{
 "topic": "Myelomeningocele",
 "slug": "myelomeningocele",
 "category_id": 15132,
 "passage_count": 14,
 "source_chars": 11523,
 "enough_material": true,
 "references": [
  {
   "title": "Kliegman R. Nelson Textbook of Pediatrics 2-Volume Set 22ed 2024",
   "author": null,
   "pages": [
    3599,
    3600
   ]
  },
  {
   "title": "Zitelli and Davis' Atlas of Pediatric Physical Diagnosis: Expert Consult - Online",
   "author": null,
   "pages": [
    893
   ]
  },
  {
   "title": "Textbook of Neonatal Resuscitation (8th ed)",
   "author": null,
   "pages": [
    260
   ]
  },
  {
   "title": "MedStudy Pediatrics Core 11th Edition 2024-2025",
   "author": null,
   "pages": [
    437
   ]
  },
  {
   "title": "Gomella's Neonatology: Management, Procedures, On-Call Problems, Diseases, and Drugs, Eighth Edition",
   "author": "Tricia Lacy Gomella, Fabien G. Eyal and Fayez Bany-Mohammed",
   "pages": [
    1043
   ]
  },
  {
   "title": "Diagnostic Imaging: Pediatrics",
   "author": "A. Carlson Merrow Jr. MD",
   "pages": [
    1130,
    1134
   ]
  },
  {
   "title": "Cover",
   "author": "Vitalsource Download",
   "pages": [
    8394
   ]
  },
  {
   "title": "Pediatric Decision-Making Strategies",
   "author": "Pomeranz, Albert J.",
   "pages": [
    122
   ]
  }
 ],
 "passages": [
  {
   "source": "Kliegman R. Nelson Textbook of Pediatrics 2-Volume Set 22ed 2024, p. 3600",
   "text": "Other CNS anomalies are associated with myelomeningocele, including cortical dysplasias, polymicrogyria, callosal abnormalities, and cerebellar abnormalities. Long- term complications include tethered cord, usually secondary to scar tissue at the surgical repair site, and hydromyelia, which is CSF buildup in the central canal of the spinal cord caused by worsening hydrocephalus. ## **TREATMENT (SEE ALSO CHAPTER 754)** Management and supervision of a child and family with a myelomeningocele require a multidisciplinary team approach, including surgeons, other physicians, and therapists, with one individual (often a pediatrician) acting as the advocate and coordinator of the treatment program."
  },
  {
   "source": "Zitelli and Davis' Atlas of Pediatric Physical Diagnosis: Expert Consult - Online, p. 893",
   "text": "## **Spina Bifida Cystica (Myelomeningocele)** The myelodysplasias are a group of congenital deformities that result from defective closure of the caudal portion of the neural tube during embryonic development. When the failure of closure involves only the posterior vertebral elements, it is termed a _meningocele;_ when it involves neural and vertebral elements, it is called a _myelomeningocele_ . In patients with myelomeningocele, the loss of neurologic function that results from abnormal cord and spinal root development has dire consequences for the developing musculoskeletal system. Affected children often require neurosurgical closure of the defect at birth to allow healing and prevent infection, and most need a ventriculoperitoneal shunt to prevent or control hydrocephalus. Because of lack of bladder innervation and control, careful urologic follow-up is necessary as well."
  },
  {
   "source": "Textbook of Neonatal Resuscitation (8th ed), p. 260",
   "text": "What special care is required for a newborn with myelomeningocele (spina bifida)? Myelomeningocele is a type of neural tube defect that affects the spinal cord and vertebrae (Figure 10.16). It most commonly involves the lower back (lumbar area). The defect occurs during the first few weeks of fetal development when the precursor of the spinal cord, the neural tube, does not completely close. A sac of fluid containing part of the spinal cord and nerves may protrude through an opening 257"
  },
  {
   "source": "MedStudy Pediatrics Core 11th Edition 2024-2025, p. 437",
   "text": "tations decrease. Location in the upper thoracic or cervical areas actually cause minimal neurologic manifestations. Example 1: A low, sacral-region myelomeningocele is likely to cause bowel and bladder incontinence and numbness of the perineal area but no motor dysfunction. Example 2: With a high lumbar myelomeningocele, the infant is likely to have bowel and bladder incontinence as in example 1 but lower extremity flaccid paralysis with no deep tendon reflexes, no response to touch or pain in the lower extremities, and postural abnormalities (e.g., clubfeet, hip subluxation) due to lack of appropriate movement in utero. Myelomeningoceles cause a downward displacement of the brain stem and cerebellar tonsils down into the spinal column\u2014a condition known as Chiari malforma- tion Type II (a.k.a. Amold-Chiari malformation; see Chiari Malformation on page 12-6). This, in turn, causes an obstruction to normal CSF flow, resulting in hydrocepha- lus ~ 85% of the time. See Hydrocephalus on page 12-4. Treatment"
  },
  {
   "source": "Gomella's Neonatology: Management, Procedures, On-Call Problems, Diseases, and Drugs, Eighth Edition, p. 1043",
   "text": "**3. Myelomeningocele.** Traditionally, postnatal surgery and management by a multidisciplinary team are the treatment for myelomeningocele. A multidisciplinary team approach, including a neonatologist, neurosurgeon, geneticist, genetic counselor, urologist, orthopedic surgeon, social worker, physiotherapist, and primary care physician, is necessary. The Management of Myelomeningocele Study (MOMS) was published in 2011 and found that prenatal surgery for myelomeningocele reduced the need for placement of a cerebrospinal fluid shunt and improved motor outcomes at 30 months but was associated with maternal and fetal risks. A Cochrane review in 2014 assessed evidence to be insufficient to recommend in utero repair for unborn babies with spina bifida; several centers in the United States continue to perform fetal surgery, and the MOMS study is ongoing."
  },
  {
   "source": "Diagnostic Imaging: Pediatrics, p. 1134",
   "text": "2. Messing-J\u00fcnger M et al: Primary and secondary management of the Chiari II malformation in children with myelomeningocele. Childs Nerv Syst. 29(9):1553-62, 2013 3. Barkovich AJ et al: Pediatric Neuroimaging. 5th ed. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins. 863-70, 2012 4. Adzick NS et al: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 364(11):993-1004, 2011 5. Bowman RM et al: Spina bifida outcome: a 25-year prospective. Pediatr Neurosurg. 34(3):114-20, 2001 - Etiology - \u25cbFailure of neural tube closure in 3rd gestational week \u2013 Likely multifactorial: Folate deficiency, toxins, genetics **1109** Lipomyelomeningocele ## **KEY FACTS** ## **TERMINOLOGY** - Subtype of closed (skin-covered) spinal dysraphism - \u25cbNeural placode of elongated spinal cord attaches to lipomatous mass - \u25cbNeural, fatty, & meningeal components extend through dysraphic posterior elements ## **IMAGING**"
  },
  {
   "source": "Cover, p. 8394",
   "text": "## **Cognitive Development** The brain development of children with myelomeningocele is affected by the presence of the Chiari II malformation. Cognition can be further affected by complications of hydrocephalus and by shunt infections, especially those that occur during infancy. Most individuals with myelomeningoceles have cognition"
  },
  {
   "source": "Kliegman R. Nelson Textbook of Pediatrics 2-Volume Set 22ed 2024, p. 3600",
   "text": "On exam, the myelomeningocele is generally readily apparent as a saclike cystic structure covered by a thin layer of partially epithelialized tissue or an exposed flat neural placode without overlying tissues (Fig. 631.2C). When a cyst or membrane is present, remnants of neural tissue are visible beneath the membrane, which occasionally ruptures and leaks CSF. Examination of the infant generally shows a flaccid paralysis of the lower extremities, an absence of deep tendon reflexes, a lack of response to touch and pain, and a high incidence of lower- extremity deformities (clubfeet, ankle and/or knee contractures, and subluxation of the hips). Some children have constant urinary dribbling and a relaxed anal sphincter. Other children do not leak urine and in fact have a highpressure bladder and sphincter dyssynergy. Myelomeningocele above the midlumbar region tends to produce lower motor neuron signs because of abnormalities and disruption of the conus medullaris and higher spinal cord structures."
  },
  {
   "source": "Pediatric Decision-Making Strategies, p. 122",
   "text": "2[Examples of other infections that may cause splenomegaly ] include spirochetal, rickettsial, parasitic, fungal, mycobacterial, and protozoal (i.e., malaria). Congenital infections (e.g., CMV infection, herpes simplex virus infection, toxoplasmosis, rubella) also result in splenomegaly. > [Viral infection is the most common cause of splenomeg-] 3 aly in children. In the absence of hemolysis and other worrisome symptoms, a period of observation is acceptable, with additional workup recommended if the splenomegaly persists for 4 to 6 weeks. In the presence of a mononucleosis syndrome, identifying the cause may be reassuring to the physician or family but will not affect the management of the disorder. 106 Chapter 28 u Splenomegaly 107 ![](/tmp/pdf-images/pdf-0123-01.png) **----- Start of picture text -----**<br> SPLENOMEGALY<br>**----- End of picture text -----**<br> ![](/tmp/pdf-images/pdf-0123-02.png)"
  },
  {
   "source": "Diagnostic Imaging: Pediatrics, p. 1134",
   "text": "- Many types: Lipomyelomeningocele, lipomyelocele, myelocystocele, meningocele ## **Postoperative Pseudomeningocele** - History & clinical exam allow for differentiation ## **PATHOLOGY** ## **General Features** ## **CLINICAL ISSUES** ## **Presentation** - Most common neurologic deficits: Lower extremities, bowel & bladder dysfunction - \u25cbHigher level spine defect correlates with greater motor & somatosensory deficits - ~ 80-85% have hydrocephalus requiring CSF diversion, though lower incidence reported with fetal repair - \u2022 Latex hypersensitivity common (~ 1/3) ## **Natural History & Prognosis** - Stable postoperative motor deficit seen in majority (~ 73%) - \u25cbNeurological deterioration suggests complication - Tethering by scar (clinical diagnosis of exclusion) most common; symptoms of retethering in ~ 1/3 - Imaging to look for other causes - \u25a1Syringohydromyelia \u2192 disassociated sensory loss \u25a1Cervical myelopathy \u2192 spasticity & weakness of upper extremities"
  },
  {
   "source": "Kliegman R. Nelson Textbook of Pediatrics 2-Volume Set 22ed 2024, p. 3599",
   "text": "## **ETIOLOGY** The etiology of myelomeningocele remains incompletely understood, but as with all neural tube closure defects, a genetic predisposition exists. The risk of recurrence after one affected child is 3\u20134% and increases to 10% with two prior affected children. Both epidemiologic evidence and the familial aggregation studies indicate polygenic risk factors contribute to the etiology of NTDs."
  },
  {
   "source": "Ghai Essential Pediatrics, 9e (Vinod K Paul, Arvind Bagga), p. 608",
   "text": "[Figure OCR, page 608, figure 1] Childhood Malignancies Fig. 21.5: (a) A 6-year-old boy with chronic myeloid leukernia presenting with fever, anemia, significant splenomegaly and hepatomegaly of 6 months duration. (b) A 1-year-old boy presented with fever, rashes, anemia, significant splenomegaly and hepatomegaly. He was diagnosed as juvenile myelo- monocytic leukemia crisis include fever, night sweats, rapid weight loss, splenic pain, lymphadenopathy, cutaneous infiltration, bleeding or infection. Leukocytosis is present in all cases and 80% patients have leukocyte counts >100,000/cu mm. The differential Category 1 (all must be present) count showsall forms of myeloid cells from promyelocytes, myelocytes and metamyelocytes to polymorphonuclear leukocytes; basophilia is common. Genetic testing for the Philadelphia (Ph) chromosome in conjunction with marrow morphology allows confirmation of the diagnosis. Treatment The aim of treatment is to control increasing white cell"
  },
  {
   "source": "2021_Fleisher_&_Ludwig's_Textbook_of_Pediatric_Emergency_Medicine.epub",
   "text": "Principi N, Esposito S. Kingella kingae infections in children. BMC Infect Dis 2015;15:260. doi:10.1186/s12879-015-0986-9. Roderick MR, Shah R, Rogers V, et al. Chronic recurrent osteomyelitis (CRMO)\u2014advancing the diagnosis. Pediatr Rheumatol Online J 2016;14(1):47. Safdar NM, Rigsby CK, Iyer RS, et al. ACR Appropriateness Criteria\u00ae acutely limping child up to age 5. J Am Coll Radiol 2018;15(11S):S252\u2013S262. CHAPTER 47 \u25a0 LYMPHADENOPATHY KIYETTA H. ALADE, BETH M. D\u2019AMICO INTRODUCTION Lymphadenopathy, defined as an enlargement of lymph nodes, is a frequent presenting sign in children. Most illnesses causing lymphadenopathy are common viral or bacterial infections that improve spontaneously or with an appropriate course of antimicrobial therapy. However, some serious illnesses, including malignancies, can present first as lymphadenopathy. Thus, a focused history, thorough physical examination, and knowledge of various causes of adenopathy are important in formulating an appropriate differential diagnosis."
  },
  {
   "source": "Diagnostic Imaging: Pediatrics, p. 1130",
   "text": "**1103** This page intentionally left blank ## **SECTION 8** Spine ||Approach to Pediatric Spine|**1106**|| |---|---|---|---| ||**Congenital Spinal Malformations**||| ||Myelomeningocele|**1108**|| ||Lipomyelomeningocele|**1110**|| ||Dorsal Dermal Sinus|**1111**|| ||Caudal Regression|**1112**|| ||Split Cord Malformation|**1113**|| ||Terminal Myelocystocele|**1114**|| ||Neurenteric Cyst|**1115**|| ||Tethered Spinal Cord|**1116**|| ||Scoliosis|**1117**|| ||Syringomyelia|**1118**|| ||**Neoplasms**||| ||Spinal Cord Ependymoma|**1120**|| ||Spinal Cord Astrocytoma|**1122**|| ||Sacrococcygeal Teratoma|**1124**|| ||**Infammatory Lesions**||| ||Discitis/Osteomyelitis|**1128**|| ||Guillain-Barr\u00e9 Syndrome|**1130**|| ||Transverse Myelitis|**1132**|| ||**Trauma**||| ||Craniocervical Junction Injuries|**1134**|| ||Chance Fracture|**1138**|| ||Spondylolysis and Spondylolisthesis<br>DI3_Pediatric_Sectionals_Sept.07.2016.indd 14|**1140**<br>9/8/16 11:36 AM|| Approach to Pediatric Spine"
  }
 ]
}