pituitrin and Cleft-Palate

Holoprosencephaly is a developmental defect caused by incomplete cleavage of the embryonic forebrain structures during early embryogenesis. We describe a 3-month-old boy with median cleft palate, surgically reconstructed cleft lip, hypotelorism with a flat nose, cryptorchidism, clubfoot, and microcephaly. During the laboratory investigation, his blood sodium level was 154 mmol/L and urine specific gravity was 1.007. Serum osmolarity was 317 mOsm/kg and urine osmolarity was 268 mOsm/kg. Given these findings and the clinical response to vasopressin, diagnosis of central diabetes insipidus was made. Magnetic resonance imaging revealed semilobar holoprosencephaly. The patient responded very well to vasopressin treatment with restoration of serum electrolytes, which remained within normal limits on follow-up. In case of midline facial defects accompanied by hypotelorism with or without developmental delay, the brain should be imaged to confirm its morphology and investigations should be directed by a high index of suspicion of associated endocrinologic dysfunctions.

Topics: Brain; Cleft Palate; Clubfoot; Comorbidity; Diabetes Insipidus; Diagnosis, Differential; Eye Abnormalities; Holoprosencephaly; Humans; Hypothalamus; Infant; Magnetic Resonance Imaging; Male; Microcephaly; Osmolar Concentration; Vasopressins

A neonate with cleft lip and palate and hypopituitarism had persistent hyponatremia despite treatment with hydrocortisone, L-thyroxine, and growth hormone. Serum sodium concentration and urinary osmolality increased and decreased appropriately and concurrently with alterations in sodium and water intake. The ability to regulate serum concentrations of antidiuretic hormone at subnormal serum sodium concentrations indicated a reset osmostat.

Topics: Cleft Lip; Cleft Palate; Female; Humans; Hyponatremia; Hypopituitarism; Infant, Newborn; Vasopressins; Water-Electrolyte Balance

A boy presented with ectrodactyly (lobster claw deformity), bilateral cleft lip and palate, semilobar holoprosencephaly and microcephaly, associated with congenital hypogonadotropic hypogonadism and central diabetes insipidus. Other aspects of pituitary function were normal. We suggest that the ectrodactyly-ectodermal dysplasia-clefting syndrome can be associated with a variety of hypothalamo-pituitary dysfunctions, in addition to the already described isolated growth hormone deficiency.

Topics: Abnormalities, Multiple; Cleft Lip; Cleft Palate; Deficiency Diseases; Diabetes Insipidus; Ectodermal Dysplasia; Growth Hormone; Hand Deformities, Congenital; Holoprosencephaly; Humans; Hypogonadism; Infant, Newborn; Male; Microcephaly; Radiography; Vasopressins

A bilateral cleft lip and palate case received ornithine-vasopressin intra-operatively in preparation for a vasoconstricted field of the various lip segments prior to the repair of the cleft lip. A cyanotic tinge appeared immediately. This eventually led to total necrosis of the upper lip. Various relevant blood tests were done and a mild thrombocytosis was found. Surgical reconstruction of the upper lip was performed by means of a forked cross-lip flap--the main blood supply coming from the columella--as well as by means of an inferiorly pedicled cheek flap from the para-nasal area.

Topics: Cleft Lip; Cleft Palate; Gangrene; Humans; Infant; Lip; Lip Diseases; Male; Necrosis; Ornithine; Surgical Flaps; Thrombocytosis; Vasopressins

The effectiveness of ornipressin and adrenaline as local vasoconstrictors is compared, with special reference to cleft-lip-and-palate surgery. A latent period of 3 - 5 minutes with the use of ornipressin is advised. A brief review comparing the local and general effects of ornipressin and adrenaline is included.

Topics: Cleft Lip; Cleft Palate; Epinephrine; Hemorrhage; Hemostasis, Surgical; Humans; Infant; Infant, Newborn; Ornithine; Vasopressins

Topics: Adjuvants, Anesthesia; Anesthesia, Local; Cleft Lip; Cleft Palate; Epinephrine; Hemostatics; Humans; Infant; Lysine; Oral Hemorrhage; Ornithine; Phenylalanine; Vasoconstrictor Agents; Vasopressins

Topics: Arginine Vasopressin; Child; Cleft Palate; Epinephrine; Felypressin; Hemorrhage; Hemostatics; Humans; Infant; Pharmacology; Surgery, Oral; Vasopressins