Fraser RK, Bourke HM, Broughton NS, Menelaus MB. Unilateral dislocation of the hip in spina bifida.

A long-term
follow-up. J Bone Joint Surg Br. 1995 Jul;77(4):615-9. PMID: 7615608.

1) Unilateral hip dislocation in spina bifida is associated with leg-length inequality and increased pressure through the lower
ischial tuberosity. Leg-length inequality impairs but does not preclude walking.

Thompson RM, Foley J, Dias L, Swaroop VT. Hip Status and Long-term Functional Outcomes in Spina Bifida. J Pediatr Orthop.
2019 Mar;39(3):e168-e172. doi: 10.1097/BPO.0000000000001266. PMID: 30300276.

Long-term outcomes are not associated with hip status in adult patients with myelomeningocele. Functional outcomes are more
closely correlated with neurological level and hip range of motion. These results suggest efforts to keep myelomeningocele hips
reduced are likely without functional benefit and should be avoided in favor of maintaining motion with contracture release as
needed.

Swarup I, Talwar D, Howell LJ, Adzick NS, Horn BD. Orthopaedic outcomes of prenatal versus postnatal repair of
myelomeningocele. J Pediatr Orthop B. 2020 Nov 6:10.1097/BPB.0000000000000827. doi: 10.1097/BPB.0000000000000827.
Epub ahead of print. PMID: 33165214; PMCID: PMC8099935.

The rate of LLD was lower in the prenatal repair group at 12 and 30 months (7 vs. 16% at 30 months, P = 0.047). There were no
differences in rates of scoliosis, kyphosis, hip abnormality, clubfoot or tibial torsion between patients treated with prenatal or
postnatal repair.

Rális ZA, Rális HM, Randall M, Watkins G, Blake PD. Changes in shape, ossification and quality of bones in children with spina
bifida. Dev Med Child Neurol Suppl. 1976;(37):29-41. doi: 10.1111/j.1469-8749.1976.tb04278.x. PMID: 828114.

En las tibias de 10s niiios con espina bifida con paralisis, se ha116 que el area total de la
zona cortical del hueso, su espesor, el numero de sistemas de Havers y el n6mero de grandes
cavidades de remodelaci6n estan todas disminuidas.

La cantidad total de tejido osteoide del hueso de la espina biiida paralizada esta aumentado. Este retraso en la mineralizaci6n de
la matriz 6sea recientemente depositada podria conducir a un reblandecimiento de la matriz neoosea y de las regiones
subepifisarias ricas en tejido osteoide. Este ‘raquitismo paralitico’, juntamente con la disminucion de la masa total del hueso,
probablemente podria ser la causa de la frecuencia de fracturas en estas regiones en niiios con espina bifida.
The diminished square area, cortical thickness and circumference all indicate bone thinning and atrophy in the spina-bifida child
and are in agreement with the literature (Sharrard 1971). The diminished number of Haversian systems, and especially of bone
remodelling cavities, indicates the lack of remodelling activity, which of course would result in retardation of further growth, in
addition to the rarefaction of bone which occurs in the paralysed, tenotomised and immobilised limbs (Whedon and Shorr 1957,
Geiser and
Trueta 1958, Sharrard 1971)
Liptak GS, El Samra A. Optimizing health care for children with spina bifida. Dev Disabil Res Rev. 2010;16(1):66-75. doi:
10.1002/ddrr.91. PMID: 20419773.

Los niños con espina bífida tienden a ser más bajos que sus pares. Por una razón la disfunción de la medula espinal lidia con el
crecimiento anormal de los músculos y huesos en los miembros inferiores.

La estatura baja ha sido encontrada en casi el 50% de los niños con espina bífida, a un nivel mas alto de lesión ,la mayor estatura
baja.Rosemblum encontró que la estatura baja ocurre en el 80% de los niños con lesiones en L3 o arriba y en un 45% de niños
con lesiones L4-L5.Una alternativa para medir la altura es usar el largo de los brazos como un aproximado.

Algunos niños con espina bífida que son bajos tienen deficiencia de la hormona de crecimiento.Aunque esta deficiencia es difícil
de medir ,el largo de los brazos,el factor de crecimiento 1 y similar a la insulina niveles de proteína 3 de unión al factor de
crecimiento se utilizan como parámetros de detección de GH deficiencia

Mukherjee S, Pasulka J. Care for Adults with Spina Bifida: Current State and Future Directions. Top Spinal Cord Inj Rehabil. 2017
Spring;23(2):155-167. doi: 10.1310/sci2302-155. PMID: 29339892; PMCID: PMC5672883.

Bone health :It has been reported that bone mineral density is reduced in patients with SB compared to typically developing
peers.62,63 One study found that children and young adults with SB have decreased levels of vitamin D, and hypophosphatemia
as a result, when compared to the general population

Valtonen KM, Goksör LA, Jonsson O, Mellström D, Alaranta HT, Viikari-Juntura ER. Osteoporosis in adults with
meningomyelocele: an unrecognized problem at rehabilitation clinics. Arch Phys Med Rehabil. 2006 Mar;87(3):376-82. doi:
10.1016/j.apmr.2005.11.004. PMID: 16500172.
It can be assumed that patients with meningomyelocele are at potential risk to develop osteoporosis at a younger age because
of impaired walking ability and subsequent low physical loading of the lower limbs. Furthermore, because of neurogenic bladder
dysfunction, these patients are more prone to develop renal failure, which is a known risk factor for osteoporosis

However, it has been reported that BMD in children and young adults with meningomyelocele falls 1 to 2 standard deviations
(SDs) below that of the normative population

Our results suggest that besides low degree or lack of loading of the extremities, osteoporosis can be caused by neurogenic and
metabolic mechanisms in this patient group

Impairments in ambulation and functioning may cause subjects with meningomyelocele to spend more time inside and become
even more easily institutionalized. This may result in diminished exposure to sunshine, which in turn may lead to reduced
production of vitamin D in the body and disturbance of calcium metabolism

Trinh A, Wong P, Brown J, Hennel S, Ebeling PR, Fuller PJ, Milat F. Fractures in spina bifida from childhood to young adulthood.
Osteoporos Int. 2017 Jan;28(1):399-406. doi: 10.1007/s00198-016-3742-0. Epub 2016 Aug 24. PMID: 27553445.

Precocious/earlypuberty is well recognised in patients with spina bifida whohave hydrocephalus, with reported rates of 10–50 %
[32, 33].It is more common in girls than boys with spina bifida whichmirrors the prevalence in the general population
[34].Precocious puberty leads to early bone maturation and shorterfinal height, with apparent higher bone mass for
chronologicalage, but no difference if adjusted for bone age

O'Neil J, Fuqua JS. Short stature and the effect of human growth hormone: Guidelines for the care of people with spina bifida. J
Pediatr Rehabil Med. 2020;13(4):549-555. doi: 10.3233/PRM-200710. PMID: 32986629; PMCID: PMC7838964.

 The short statureis primarily due to disproportionate short growth ofthe lower body segments.
 The etiology of short stature among children, adoles-cents, and adults with spina bifida is multifactorial andis a function
of inadequate innervation, lack of use ofmuscles, hip dysplasia, scoliosis, and shortened lowerbody segments
 Prior work hasdemonstrated that hydrocephalus impacts the secretionof pituitary hormones responsible for growth
and pu-bertal development
 Multiple studies have demon-strated an increased prevalence of growth hormone de-ficiency (GHD) in children with
spina bifida
 Per-rone et. al. noted abnormal hypothalamic-pituitary func-tion in children and adolescents with myelomeningo-cele
[6]; it is estimated that approximately 30% of chil-dren with spina bifida have GHD
 This is importantbecause GHD contributes to abnormal body composi-tion in these individuals that is exacerbated by
inactiv-ity, caloric intake, and other factors, increasing the riskof obesity.
 Adult height is known to be limited in those withspina bifida. One study demonstrated that the meanadult height of
men with spina bifida was 152.1±13 cm, and in women adult height averaged 141.9±12 cm, far below the average
height for healthy adultsof 176 cm and 163.5 cm, respectively
 Measurement of arm span avoids the issues of ab-normal lower segment growth and scoliosis and corre-lates well with
height in healthy children. Arm span hasbeen used successfully to identify children with spinabifida who have GHD
 It is also important to consider the pu-bertal status of the child. Central precocious puberty iscommon in children with
spina bifida, especially girls.
 The pubertal growth spurt also occurs early in childrenwith precocious puberty
 Endocrine test-ing usually includes measurement of thyroid functionand screening tests of GH secretion such as insulin-
like growth factor-1 (IGF-1) and insulin-like growthfactor binding protein-3 (IGFBP-3).

Children with GHD also have clear decreases in mus-cle mass

Almutlaq N, O'Neil J, Fuqua JS. Central precocious puberty in spina bifida children: Guidelines for the care of people with spina
bifida. J Pediatr Rehabil Med. 2020;13(4):557-563. doi: 10.3233/PRM-200728. PMID: 33325409; PMCID: PMC7838954.

 Central precocious puberty (CPP) is defined as thepremature activation of the hypothalamic-pituitary-gonadal (HPG)
axis, which leads to the early appear-ance of secondary sexual characteristic
 Traditionally,CPP is considered to be the onset of pubertal changesbefore age eight years in girls and nine years in
boys.However, to be more precise, it is the onset of pubertalchanges at an age younger than the accepted norm forthat
individual’s ethnicity and race.
 In both sexes, growth acceleration leads to increasedheight percentiles and advanced bone age (often morethan 2
standard deviations greater than chronologicage). This acceleration of skeletal maturation may leadto permanent
loss of adult height.
 Children with myelomeningocele (MMC) have anincreased incidence of CPP. Previous studies reportedthat the
prevalence of CPP in girls with MMC is ashigh as 50%. In contrast, estimates in boys range from10–30%
  While the exact causative mechanismin children with MMC is not known, several studiesdemonstrated an association
with hydrocephalus, whichmay alter HPG axis function Additionally, evi-dence shows that increased perinatal
intracranial pres-sure and brainstem malformations, i.e. Chiari II mal-formations, are influential prognostic factors for
thedevelopment of CPP.
 Historically, puberty wasbelieved to start between 8–13 years in girls and 9–14 years in boys
 Findings suggesting pathological CPP include thepresence of pubic hair and breast enlargement/testicularenlargement
at the same time, advanced stages of puber-tal development, increased linear growth, or advancedskeletal maturation.

Klauschie J, Rose SR. Incidence of short stature in children with hydrocephalus. J Pediatr Endocrinol Metab. 1996 Mar-
Apr;9(2):181-7. PMID: 8887141.

Medical problems with increased risk of SS included spina bifida or meningomyelocele, Dandy Walker syndrome, brain tumor,
cerebral palsy, epilepsy, impaired vision, mental retardation, and pulmonary disorders.

Children with hydrocephalus are at increased risk for short stature, slow growth velocity, or accelerated growth. Observation of
premature entry to puberty, or accelerated or slow growth velocity should prompt an endocrine evaluation.

Chadwick KP, Mueske NM, Horenstein RE, Shefelbine SJ, Wren TAL. Children with myelomeningocele do not exhibit normal
remodeling of tibia roundness with physical development. Bone. 2018 Sep;114:292-297. doi: 10.1016/j.bone.2018.07.001. Epub
2018 Jul 4. PMID: 29991457; PMCID: PMC7173944.

The results in this study indicate that ambulation is important throughout early physical development in order to more closely
attain normal bone cross-sectional shape.

Our results also show that less ambulatory children with MM have rounder cross-sections than more ambulatory children with
MM and TD children.

These differences likely result from a lack of the loading needed to stimulate bone accretion, modeling, and remodeling. Without
normal loading, the bone fails to develop, and an immature morphology and mechanical structure are retained.