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Child Abuse & Neglect: Psychosocial Dwarfism

Author: Andrew P Sirotnak, MD, Department Head, Child Abuse and Neglect, Director, Kempe Child Protection Team
Contributor Information and Disclosures

Updated: Mar 19, 2008

Introduction

Background

Psychosocial short stature (PSS) is a disorder of short stature or growth failure and/or delayed puberty of infancy, childhood, and adolescence that is observed in association with emotional deprivation, a pathologic psychosocial environment, or both. A disturbed relationship between child and caregiver is usually noted. A number of pediatric endocrinologists have studied and categorized several generally accepted subtypes; these clinicians also have described therapeutic interventions for children with PSS.

The following 3 subtypes are described, based on the patient's age at presentation and the clinical findings:

• In type I PSS, the age of onset is infancy. Usually, failure to thrive (FTT) is present, but no bizarre behaviors are observed. Patients are often depressed. Normal growth hormone (GH) secretion is found, but responsiveness to GH is unknown. No history of parental rejection is present in type I PSS.
• In type II PSS, the age of onset is 3 years or older. Some of these patients have FTT. Bizarre behaviors are usually observed, and patients are often depressed. Decreased or absent GH secretion is found with minimal responsiveness to GH. A history of parental rejection or pathology is present.
• In type III PSS, the age of onset is in infancy or older. FTT is not usually present, and bizarre behavior is not usually observed. GH secretion is normal; responsiveness to GH is significant. No history of parental rejection is present.

Pathophysiology

The pathophysiology of PSS is complex, involving some nutritional factors and a heterogenous endocrine dysfunction; however, the pathophysiology mostly involves psychological and environmental pathology, affecting the growth and development of the child. Endocrine disturbances of GH, secondary thyroid dysfunction, and somatomedin C (Sm-C) levels are reported in persons with type II PSS. Linear growth is obviously delayed. Growth arrest lines are observed in long bones, and temporary widening of cranial sutures has been reported. Sleep disturbance and pain agnosia are observed. Sequelae in higher cognitive and other psychological functions have been described in adults with a history of this disorder.

Frequency

United States

Frequency in the United States is unknown.

International

Worldwide frequency is unknown.

Mortality/Morbidity

Mortality rates are unknown. Because this is a form of severe child neglect, morbidity can be considered present in all children diagnosed with PSS. Therefore, the rate and severity of morbidity relates to the chronic nature of the deprivation, time of diagnosis, subsequent placement into a nurturing environment, and, finally, the long-term follow-up care while living in a secure and nurturing environment.

Race

All races are affected by child neglect; however, literature and early studies report that most cases of PSS occur in Caucasians.

Sex

Increased occurrence in males has been suggested only by anecdotal reports.

Age

The age of onset for type I PSS is infancy. The onset of type II PSS is in children aged 3 years or older. The onset in type III PSS occurs in infancy or later in childhood.

Clinical

History

Medical literature on psychosocial short stature (PSS) has consistently described children with a history of abuse or neglect and emotional deprivation as the key historic factor in making this diagnosis. Early case reports describe the hospitalization of such children, observation of bizarre behaviors, relatively benign physical examinations, and subsequent endocrine system testing.

Several authors have suggested that the following historic factors are important in making the diagnosis of classic PSS:¹

• Psychological disturbance is present.
  • Bizarre behaviors centered on food and water acquisition, despite seemingly adequate caloric and fluid intake and its availability (polyphagia, polydipsia, hoarding food, gorging and vomiting, eating from garbage bin, drinking from toilet, stealing food)²
  • Sleep disturbances (insomnia, night wandering)
  • Abnormal behaviors (withdrawal, apathy, anxiety, irritability, temper tantrums, shyness, accident proneness, self-injury)
  • Developmental delays (speech retardation, cognitive retardation, psychomotor retardation)
• The caregiver appears to have some psychopathology, and the relationship with the child appears or is known to be abnormal. The following can apply to mothers or caregivers and/or the environment:
  • Depression
  • Anxiety
  • Personality disorders
  • Domestic violence or marital instability³
  • Substance abuse
  • Absent spouse or father of child
  • Myriad of other child abuse–associated issues involving poverty, poor education, generational abuse, and neglect
• Abnormal endocrine function is present but normalizes when the child is removed from the unsafe and nonnurturing environment.⁴
• Malnutrition or inadequate caloric intake alone is not demonstrated to be the primary cause of the growth failure. However, steatorrhea is often observed in individuals with type II PSS.
• Diagnosis of PSS is confirmed by the removal of the child from the unsafe or nonnurturing environment and observation of the following with time:⁵
  • Demonstration of catch-up growth
  • Improvements in behaviors
  • Normalization of hormonal disturbances

Physical

Physical examination of the infant or child with PSS reveals short stature; height less than the third percentile for chronologic age of the child is the most common and important physical examination finding of PSS. The disorder may be mistaken for primary or idiopathic GH deficiency.⁶ Many, but not all, children with PSS are underweight for height; a few children with PSS may be overweight for height. Neurologic examination findings other than those from the mental status examination are usually normal.

• Lung and cardiac examinations are important in excluding organic causes of growth failure, such as cystic fibrosis or heart defects.
• Protuberant abdomen and hepatomegaly, which often are found in children who are malnourished, can be observed in individuals with PSS.
• Perform skin examination for signs of past or chronic abuse (eg, scars, burns, pattern injury) and signs of nutritional or vitamin deficiency.
• Perform examination of genitals and anus for evidence of past or chronic sexual abuse in any patient with child abuse or neglect.
• A neurologic examination to exclude organic causes of bizarre behaviors is usually performed.

Causes

Psychological factors of emotional deprivation have been demonstrated to cause transient GH deficiency in children with PSS. Nutritional deprivation or general caloric deprivation alone is not a major causal factor. The underlying cause of this deprivation by the caregiver can be determined only after the child is removed from the home and a multidisciplinary investigation of the family environment is completed. As in many cases of child abuse and neglect, the reason for the maltreatment may never be determined nor completely explained.

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