | TLA | Tr | TLA + Tr | MDO + Tr |
p value* | |||||
Total number of patients | 40 (60 %) | 27 (40 %) | 14 (52 %) | 3 (11 %) | 7 (26 %) | 1 (4 %) | 2 (7 %) | |||
Feeding difficulties, yes | 34 (85 %) | 27 (100 %) | 0.04 | 14 (100 %) | 3 (100 %) | 7 (100.0 %) | 1 (100.0 %) | 2 (100 %) | n.a. | |
NG-tube feeding, yes | 28 (63 %) | 25 (93 %) | 0.03 | 14 (100 %) | 3 (100 %) | 6 (86 %) | 0 (0 %) | 2 (100 %) | 0.006 | |
NG-tube duration (days)a
| Median = 21.0 | Mean = 72.0 | 0.011 | Median = 36.5 | Median = 183.0 | Median = 461.5 | NC | Median = 38.0 | 0.003 | |
Mean rank = 19.2 | Mean rank = 29.4 | Mean rank = 8.9 | Mean rank = 18.3 | Mean rank = 21.2 | NC | Mean rank = 9.0 | ||||
Sex | Male | 18 (45 %) | 12 (44 %) | 0.96 | 6 (43 %) | 1 (33 %) | 3 (43 %) | 0 (0 %) | 2 (100 %) | 0.48 |
Female | 22 (55 %) | 15 (56 %) | 8 (59 %) | 2 (67 %) | 4 (57 %) | 1 (100 %) | 0 (0 %) | |||
Gestational age | <37 weeks | 5 (13 %) | 2 (7 %) | 0.50 | 0 (0 %) | 0 (0 %) | 1 (14 %) | 1 (100 %) | 0 (0 %) | 0.006 |
≥37 weeks | 35 (88 %) | 25 (93 %) | 14 (100 %) | 3 (100 %) | 6 (86 %) | 0 (0 %) | 2 (100 %) | |||
Birth weight (g) | Mean = 3139 | Mean = 3409 | 0.10 | Median = 3205 | Median = 3785 | Median = 3920 | Median = 2680 | Median = 3287 | 0.19 | |
SD = 713 | SD = 521 | Mean rank = 12.11 | Mean rank = 16.33 | Mean rank = 19.07 | Mean rank = 2.5 | Mean rank = 11.75 | ||||
Grade of CPb
| 1. Submucous cleft or bifid uvula | 3 (8 %) | 0 (0 %) | 0.45 | 0 (0 %) | 0 (0 %) | 0 (0 %) | 0 (0 %) | 0 (0 %) | 0.13 |
2. Soft palate only | 5 (13 %) | 5 (19 %) | 4 (31 %) | 1 (33 %) | 0 (0 %) | 0 (0 %) | 0 (0 %) | |||
3. Soft palate and segment of hard palate | 21 (53 %) | 15 (58 %) | 5 (39 %) | 2 (67 %) | 7 (100 %) | 0 (0 %) | 1 (50 %) | |||
4. Total palate up to incisive foramen | 11 (28 %) | 6 (23 %) | 4 (31 %) | 0 (0 %) | 0 (0 %) | 1 (100 %) | 1 (50 %) | |||
Associated disorders | Isolated | 21 (53 %) | 11 (41 %) | 0.35 | 4 (29 %) | 0 (0 %) | 4 (57 %) | 1 (100 %) | 2 (100 %) | 0.089 |
Non-isolatedc
| 19 (48 %) | 16 (59 %) | 10 (71 %) | 3 (100 %) | 3 (43 %) | 0 (0 %) | 0 (0 %) |